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Neurodevelopmental Implications of head positioning
in term and preterm infants Stacey Belliard DPT NTMTC
Disclosures NO affiliations with or involvement in any organization or entity with
any financial interest (such as honoraria educational grants
participation in speakersrsquo bureaus membership employment
consultancies stock ownership or other equity interest and expert
testimony or patent-licensing arrangements) or non-financial interest
(such as personal or professional relationships affiliations
knowledge or beliefs) in the subject matter or materials discussed in
this presentation
Learning Objectives By completion of this session participants will be able to
raquo1) Describe neurodevelopmental implications of poor head positioning in
preterm and term infants
raquo2) Demonstrate neuroprotective head positioning techniques for
premature infants under 32 weeks gestation
raquo3) Identify characteristics of deformational plagiocephaly brachycephaly
and dolichocephaly
raquo4) Demonstrate neuroprotective strategies for preventing and treating
various cranial deformations
Head Positioning and IVH
Positioning and handling to prevent IVH in preterm infants raquo 2003 Vermont Oxford Network focus group developed an evidence based protocol for potentially
best practices to prevent IVH in VLBW infants which includes
raquo Midline head (supine or sidelying) first 72 hours of life
raquo Head of bed elevated to 30 degrees
raquo No lifting of legs
raquo 2017 Systematic review and meta-analysis concluded there is insufficient evidence regarding the
effect of head positioning and tilting on the incidence of GMH-IVH and cerebral hemodynamics and
oxygenation in preterm infants Neither recommend nor refute the use of a neutral head position
andor head tilting in order to prevent GMH-IVH
McLendon et al 2003 de Bijl-
Marcus 2017
CRANIAL DEFORMATIONS
Brain Growth and Development raquoThe rapid growth in neuronal cell number during the 10th through
18th weeks of gestation achieves near adult cell numbers
raquo this is followed by dramatic increases in dendritic growth and
arborization then myelinization
raquoAt 15 months of age the brain is roughly 65 adult size while the
cerebellum has achieved adult proportion
raquoThe majority of myelinization is complete by 2 years of age
Bronfin 2001
Brain Growth raquo A newborns head circumference is larger than the chest circumference at
birth
raquo Average occipital-frontal circumference (OFC) is 35 cm in the term
newborn 45 cm at 1 year and 55 cm in an adult
raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm
per month for the second 3 months of life and 05 cm per month from 6ndash12
months
raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the
adult size at age 10 years In contrast facial size is 40 of adult size at
birth and 65 at 10 years
Bronfin 2001
Anatomy of the Skull
Sutures raquo ―syndesmotic joints
raquo permit growth
raquo Sagittal
raquo Coronal
raquo Lambdoidal
raquo Metopic
Bronfin 2001
Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that
interferes with cell migration and differentiation through genetically
programmed biochemical processes or through extrinsic chemical
interference (teratogens) In essence this process represents an error in
the normal development of a part
raquo Deformation an alteration of a body part that is developing normally until
a mechanical force is applied
Bronfin 2001
Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures
Cranial Deformations raquo Deformational Brachycephaly
raquo Deformational Plagiocephaly
raquo Deformational Dolichocephaly
raquo True Scaphocephaly (Not deformational)
Differentiation between synostotic and non-synostotic head shapes
Deformational Brachycephaly
bull Occipital flattening
bull Increased CI (wide head)
bull Often have some
asymmetryplagiocephaly
bull May have turricephaly
(increased cranial height
limited to posterior cranium)
Synostotic Brachycephaly
bull Bilateral coronal sutures
fused
bull Very rare
bull Congenital not developed
bull Severe forehead retrusion
bull Anterior turricephaly
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Disclosures NO affiliations with or involvement in any organization or entity with
any financial interest (such as honoraria educational grants
participation in speakersrsquo bureaus membership employment
consultancies stock ownership or other equity interest and expert
testimony or patent-licensing arrangements) or non-financial interest
(such as personal or professional relationships affiliations
knowledge or beliefs) in the subject matter or materials discussed in
this presentation
Learning Objectives By completion of this session participants will be able to
raquo1) Describe neurodevelopmental implications of poor head positioning in
preterm and term infants
raquo2) Demonstrate neuroprotective head positioning techniques for
premature infants under 32 weeks gestation
raquo3) Identify characteristics of deformational plagiocephaly brachycephaly
and dolichocephaly
raquo4) Demonstrate neuroprotective strategies for preventing and treating
various cranial deformations
Head Positioning and IVH
Positioning and handling to prevent IVH in preterm infants raquo 2003 Vermont Oxford Network focus group developed an evidence based protocol for potentially
best practices to prevent IVH in VLBW infants which includes
raquo Midline head (supine or sidelying) first 72 hours of life
raquo Head of bed elevated to 30 degrees
raquo No lifting of legs
raquo 2017 Systematic review and meta-analysis concluded there is insufficient evidence regarding the
effect of head positioning and tilting on the incidence of GMH-IVH and cerebral hemodynamics and
oxygenation in preterm infants Neither recommend nor refute the use of a neutral head position
andor head tilting in order to prevent GMH-IVH
McLendon et al 2003 de Bijl-
Marcus 2017
CRANIAL DEFORMATIONS
Brain Growth and Development raquoThe rapid growth in neuronal cell number during the 10th through
18th weeks of gestation achieves near adult cell numbers
raquo this is followed by dramatic increases in dendritic growth and
arborization then myelinization
raquoAt 15 months of age the brain is roughly 65 adult size while the
cerebellum has achieved adult proportion
raquoThe majority of myelinization is complete by 2 years of age
Bronfin 2001
Brain Growth raquo A newborns head circumference is larger than the chest circumference at
birth
raquo Average occipital-frontal circumference (OFC) is 35 cm in the term
newborn 45 cm at 1 year and 55 cm in an adult
raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm
per month for the second 3 months of life and 05 cm per month from 6ndash12
months
raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the
adult size at age 10 years In contrast facial size is 40 of adult size at
birth and 65 at 10 years
Bronfin 2001
Anatomy of the Skull
Sutures raquo ―syndesmotic joints
raquo permit growth
raquo Sagittal
raquo Coronal
raquo Lambdoidal
raquo Metopic
Bronfin 2001
Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that
interferes with cell migration and differentiation through genetically
programmed biochemical processes or through extrinsic chemical
interference (teratogens) In essence this process represents an error in
the normal development of a part
raquo Deformation an alteration of a body part that is developing normally until
a mechanical force is applied
Bronfin 2001
Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures
Cranial Deformations raquo Deformational Brachycephaly
raquo Deformational Plagiocephaly
raquo Deformational Dolichocephaly
raquo True Scaphocephaly (Not deformational)
Differentiation between synostotic and non-synostotic head shapes
Deformational Brachycephaly
bull Occipital flattening
bull Increased CI (wide head)
bull Often have some
asymmetryplagiocephaly
bull May have turricephaly
(increased cranial height
limited to posterior cranium)
Synostotic Brachycephaly
bull Bilateral coronal sutures
fused
bull Very rare
bull Congenital not developed
bull Severe forehead retrusion
bull Anterior turricephaly
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Learning Objectives By completion of this session participants will be able to
raquo1) Describe neurodevelopmental implications of poor head positioning in
preterm and term infants
raquo2) Demonstrate neuroprotective head positioning techniques for
premature infants under 32 weeks gestation
raquo3) Identify characteristics of deformational plagiocephaly brachycephaly
and dolichocephaly
raquo4) Demonstrate neuroprotective strategies for preventing and treating
various cranial deformations
Head Positioning and IVH
Positioning and handling to prevent IVH in preterm infants raquo 2003 Vermont Oxford Network focus group developed an evidence based protocol for potentially
best practices to prevent IVH in VLBW infants which includes
raquo Midline head (supine or sidelying) first 72 hours of life
raquo Head of bed elevated to 30 degrees
raquo No lifting of legs
raquo 2017 Systematic review and meta-analysis concluded there is insufficient evidence regarding the
effect of head positioning and tilting on the incidence of GMH-IVH and cerebral hemodynamics and
oxygenation in preterm infants Neither recommend nor refute the use of a neutral head position
andor head tilting in order to prevent GMH-IVH
McLendon et al 2003 de Bijl-
Marcus 2017
CRANIAL DEFORMATIONS
Brain Growth and Development raquoThe rapid growth in neuronal cell number during the 10th through
18th weeks of gestation achieves near adult cell numbers
raquo this is followed by dramatic increases in dendritic growth and
arborization then myelinization
raquoAt 15 months of age the brain is roughly 65 adult size while the
cerebellum has achieved adult proportion
raquoThe majority of myelinization is complete by 2 years of age
Bronfin 2001
Brain Growth raquo A newborns head circumference is larger than the chest circumference at
birth
raquo Average occipital-frontal circumference (OFC) is 35 cm in the term
newborn 45 cm at 1 year and 55 cm in an adult
raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm
per month for the second 3 months of life and 05 cm per month from 6ndash12
months
raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the
adult size at age 10 years In contrast facial size is 40 of adult size at
birth and 65 at 10 years
Bronfin 2001
Anatomy of the Skull
Sutures raquo ―syndesmotic joints
raquo permit growth
raquo Sagittal
raquo Coronal
raquo Lambdoidal
raquo Metopic
Bronfin 2001
Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that
interferes with cell migration and differentiation through genetically
programmed biochemical processes or through extrinsic chemical
interference (teratogens) In essence this process represents an error in
the normal development of a part
raquo Deformation an alteration of a body part that is developing normally until
a mechanical force is applied
Bronfin 2001
Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures
Cranial Deformations raquo Deformational Brachycephaly
raquo Deformational Plagiocephaly
raquo Deformational Dolichocephaly
raquo True Scaphocephaly (Not deformational)
Differentiation between synostotic and non-synostotic head shapes
Deformational Brachycephaly
bull Occipital flattening
bull Increased CI (wide head)
bull Often have some
asymmetryplagiocephaly
bull May have turricephaly
(increased cranial height
limited to posterior cranium)
Synostotic Brachycephaly
bull Bilateral coronal sutures
fused
bull Very rare
bull Congenital not developed
bull Severe forehead retrusion
bull Anterior turricephaly
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Head Positioning and IVH
Positioning and handling to prevent IVH in preterm infants raquo 2003 Vermont Oxford Network focus group developed an evidence based protocol for potentially
best practices to prevent IVH in VLBW infants which includes
raquo Midline head (supine or sidelying) first 72 hours of life
raquo Head of bed elevated to 30 degrees
raquo No lifting of legs
raquo 2017 Systematic review and meta-analysis concluded there is insufficient evidence regarding the
effect of head positioning and tilting on the incidence of GMH-IVH and cerebral hemodynamics and
oxygenation in preterm infants Neither recommend nor refute the use of a neutral head position
andor head tilting in order to prevent GMH-IVH
McLendon et al 2003 de Bijl-
Marcus 2017
CRANIAL DEFORMATIONS
Brain Growth and Development raquoThe rapid growth in neuronal cell number during the 10th through
18th weeks of gestation achieves near adult cell numbers
raquo this is followed by dramatic increases in dendritic growth and
arborization then myelinization
raquoAt 15 months of age the brain is roughly 65 adult size while the
cerebellum has achieved adult proportion
raquoThe majority of myelinization is complete by 2 years of age
Bronfin 2001
Brain Growth raquo A newborns head circumference is larger than the chest circumference at
birth
raquo Average occipital-frontal circumference (OFC) is 35 cm in the term
newborn 45 cm at 1 year and 55 cm in an adult
raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm
per month for the second 3 months of life and 05 cm per month from 6ndash12
months
raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the
adult size at age 10 years In contrast facial size is 40 of adult size at
birth and 65 at 10 years
Bronfin 2001
Anatomy of the Skull
Sutures raquo ―syndesmotic joints
raquo permit growth
raquo Sagittal
raquo Coronal
raquo Lambdoidal
raquo Metopic
Bronfin 2001
Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that
interferes with cell migration and differentiation through genetically
programmed biochemical processes or through extrinsic chemical
interference (teratogens) In essence this process represents an error in
the normal development of a part
raquo Deformation an alteration of a body part that is developing normally until
a mechanical force is applied
Bronfin 2001
Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures
Cranial Deformations raquo Deformational Brachycephaly
raquo Deformational Plagiocephaly
raquo Deformational Dolichocephaly
raquo True Scaphocephaly (Not deformational)
Differentiation between synostotic and non-synostotic head shapes
Deformational Brachycephaly
bull Occipital flattening
bull Increased CI (wide head)
bull Often have some
asymmetryplagiocephaly
bull May have turricephaly
(increased cranial height
limited to posterior cranium)
Synostotic Brachycephaly
bull Bilateral coronal sutures
fused
bull Very rare
bull Congenital not developed
bull Severe forehead retrusion
bull Anterior turricephaly
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Positioning and handling to prevent IVH in preterm infants raquo 2003 Vermont Oxford Network focus group developed an evidence based protocol for potentially
best practices to prevent IVH in VLBW infants which includes
raquo Midline head (supine or sidelying) first 72 hours of life
raquo Head of bed elevated to 30 degrees
raquo No lifting of legs
raquo 2017 Systematic review and meta-analysis concluded there is insufficient evidence regarding the
effect of head positioning and tilting on the incidence of GMH-IVH and cerebral hemodynamics and
oxygenation in preterm infants Neither recommend nor refute the use of a neutral head position
andor head tilting in order to prevent GMH-IVH
McLendon et al 2003 de Bijl-
Marcus 2017
CRANIAL DEFORMATIONS
Brain Growth and Development raquoThe rapid growth in neuronal cell number during the 10th through
18th weeks of gestation achieves near adult cell numbers
raquo this is followed by dramatic increases in dendritic growth and
arborization then myelinization
raquoAt 15 months of age the brain is roughly 65 adult size while the
cerebellum has achieved adult proportion
raquoThe majority of myelinization is complete by 2 years of age
Bronfin 2001
Brain Growth raquo A newborns head circumference is larger than the chest circumference at
birth
raquo Average occipital-frontal circumference (OFC) is 35 cm in the term
newborn 45 cm at 1 year and 55 cm in an adult
raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm
per month for the second 3 months of life and 05 cm per month from 6ndash12
months
raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the
adult size at age 10 years In contrast facial size is 40 of adult size at
birth and 65 at 10 years
Bronfin 2001
Anatomy of the Skull
Sutures raquo ―syndesmotic joints
raquo permit growth
raquo Sagittal
raquo Coronal
raquo Lambdoidal
raquo Metopic
Bronfin 2001
Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that
interferes with cell migration and differentiation through genetically
programmed biochemical processes or through extrinsic chemical
interference (teratogens) In essence this process represents an error in
the normal development of a part
raquo Deformation an alteration of a body part that is developing normally until
a mechanical force is applied
Bronfin 2001
Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures
Cranial Deformations raquo Deformational Brachycephaly
raquo Deformational Plagiocephaly
raquo Deformational Dolichocephaly
raquo True Scaphocephaly (Not deformational)
Differentiation between synostotic and non-synostotic head shapes
Deformational Brachycephaly
bull Occipital flattening
bull Increased CI (wide head)
bull Often have some
asymmetryplagiocephaly
bull May have turricephaly
(increased cranial height
limited to posterior cranium)
Synostotic Brachycephaly
bull Bilateral coronal sutures
fused
bull Very rare
bull Congenital not developed
bull Severe forehead retrusion
bull Anterior turricephaly
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
CRANIAL DEFORMATIONS
Brain Growth and Development raquoThe rapid growth in neuronal cell number during the 10th through
18th weeks of gestation achieves near adult cell numbers
raquo this is followed by dramatic increases in dendritic growth and
arborization then myelinization
raquoAt 15 months of age the brain is roughly 65 adult size while the
cerebellum has achieved adult proportion
raquoThe majority of myelinization is complete by 2 years of age
Bronfin 2001
Brain Growth raquo A newborns head circumference is larger than the chest circumference at
birth
raquo Average occipital-frontal circumference (OFC) is 35 cm in the term
newborn 45 cm at 1 year and 55 cm in an adult
raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm
per month for the second 3 months of life and 05 cm per month from 6ndash12
months
raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the
adult size at age 10 years In contrast facial size is 40 of adult size at
birth and 65 at 10 years
Bronfin 2001
Anatomy of the Skull
Sutures raquo ―syndesmotic joints
raquo permit growth
raquo Sagittal
raquo Coronal
raquo Lambdoidal
raquo Metopic
Bronfin 2001
Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that
interferes with cell migration and differentiation through genetically
programmed biochemical processes or through extrinsic chemical
interference (teratogens) In essence this process represents an error in
the normal development of a part
raquo Deformation an alteration of a body part that is developing normally until
a mechanical force is applied
Bronfin 2001
Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures
Cranial Deformations raquo Deformational Brachycephaly
raquo Deformational Plagiocephaly
raquo Deformational Dolichocephaly
raquo True Scaphocephaly (Not deformational)
Differentiation between synostotic and non-synostotic head shapes
Deformational Brachycephaly
bull Occipital flattening
bull Increased CI (wide head)
bull Often have some
asymmetryplagiocephaly
bull May have turricephaly
(increased cranial height
limited to posterior cranium)
Synostotic Brachycephaly
bull Bilateral coronal sutures
fused
bull Very rare
bull Congenital not developed
bull Severe forehead retrusion
bull Anterior turricephaly
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Brain Growth and Development raquoThe rapid growth in neuronal cell number during the 10th through
18th weeks of gestation achieves near adult cell numbers
raquo this is followed by dramatic increases in dendritic growth and
arborization then myelinization
raquoAt 15 months of age the brain is roughly 65 adult size while the
cerebellum has achieved adult proportion
raquoThe majority of myelinization is complete by 2 years of age
Bronfin 2001
Brain Growth raquo A newborns head circumference is larger than the chest circumference at
birth
raquo Average occipital-frontal circumference (OFC) is 35 cm in the term
newborn 45 cm at 1 year and 55 cm in an adult
raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm
per month for the second 3 months of life and 05 cm per month from 6ndash12
months
raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the
adult size at age 10 years In contrast facial size is 40 of adult size at
birth and 65 at 10 years
Bronfin 2001
Anatomy of the Skull
Sutures raquo ―syndesmotic joints
raquo permit growth
raquo Sagittal
raquo Coronal
raquo Lambdoidal
raquo Metopic
Bronfin 2001
Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that
interferes with cell migration and differentiation through genetically
programmed biochemical processes or through extrinsic chemical
interference (teratogens) In essence this process represents an error in
the normal development of a part
raquo Deformation an alteration of a body part that is developing normally until
a mechanical force is applied
Bronfin 2001
Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures
Cranial Deformations raquo Deformational Brachycephaly
raquo Deformational Plagiocephaly
raquo Deformational Dolichocephaly
raquo True Scaphocephaly (Not deformational)
Differentiation between synostotic and non-synostotic head shapes
Deformational Brachycephaly
bull Occipital flattening
bull Increased CI (wide head)
bull Often have some
asymmetryplagiocephaly
bull May have turricephaly
(increased cranial height
limited to posterior cranium)
Synostotic Brachycephaly
bull Bilateral coronal sutures
fused
bull Very rare
bull Congenital not developed
bull Severe forehead retrusion
bull Anterior turricephaly
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Brain Growth raquo A newborns head circumference is larger than the chest circumference at
birth
raquo Average occipital-frontal circumference (OFC) is 35 cm in the term
newborn 45 cm at 1 year and 55 cm in an adult
raquo The OFC increases by 2 cm per month for the first 3 months of life 1 cm
per month for the second 3 months of life and 05 cm per month from 6ndash12
months
raquo The volume of the cranial vault is 65 of adult size at birth and 95 of the
adult size at age 10 years In contrast facial size is 40 of adult size at
birth and 65 at 10 years
Bronfin 2001
Anatomy of the Skull
Sutures raquo ―syndesmotic joints
raquo permit growth
raquo Sagittal
raquo Coronal
raquo Lambdoidal
raquo Metopic
Bronfin 2001
Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that
interferes with cell migration and differentiation through genetically
programmed biochemical processes or through extrinsic chemical
interference (teratogens) In essence this process represents an error in
the normal development of a part
raquo Deformation an alteration of a body part that is developing normally until
a mechanical force is applied
Bronfin 2001
Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures
Cranial Deformations raquo Deformational Brachycephaly
raquo Deformational Plagiocephaly
raquo Deformational Dolichocephaly
raquo True Scaphocephaly (Not deformational)
Differentiation between synostotic and non-synostotic head shapes
Deformational Brachycephaly
bull Occipital flattening
bull Increased CI (wide head)
bull Often have some
asymmetryplagiocephaly
bull May have turricephaly
(increased cranial height
limited to posterior cranium)
Synostotic Brachycephaly
bull Bilateral coronal sutures
fused
bull Very rare
bull Congenital not developed
bull Severe forehead retrusion
bull Anterior turricephaly
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Anatomy of the Skull
Sutures raquo ―syndesmotic joints
raquo permit growth
raquo Sagittal
raquo Coronal
raquo Lambdoidal
raquo Metopic
Bronfin 2001
Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that
interferes with cell migration and differentiation through genetically
programmed biochemical processes or through extrinsic chemical
interference (teratogens) In essence this process represents an error in
the normal development of a part
raquo Deformation an alteration of a body part that is developing normally until
a mechanical force is applied
Bronfin 2001
Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures
Cranial Deformations raquo Deformational Brachycephaly
raquo Deformational Plagiocephaly
raquo Deformational Dolichocephaly
raquo True Scaphocephaly (Not deformational)
Differentiation between synostotic and non-synostotic head shapes
Deformational Brachycephaly
bull Occipital flattening
bull Increased CI (wide head)
bull Often have some
asymmetryplagiocephaly
bull May have turricephaly
(increased cranial height
limited to posterior cranium)
Synostotic Brachycephaly
bull Bilateral coronal sutures
fused
bull Very rare
bull Congenital not developed
bull Severe forehead retrusion
bull Anterior turricephaly
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Sutures raquo ―syndesmotic joints
raquo permit growth
raquo Sagittal
raquo Coronal
raquo Lambdoidal
raquo Metopic
Bronfin 2001
Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that
interferes with cell migration and differentiation through genetically
programmed biochemical processes or through extrinsic chemical
interference (teratogens) In essence this process represents an error in
the normal development of a part
raquo Deformation an alteration of a body part that is developing normally until
a mechanical force is applied
Bronfin 2001
Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures
Cranial Deformations raquo Deformational Brachycephaly
raquo Deformational Plagiocephaly
raquo Deformational Dolichocephaly
raquo True Scaphocephaly (Not deformational)
Differentiation between synostotic and non-synostotic head shapes
Deformational Brachycephaly
bull Occipital flattening
bull Increased CI (wide head)
bull Often have some
asymmetryplagiocephaly
bull May have turricephaly
(increased cranial height
limited to posterior cranium)
Synostotic Brachycephaly
bull Bilateral coronal sutures
fused
bull Very rare
bull Congenital not developed
bull Severe forehead retrusion
bull Anterior turricephaly
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Malformation vs Deformation raquo Malformation an intrinsically altered developmental process that
interferes with cell migration and differentiation through genetically
programmed biochemical processes or through extrinsic chemical
interference (teratogens) In essence this process represents an error in
the normal development of a part
raquo Deformation an alteration of a body part that is developing normally until
a mechanical force is applied
Bronfin 2001
Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures
Cranial Deformations raquo Deformational Brachycephaly
raquo Deformational Plagiocephaly
raquo Deformational Dolichocephaly
raquo True Scaphocephaly (Not deformational)
Differentiation between synostotic and non-synostotic head shapes
Deformational Brachycephaly
bull Occipital flattening
bull Increased CI (wide head)
bull Often have some
asymmetryplagiocephaly
bull May have turricephaly
(increased cranial height
limited to posterior cranium)
Synostotic Brachycephaly
bull Bilateral coronal sutures
fused
bull Very rare
bull Congenital not developed
bull Severe forehead retrusion
bull Anterior turricephaly
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Cranial Malformations (Craniosynostosis) raquo Craniosynostosis the premature fusion of one or more cranial sutures
Cranial Deformations raquo Deformational Brachycephaly
raquo Deformational Plagiocephaly
raquo Deformational Dolichocephaly
raquo True Scaphocephaly (Not deformational)
Differentiation between synostotic and non-synostotic head shapes
Deformational Brachycephaly
bull Occipital flattening
bull Increased CI (wide head)
bull Often have some
asymmetryplagiocephaly
bull May have turricephaly
(increased cranial height
limited to posterior cranium)
Synostotic Brachycephaly
bull Bilateral coronal sutures
fused
bull Very rare
bull Congenital not developed
bull Severe forehead retrusion
bull Anterior turricephaly
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Cranial Deformations raquo Deformational Brachycephaly
raquo Deformational Plagiocephaly
raquo Deformational Dolichocephaly
raquo True Scaphocephaly (Not deformational)
Differentiation between synostotic and non-synostotic head shapes
Deformational Brachycephaly
bull Occipital flattening
bull Increased CI (wide head)
bull Often have some
asymmetryplagiocephaly
bull May have turricephaly
(increased cranial height
limited to posterior cranium)
Synostotic Brachycephaly
bull Bilateral coronal sutures
fused
bull Very rare
bull Congenital not developed
bull Severe forehead retrusion
bull Anterior turricephaly
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Differentiation between synostotic and non-synostotic head shapes
Deformational Brachycephaly
bull Occipital flattening
bull Increased CI (wide head)
bull Often have some
asymmetryplagiocephaly
bull May have turricephaly
(increased cranial height
limited to posterior cranium)
Synostotic Brachycephaly
bull Bilateral coronal sutures
fused
bull Very rare
bull Congenital not developed
bull Severe forehead retrusion
bull Anterior turricephaly
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Differentiation between synostotic and non-synostotic head shapes
Deformational
(Scapho)Dolichocephaly
bull Long slender head ―toaster
bull Usually result of extreme head
rotation to one side
bull Premature infants positioned side-
to-side in NICU
bull Normal increase in width from
anterior to posterior
Synostotic Scaphocephaly
(Sagittal Synostosis)
bull ―Boat shaped head
bull Frontal bossing
bull Bilateral occipitalparietal narrowing
posterior to anterior fontanelle
bull Decreased vertical height posterior
cranium
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Differentiation between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
bull Frontal bossing ipsilateral
bull Often associated with head
preference or torticollis
bull Anterior shift of ipsilateral forehead
ear and cheek
bull Eye may appear more open where
forehead shows increased bossing
Unilateral Coronal Synostosis
bull Flattening of forehead ipsilateral
bull Nasal root and midfacial angulation
bull Anteror displacement of ipsilateral
ear
bull Eye appears more open where
forehead is flattened contralateral
eyelid ptosis
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Differentiaion between synostotic and non-synostotic head shapes
Deformational Plagiocephaly
(Positional Plagiocephaly)
bull Unilateral occipital flattening
bull Ipsilateral frontal bossing
bull Contralateral occipital bossing
bull Parallelogram (sort of)
bull Ipsilateral ear shift anterior DP LS
Lambdoidal Synostosis
(Synostotic Posterior Plagiocephaly)
bull Unilateral occipital flattening
bull Contralateral parietal bossing
bull Minimal contralateral frontal bossing
bull Trapezoid windswept
bull Ipsilateral ear shift posterior
DP vs LS
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Assessment raquo No standards
raquo Visual Assessment
raquo Anthropometric Caliper measurement
raquo Flexicurve
raquo Plagiocephalometry
raquo 3D Photography
raquo Radiological imaging
raquo In the near future app under development in Germany
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Measurements From To
Length Glabella Opisthocranium
Width Eurion (1 cm above the
otobasiun superious)
Eurion (1 cm above the
otobasiun superious)
Oblique Frontotemporal point (lateral
point of the ipsilateral eyebrow)
Lamboidal point
Circumference Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Include Glabella and
Opisthocranium (lower edge of
measurement tape directly
above eyebrow)
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Measurements Cranial Index (Cephalic Index) CI
Represents the ratio of maximum cranial width to maximum cranial length
Commonly used for isolated sagittal synostosis (ISS) but also used for any
scaphocephalydolichocephaly or brachycephaly head shape
Scale for Cephalic Index (CI)
raquo Normal 75 ndash 90 mm
raquo Mild 91 ndash 93 mm
raquo Moderate 94 ndash 97 mm
raquo Severe gt97 mm
raquo Normocephaly or plagiocephaly = CI gt76-lt90
raquo Brachycephaly = CI gt90 CI=CWCL X 100
raquo Dolichocephaly = CI lt76
Ruiz-Correa 2006 Likus 2014
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Deformational Dolichocephaly
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Deformational Brachycephaly
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Measurements Cranial Vault Asymmetry Index (CVAI)
(used to measure plagiocephaly)
Level of Deformity CVAI
1 Normal lt35
2 Mild 35-625
3 Moderate 625-875
4 Severe 875-110
5 Very Severe gt110
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Deformational Plagiocephaly
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Neurodevelopmental Implications of Synostotic Cranial Malformations
bull 50 of children with nonsyndromic sagittal suture craniosynostosis had
reading andor spelling learning disability
bull 37 of children with isolated sagittal synostosis had speech andor
language impairment of whom 71 had moderate or severe impairments
bull Children with nonsyndromic craniosynostosis had significantly different
standardized distribution of BSID-II PDI=0 accelerated 43 normal 48
mild delay and 9 significant delay
bull consistently lower mean neurodevelopmental scores in children with single-
suture craniosynostosis compared with controls These results provide
further support for neurodevelopmental screening in young children with
single-suture craniosynostosis Magge 2002 Shipster 2003 Panchal 2001 Starr 2012
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Neurodevelopmental Implications of Synostotic Cranial Malformations bull ―Craniosynostosis is no more considered merely an aesthetic
disorder as numerous reports of neurodevelopmental outcomes are
being published Although there are many issues to be addressed and
confounding variables to take into account patients and their families
are making more complex demands not only in terms of cosmetic
appearance but also in terms of cognitive results The clinicians point
of view should be geared toward comprehensive management
embracing appearance as well as function
bull ―Overall findings suggest that isolated craniosynostosis is associated
with a 3-5 fold increase in risk for cognitive deficits or
learninglanguage disabilities
Shim 2016 Collett 2005
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Incidence of Otitis Media
bull ―hellipstructural differences in the anatomy of the middle ear and eustachian tube can
result in an increased susceptibility to otitis media
bull A retrospective study using a parent questionnaire comparing 1259 patients with
deformational plagiocephaly to patient data from CDC Questionnaire showed a
trend directly correlating otitis media and severity level of DP (not statistically
significant)
bull In addition a subset of infants with DP were administered a tympanogram to assess
state of middle ear Tympanogrametry showed a marked percentage of infants with
DP to have eustachian tube dysfunction
bull The more severe cases (types IV-V) of plagiocephaly had a higher percentage of
otitis media than the less severe cases (types I-III)
bull The significantly high percentage of tympanogram readings that pointed to otitis
mediasuggests an overall malfunction of the middle ear drainage function of the
eustachian tube in these children Purzycki 2009
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Auditory ERPs and Plagiocephaly
bull ―It is suspected that the developmental delay in school-aged children
diagnosed as infants suffering from plagiocephaly is caused by the
modification of the skull form
bull This study compared auditory ERPs in infants with DP and healthy
controls
bull ―Differences between the patients and control subjects indicate that already
at this early age the presence of the plagiocephalic skull signals
compromise of brain functioning
bull ―The present data suggest that most of the plagiocephalic infants have an
elevated risk of auditory processing disorders
bull This study demonstrated ―for the first time that the central sound
processing as reflected by ERPs is affected in children with
plagiocephaly Balan et al
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Figure 1 Typical auditory ERPs of plagiocephalic (solid line) and control (dashed line)
children of 6 to 16 months of age The P150 and N250 deflections are visible Balan et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations ERPs and Deformational Plagiocephaly
bull In contrast to prior study infants with deformational plagiocephaly did not
differ from controls with respect to the maximum P150 or N450 signals
bull ―This study marks the first electrophysiologic examination to demonstrate
that deformational plagiocephaly is not likely associated with significant
impariments in language function
bull This study used phonemes rather than tones as the external stimulus to
directly examine language processing not just auditory processing
bull ―The scope of this investigation was limited to language systems and other
areas of brain functioning may be at risk in infants with deformational
plagiocephaly
Hashim et al
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Aggregate event-related potential waveforms from plagiocephalic and control infants over the left frontal scalp
illustrating the P150 and N450 components Hashim et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull two abnormal conditions were identified hemifield asymmetries in which the breadth
of the left and right hemifields differed by more than 20 degrees and constricted
hemifields in which a given hemifield was at least 20 degrees less than those
established for normal controls of the same age
bull Interestingly there was no statistical correlation between the side of the visual-field
defects relative to the side of the occipital flatness However for the majority of the
16 infants for whom these comparisons were investigated the visual defects were
ipsilateral to the flattened occiput A correlation between the severity of the hemifield
constriction and the severity of the cranial asymmetry was also observed
bull These observations do not speak to whether these defects are a product of
plagiocephaly itself or the result of a more global developmental delay
Siatakowski et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Visual Field Defects
bull Eight of 93 patients (9) with deformational plagiocephaly had unilateral
astigmatism and 14 (15) had bilateral astigmatism
bull Children with deformational plagiocephaly do not have an increased
prevalence of strabismus compared with the general population but do
have an increased prevalence of astigmatism whereas children with
nonsyndromic craniosynostotic plagiocephaly have an increased
prevalence of strabismus and astigmatism
Gupta et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Mandibular Symmetry
bull Observations made at the Washington University School of Medicine St Louis
Missouri in 1996 At that time CT scans were commonly obtained
bull 23 scans available to analyze the shape and volume of the mandible which
although not directly affected by positional forces appeared to be affected by the
altered shape of the cranium
bull This analysis found that the volume of the mandible on the affected side (ie the
side of the occipital flatness) was roughly 4 percent larger than on the contralateral
side
bull Similarly small but consistently significant differences were observed in several
other defined linear measurements between the affected and non-affected sides of
the mandible
bull Unfortunately given the preliminary nature of the article neither the possible effects
of these mandibular asymmetries on oral functions nor their response to
interventions such as remolding orthoses were investigated Kane et al
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations
Developmental Delay
bull Children with DP were more likely to require special education services in school than
their non-affected siblings (349 vs 66) Required services included speech therapy
occupational therapy and physical therapy
bull 36 of children with deformational plagiocephaly had delays in one or more domain on
the ASQ (Ages and Stages Questionnaire)
bull before any intervention infants with deformational plagiocephaly show significant delays
in both mental and psychomotor development Also of particular note is that no child with
deformational plagiocephaly showed accelerated development
bull Children with DP scored lower on all scales of the BSID-III than children without DP
Differences were largest in cognition language and adaptive behavior and smallest in
motor development These findings do not imply that DP causes developmental
problems but may nonetheless serve as a marker of developmental risk Clinicians
should screen children with DP for developmental concerns to facilitate early identification
and intervention Miller Hutchinson
Kordestani Collett
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Neurodevelopmental Implications of Nonsynostotic Cranial Deformations Developmental Delay
bull Australian infants with DP displayed significantly weaker motor skills than normative
estimates malesgt females at very least a diagnostic marker of neurodev problems
bull A positive association between plagiocephaly and developmental delay was reported in
13 of 19 studies including 4 of 5 studies with ―strong methodological quality
bull This review suggests plagiocephaly is a marker of elevated risk of developmental delays
Motor delay was the most commonly affected domain reported in high-quality papers
Clinicians should closely monitor infants with plagiocephaly
bull These studies suggest that ―infants with DP are at increased risk for developmental
delays in infancy and the level of risk is comparable to or even greater than the risk
level for infants with craniosynostosis
bull Results of these studies ―very tentatively suggest that DP is associated with increased
risk for developmental delay however a causal association should not be presumed
Knight Martinuk Collett
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Incidence of Deformational Plagiocephaly bull The incidence of plagiocephaly varies widely and is based on anecdotal evidence of
increase in the number of referrals to specialty clinics Five studies have produced
varying results indicating that the incidence of plagiocephaly ranges from 31 to
610
bull This is the first study to estimate the incidence of positional plagiocephaly using 4
community-based data collection sites in infants ranging from 7 to 12 weeks of age
The estimated incidence of positional plagiocephaly was found to be 466
bull Of the 440 infants assessed 205 were observed to have some form of
plagiocephaly
bull The incidence of plagiocephaly in infants at 7 to 12 weeks of age was estimated to
be 466
bull Of all infants with plagiocephaly 632 were affected on the right side
bull Of all infants with plagiocephaly783 had a mild form
Mawji et al
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Risk Factors for Deformational Plagiocephaly bull Premature
bull Gender (boys)
bull Birth rank (first)
bull Only bottle feeding
bull Same side when bottle feeding
bull Infant head preference in supine sleeping
bull Prone positioning on monitors (tummy time lt 3 timesday when
awake)
bull ―Children with occipital VP shunts are at significant risk of
developing contralateral positional plagiocephaly particularly in the first
12 months of life
Vlimmeren 2007Stuart 2017Hutchison 2003
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Mechanism of Cranial Deformation Several Hypotheses
1) Infantrsquos head is ―soft or ―malleable (water balloon model)
-No immediate deformation takes place (severity peaks ~ 4 months)
-not all infants have their ―soft heads deformed
2) Inherent problem with bone mineralization
-no evidence to support this hypothesis either
3) Hereditary
-identical twins
-human cerebrum is not naturally flat or asymmetric
Rogers 2011
Rogers
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
History of Cranial Deformation raquo Intentional cranial deformation
Common in ancient cultures of Peru N American Chinook Indians
French aristocracy and others
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Modern Cranial Deformation raquo Incidence of deformational plagiocephaly and brachycephaly has risen drastically
since 1996 and the introduction of safe sleep protocol
raquo Container Babies
raquo New life saving respiratory equipment
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Newtonrsquos first law for cranial deformation
raquo An object at rest (infantrsquos head on bed) there will be an equal but opposite force
from the bed to the infantrsquos head
raquo This counterforce will resist cranial growth in the area of contactand consequently
volume increases will be displaced to areas where there is no resistance
raquo The force applied by the head to the resting surface equals the weight of the
infantrsquos head multiplied by the force of gravity (F=mg)
raquo The process is fastest in early infancy and tapers dramatically even after the first
year of life
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
The pumpkin analogy
A stationary pumpkin growing against a firm planar surface
will become flat over time The degree of deformation is
proportionate to the rate of growth against a constant and
fixed external force This may explain why infants with larger
average head sizes (eg males and larger infants) and those
with rapid rates of head growth (eg premature infants) are
more likely to develop DP and DB
Rogers 2011
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Proposed Developmental Pathways for Mechanism of DP and Developmental Delay
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Prevention and Treatment Strategies raquo 1 Alternating infantrsquos head of bed direction
raquo 2 Changing crib position from parallel to perpendicular to headwall
raquo 3 Rotating infantsrsquo position in bed on schedule between supine prone and leftright
side-lying
raquo 4 Providing cares from both sides of bed
raquo 5 Providing daily prone positioning (when awake at home on monitors at hospital)
raquo 6 Alternate feeding positions (use both arms)
raquo 7 Use supportive equipment for respiratory tubing as necessary
raquo 8 Assess size and fit of respiratory caps every cares (adjust as necessary)
raquo 9 Weekly assessment monitoring and education by physical therapist
raquo 10 Helmet therapy if severe (best at 56 months-12 months of age still helps up to 18 months)
ldquoA 4-month standardized pediatric physical therapy program to treat positional preference
significantly reduced the prevalence of severe deformational plagiocephaly compared with usual
care ―
Danner-Bowman 2015 Rogers 2011 Vlimmeren 2008
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
Equipment and Positioning Aides raquo Alternative sleeping surfaces
raquo Custom fabricated foam cups
raquo Tortle hat
raquo Cuddle Cup (coming soon from DandleLion)
raquo Specialized pillows
raquo Orthotic HelmetBand
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
References raquo Balan P Kushnerenko E Sahlin P Huotilainen M Naumlaumltaumlnen R Hukki J Auditory ERPs reveal brain dysfunction
in infants with plagiocephaly J Craniofac Surg 2002 Jul13(4)520-5 discussion 526
raquo de Bijl-Marcus K Brouwer A de Vries Lrsquo van Wezel-Meijler G The Effect of Head Positioning and Head Tilting
on the Incidence of Intraventricular Hemorrhage in Very Preterm Infants A systematic Review Neonatology
2017 111267-279
raquo Bronfin Daniel R Misshapen Heads in Babies Position or Pathology Ochsner J 2001 Oct 3(4) 191ndash199
raquo Collett B Brieger D King D Cunningham M Speltz M Neurodevelopmental Implications of ―Deformational
Plagiocephaly Jdev Behav Pediatr 2005 October 26(5) 379-389
raquo Collett Brent R Gray Kristen E Starr Jacqueline R Heike Carrie L Cunningham Michael L Speltz
Matthew L Development at Age 36 Months in Children With Deformational Plagiocephaly Pediatrics January
2013 Volume 131 Number 1
raquo Danner-Bowman K Cardin A Neuroprotective Core Measure 3 Positioning and Handling-A Look at Preventing
Positional Plagiocephaly
raquo Gupta Pankaj C BA Foster Jill MDdagger Crowe Susan COT Papay Frank A MD FACS FAAPDagger Luciano Mark
MD PhDsect Traboulsi Elias I MD
raquo Journal of Craniofacial Surgery July 2003 - Volume 14 - Issue 4 - pp 529-532
raquo Hashim PW Travieso R Persing JA Coffman M Mukerji C Naples A Tillman RM Terner J Landi N Patel
A Steinbacher D Mayes L McPartland J Brain electrophysiology reveals intact processing of speech sounds in
deformational plagiocephaly Plast Reconstr Surg 2014 Jun133(6)835e-841e
raquo Hutchison B Thompson J Mitchell E Determinants of Nonsynostotic Plagiocephaly A Case-Control Study
Pediatrics Vol 112 No 4 October 2003
raquo Hutchinson BL Stewart Alistair Mitchell Edwin characteristics head shape measurements and developmental
delay in 287 consecutive infants attending a plagiocephaly clinic Acta Paediatric 2009 Vo 98 p 1494-1499
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
References raquo Kane AA L Lo M W Vannier and JL Marsh 1996 Mandibular dysmorphology in unicoronal synostosis and
plagiocephaly without synostosis Cleft Palate-Craniofacial Journal 33(5)418-23
raquo Knight S Anderson VMeara J Da Costa A Early Neurodevlopment in Infants With Deformational Plagiocephaly The
Journal of Carniofacial Surgery Vol 24 Number 4 July 2013
raquo Kordestani RK1 Patel S Bard DE Gurwitch R Panchal JNeurodevelopmental delays in children with deformational
plagiocephalyPlast Reconstr Surg 2006 Jan117(1)207-18 discussion 219-20
raquo Likus W Bajor G Gruszczyńska K Baron J Markowski J Machnikowska-Sokołowska M MilkaD and Lepich T
Cephalic Index in the First Three Years of Life Study of Children with Normal Brain Development Based on Computed
Tomography The Scientific World Journal
Volume 2014 (2014)
raquo Magge SN Westerveld M Pruzinsky T Persing JA Lon-term neuropsychological effects of sagittal craniosynostosis on
child development J Craniofac Surg 2002 Jan13(1) 99-104
raquo Mawji A Ardene Vollman R Hatfield J McNeil D Sauveacute R The Incidence of Positional Plagiocephaly A Cohort Study
Pediatrics Jul 2013 peds2012-3438
raquo Martinuk Alexandra L C Vujovich-Dunn Cassandra Park Miles Yu William Lucas Barbara R Plagiocephaly and
Developmental Delay A Systematic Review Journal of Developmental amp Behavioral Pediatrics January 2017-Volume 38-
Issue 1-p 67-68
raquo McLendon D Check J Carteaux P Michael L Moehring J Secrest JW Clark SE Cohen H Klein SA Boyle D George
JA Okuno-Jones S Buchanan DS McKinley P Whitfield JM Implementation of potentially better practices for the
prevention of brain hemorrhage and ischemic brain injury in very low birth weight infants Pediatrics 2003 Apr111(4 Pt
2)e497-503
raquo Miller Rober I Clarren Sterling K Long-Term Developmental Outcomes in Patients with Deformational Plagiocephaly
Pediatrics February 2000 Vol 105Issue 2
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
References raquo Panchal J Amirsheybani H Gurwitch R Cook V Francel P Neas B Levine N Neurodeveloment in children with single-
suture craniosynostosis and plagiocephaly without synostosis Plast Reconstr Surg 2001 Nov 108(6) 1492-8 discussion
1499-500
raquo Purzycki A1 Thompson E Argenta L David L Incidence of otitis media in children with deformational plagiocephaly J
Craniofac Surg 2009 Sep20(5)1407-11 doi 101097SCS0b013e3181aee369
raquo Rogers Gary F Deformational Plagiocephaly Brachycephaly and Scaphocephaly Part I Terminology Diagnosis and
Etiopathogenesis The Journal of Craniofacial Surgery Vol 22 Number 1 January 2011
raquo Ruiz-Correa S Sze R Starr J Lin H Speltz M Cunningham M Hing A New Scaphocephaly Severity Indices of
Sagittal Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal
March 2006 Vol 43 No 2
raquo Salvador Ruiz-Correa PhD Raymond W Sze MD Jacqueline R Starr PhD Hen-Tzu J Lin MS Matthew L Speltz
PhD Michael L Cunningham MD PhD Anne V Hing MD New Scaphocephaly Severity Indices of Sagittal
Craniosynostosis A Comparative Study With Cranial Index Quantifications Cleft PalatendashCraniofacial Journal March 2006
Vol 43 No 2
raquo Shim K Park E Kim J Kim Y Kim D Neurodevelopmental Problems in Non-Syndromic Craniosynostosis J Korean
Neurosurg Soc 2016 May 59(3) 242ndash246Published online 2016 May 10
raquo Shipster C Hearst D Somerville A Stackhouse J Hayward R Wade A Speech language and cognititve development in
children with isolated sagittal synostosis Dev Med Child Neurol 2003 Jan 45(1)34-43
raquo Siatkowski RM AC Fortney SA Nazir et al 2005 Visual field defects in deformational posterior plagiocephaly Journal
of the American Association for Pediatric Ophthalmology and Strabismus 9(3)274-78
raquo Starr JD Collett B Gaither R Kapp-Simon K Cradock M Cunningham M and Speltz M Multicenter Study of
Neurodevelopment in 3-Year-Old Children With and Without Single-Suture CraniosynostosisArch Pediatr Adolesc Med
2012 Jun 1 166(6) 536ndash542
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
References raquo Stuart A G Roberts Joseph D Symonds Reema Chawla Emma TomanJonathan Bishopand Guirish A Solanki
Positional plagiocephaly following ventriculoperitoneal shunting in neonates and infancymdashhow serious is it Childs Nerv
Syst 2017 33(2) 275ndash28
raquo Van Vlimmeren LAvan der Graaf YBoere-Boonekamp MM LrsquoHoir MP Helders PJ Engelbert RH Risk factors for
deformational plagiocephaly at birth and at 7 weeks of age a prospective cohort study Pediatrics 2007 Feb119(2)e408-
18
raquo httpthejnsorgdoiabs103171ped200510220253journalCode=ped
raquo httpswwwlinkedincompulseapp-measurement-cranial-deformation-olga-greb
raquo httpswwwtransmitdegeschaeftsbereichepatente-innovations-und-gruenderberatung
raquo httpadcbmjcomcontent93182full
raquo httpswwwcappskidsorgcephalic-index-what-do-the-numbers-mean
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