Congenital Chest Wall Diseases

Congenital Chest Wall Congenital Chest Wall Disorders: A Radiological Disorders: A Radiological

AnalysisAnalysis

Alyssa Courtney, Harvard Medical School, Alyssa Courtney, Harvard Medical School, University of Queensland.University of Queensland.

Gillian Lieberman, MD.Gillian Lieberman, MD.

September 2010Alyssa Courtney, Year IVGillian Lieberman, MD

Presentation OutlinePresentation Outline

Chest Wall Anatomy ReviewChest Wall Anatomy Review

Types of Chest Wall Disorders in Types of Chest Wall Disorders in ChildrenChildren

Imaging ModalitiesImaging Modalities

Congenital Osseous AbnormalitiesCongenital Osseous Abnormalities

Congenital Soft Tissue AbnormalitiesCongenital Soft Tissue Abnormalities

Final PatientFinal Patient

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Alyssa Courtney, Year IVGillian Lieberman, MD


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Anatomy: Thoracic SkeletonAnatomy: Thoracic Skeleton

ScapulaScapula

AcromionAcromion processprocess

CoracoidCoracoid processprocess

ClaviclesClavicles

SternumSternum

ManubriumManubrium

AngleAngle

BodyBody

XiphoidXiphoid processprocess

RibsRibs44From Anatomy TV. http://www.anatomy.tv. Accessed 9th September 2010.


http://www.anatomy.tv/

Anatomy: Thoracic Anatomy: Thoracic Muscles, Posterior Muscles, Posterior Chest WallChest Wall

Internal Internal intercostalsintercostals

SubcostalsSubcostals

55


From Anatomy TV. http://www.anatomy.tv. Accessed 9th September 2010.


Anatomy: Thoracic Muscles, Anatomy: Thoracic Muscles, Anterior Chest WallAnterior Chest Wall

SubscapularisSubscapularis

PectoralisPectoralis minorminor

External External intercostals intercostals

IntercostalIntercostal fasciafascia

Underlying musclesUnderlying muscles

Internal and innermost Internal and innermost intercostalsintercostals

TransversusTransversus thoracisthoracis

66




Anatomy: Thoracic Anatomy: Thoracic Muscles Muscles –– PectoralisPectoralis MajorMajor

PectoralisPectoralis majormajor

77




Anatomy: Thoracic Anatomy: Thoracic Vessels and NervesVessels and Nerves

Superior, Superior, internal internal and and laterallateral thoracic thoracic arteries and arteries and veinsveins

IntercostalIntercostal vessels and vessels and nervesnerves 88




Types of Chest Wall Disorders Types of Chest Wall Disorders in Childrenin Children

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Chest Wall Disorders in Children: Chest Wall Disorders in Children: Osseous AbnormalitiesOsseous Abnormalities

Congenital

Pectus excavatum

Pectus carinatum

Tilting of the sternum

Sternal fusion, rib, and scapula abnormalities

Benign

Osteochondroma

Enchondroma

Fibrous Dysplasia

Infection

Langerhans Cell Histiocytosis

Benign Masses

Mesenchymal hamartoma

Malignant Masses

Ewing’s sarcoma

Osteosarcoma

1010Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.


Chest Wall Disorders in Children: Chest Wall Disorders in Children: Soft Tissue AbnormalitiesSoft Tissue Abnormalities

Congenital

Poland syndrome

Lymphatic and venous malformations

Benign

Hemangioma

Rarer –

Lipoblastoma

Fibroma

Fibromatosis

Infantile myofibromatosis

Neurofibromas

Schwannomas

Infection

Langerhans Cell Histiocytosis

Malignant Masses

Primitive neuroectodermal tumor

Rhabdomyosarcoma

Lymphoma

Rarer –

Congenital fibrosarcoma

Malignant peripheral nerve sheath tumor

Mesenchymal chondrosarcoma

Neuroblastoma1111


Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.

Imaging ModalitiesImaging Modalities

1212


Imaging Modalities: RadiographyImaging Modalities: Radiography

Primary screening modality forPrimary screening modality for

Symptomatic or palpable chest wall Symptomatic or palpable chest wall processesprocesses

May provide a definitive diagnosis of May provide a definitive diagnosis of benign osseous lesionsbenign osseous lesions

Can be useful in preliminary assessment Can be useful in preliminary assessment of suspected malignant osseous lesionof suspected malignant osseous lesion

1313



Imaging Modalities: Use of Imaging Modalities: Use of Computed Tomography (CT)Computed Tomography (CT)

Useful for Useful for

Further evaluation if Further evaluation if ––

Normal radiographsNormal radiographs

Inconclusive radiographsInconclusive radiographs

Defining lesion extentDefining lesion extent

Determining nature of a disorderDetermining nature of a disorder

Narrowing the range of differentialsNarrowing the range of differentials

But concerns remain for radiation dose and But concerns remain for radiation dose and possible carcinogenic effectspossible carcinogenic effects

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Imaging Modalities: CTImaging Modalities: CT

Can use singleCan use single--detector or detector or multidetectormultidetector CTCT

Maximize spatial resolution by using Maximize spatial resolution by using smallest possible field of viewsmallest possible field of view

If infectious or If infectious or neoplasticneoplastic processes processes considered, use a nonconsidered, use a non--ionic intravenous ionic intravenous contrast materialcontrast material

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Imaging Modalities: Use of Magnetic Imaging Modalities: Use of Magnetic Resonance Imaging (MRI)Resonance Imaging (MRI)

Superior contrast and spatial resolution Superior contrast and spatial resolution without ionizing radiation or iodinated without ionizing radiation or iodinated contrastcontrast

Limited in smaller children due to the Limited in smaller children due to the relatively long duration of examination relatively long duration of examination resulting in resulting in --

Possible sedation Possible sedation

Respiratory artifact from breathingRespiratory artifact from breathing1616



Imaging Modalities: MRIImaging Modalities: MRI

Often reserved for Often reserved for --

ProblemProblem--solvingsolving

Evaluation of vascular anomaliesEvaluation of vascular anomalies

Optimal results if Optimal results if --

Use smallest field of view possibleUse smallest field of view possible

MinimiseMinimise patient motionpatient motion

MinimiseMinimise scan timescan time

Intravenous contrast is used in most casesIntravenous contrast is used in most cases

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Imaging Modalities: MRI Protocols Imaging Modalities: MRI Protocols

Soft tissue pathology Soft tissue pathology ––

MultiplanarMultiplanar T1T1--weighted turbo spin echo and a weighted turbo spin echo and a fatfat--suppression sequencesuppression sequence

If If neoplasticneoplastic, infectious or vascular suspicions , infectious or vascular suspicions use a three dimensional gradient echo T1use a three dimensional gradient echo T1-- weighted imaging with fat suppressionweighted imaging with fat suppression

Bone pathology Bone pathology ––

include include multiplanarmultiplanar spin echo T1spin echo T1-- and T2and T2-- weighted sequences for assessment of marrow weighted sequences for assessment of marrow signalsignal

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Imaging Modalities: MRI Imaging Modalities: MRI PostprocessingPostprocessing

PostprocessingPostprocessing techniques are performed techniques are performed to further define and characterize the to further define and characterize the pathologypathology

SubtractionSubtraction

MultiplanarMultiplanar reconstructionreconstruction

MaximalMaximal--intensity projectionsintensity projections

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Imaging Modalities: Use of Imaging Modalities: Use of UltrasoundUltrasound

Evaluation of palpable, superficial, softEvaluation of palpable, superficial, soft-- tissue chest wall pathologytissue chest wall pathology

Useful in children as Useful in children as ––

RiskRisk--freefree

NonNon--invasiveinvasive

Fast examination timeFast examination time

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Imaging Modalities: Details of Imaging Modalities: Details of UltrasoundUltrasound

Use a highUse a high--frequency linear transducer to frequency linear transducer to

Determine if lesion presentDetermine if lesion present

Determine if cystic or solidDetermine if cystic or solid

Use color Doppler ultrasound and spectral Use color Doppler ultrasound and spectral tracings for information about vascular tracings for information about vascular flow in flow in ––

Vascular malformationsVascular malformations

HemangiomasHemangiomas2121



Congenital Osseous Congenital Osseous AbnormalitiesAbnormalities

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1.1. PectusPectus ExcavatumExcavatum2.2. PectusPectus CarinatumCarinatum3.3. Tilting of the SternumTilting of the Sternum4.4. SternalSternal Fusion AbnormalitiesFusion Abnormalities5.5. Rib AbnormalitiesRib Abnormalities6.6. Scapula AbnormalitiesScapula Abnormalities

2323


PectusPectus ExcavatumExcavatum

AA deformity of the chest wall characterized deformity of the chest wall characterized by a by a sternalsternal depression typically beginning depression typically beginning over the middle of the over the middle of the manubriummanubrium and and progressing inward through to the progressing inward through to the xiphoidxiphoid process.process.

Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th

September 2010.2424


PectusPectus ExcavatumExcavatum: Incidence: Incidence

1:4001:400--1000 live births1000 live births

M > FM > F

90% of anterior chest wall disorders90% of anterior chest wall disorders

Usually sporadic but increased familial Usually sporadic but increased familial incidenceincidence


September 2010.2525


PectusPectus ExcavatumExcavatum: Pathophysiology: Pathophysiology

Several hypotheses Several hypotheses --

Abnormal cartilage developmentAbnormal cartilage development

Underlying pulmonary conditions Underlying pulmonary conditions

egeg. repaired congenital diaphragmatic hernia, spinal . repaired congenital diaphragmatic hernia, spinal muscular atrophy type 1, muscular atrophy type 1, subglotticsubglottic stenosisstenosis, and , and bronchopulmonarybronchopulmonary dysplasiadysplasia

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September 2010.

PectusPectus ExcavatumExcavatum: Associations : Associations and/or Differential Diagnosesand/or Differential Diagnoses

Scoliosis (15%)Scoliosis (15%)

Mitral valve Mitral valve prolapseprolapse

Congenital heart diseaseCongenital heart disease

CardiorespiratoryCardiorespiratory compromisecompromise

Connective tissue disorders Connective tissue disorders ––

MarfanMarfan’’ss syndrome, Ehlers syndrome, Ehlers DanlosDanlos syndrome, and syndrome, and osteogenesisosteogenesis imperfectaimperfecta

Neuromuscular disease Neuromuscular disease

egeg. spinal muscular atrophy. spinal muscular atrophy

Other genetic conditions Other genetic conditions ––

Noonan syndrome, Turner syndrome, and multiple endocrine Noonan syndrome, Turner syndrome, and multiple endocrine neoplasianeoplasia type 2btype 2b

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September 2010.Schwartzstein RM. Diseases of the chest wall. Up to Date. May 2010. Accessed 11th September 2010.

PectusPectus ExcavatumExcavatum: Symptoms: Symptoms

ExertionalExertional intolerance intolerance -- 82% 82% of several hundred of several hundred pediatric patients with pediatric patients with pectuspectus excavatumexcavatum

Chest pain Chest pain –– 68%68%

Poor endurance Poor endurance –– 67%67%

Shortness of breath Shortness of breath –– 42%42%

Cosmetic concerns Cosmetic concerns ––

68% of females68% of females

40% of males40% of males

Usually subsides by 20 years of ageUsually subsides by 20 years of age

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Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th

September 2010.

PectusPectus ExcavatumExcavatum: Evaluation: Evaluation

Physical Exam Physical Exam –– sternalsternal depression, thoracic depression, thoracic abnormalities, musculoskeletal examination abnormalities, musculoskeletal examination respiratory function, and cardiovascular examinationrespiratory function, and cardiovascular examination

Exercise testingExercise testing

Imaging Imaging –– detect severity detect severity and associated scoliosisand associated scoliosis

2929

From Mayer, OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th September 2010.


Fefferman NR. Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th

September 2010.

Index Patient: Index Patient: PectusPectus ExcavatumExcavatum PA ViewPA View

Heart deviated to leftHeart deviated to left

Prominence of Prominence of vessels right of vessels right of midline midline –– obscuring obscuring right heart borderright heart border

Ribs slope Ribs slope downwards more downwards more than normalthan normal

EventrationEventration of right of right diaphragm diaphragm (not a usual (not a usual feature of feature of pectuspectus excavatumexcavatum))PACS, BIDMC

3030Grissom LE. Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in Roentgenology. 1998; Vol XXXIII (No. 2):199-208.


Index Patient: Index Patient: PectusPectus ExcavatumExcavatum PA PA View FindingsView Findings

Heart deviated to leftHeart deviated to left

Prominence of Prominence of vessels right of vessels right of midline midline –– obscuring obscuring right heart borderright heart border

Ribs slope Ribs slope downwards more downwards more than normalthan normal

EventrationEventration of right of right diaphragm diaphragm (not a usual (not a usual feature of feature of pectuspectus excavatumexcavatum))PACS, BIDMC

3131Grissom LE. Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in Roentgenology. 1998; Vol XXXIII (No. 2):199-208.


Index Patient: Index Patient: PectusPectus

ExcavatumExcavatum Lateral ViewLateral View

Deep Deep depression of depression of the sternumthe sternum

BIDMC, PACS 3232

PACS, BIDMC


Grissom LE. Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in Roentgenology. 1998; Vol XXXIII (No. 2):199-208.

PectusPectus ExcavatumExcavatum: : Haller Index Haller Index

Also known as Also known as ‘‘PectusPectus Severity IndexSeverity Index’’

Ratio of the transverse Ratio of the transverse diameter of the thorax (A) to diameter of the thorax (A) to the AP diameter at the the AP diameter at the deepest part of the deepest part of the pectuspectus (B)(B)

Evaluates for surgical repairEvaluates for surgical repair

Surgery usually required with Surgery usually required with indices > 3.25indices > 3.25

3333

From Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th

September 2010.Grissom LE, Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in Roentgenology. 1998; Vol XXXIII (No. 2):199-208. Haller JA. Kramer SS. Lietman SA. Use of CT scans in selection of patients for pectus excavatum surgery: a preliminary report. J Pediatr Surg.1987; 10: 904-906.


PectusPectus ExcavatumExcavatum: Cardiac : Cardiac DistortionDistortion

Significant cardiac Significant cardiac distortion represented distortion represented at the at the xiphoidxiphoid process process as the as the ––

Cardiac compression Cardiac compression index (H/index (H/MM))

Cardiac asymmetry Cardiac asymmetry index (index (PP//MM))

3434From Mayer OH. Pectus excavatum: Etiology and evaluation. Up to Date. May 2010. Accessed 11th

September 2010.


PectusPectus ExcavatumExcavatum: CT: CT

Axial Axial noncontrastnoncontrast CT image of CT image of the chest the chest demonstrating demonstrating rotation and rotation and marked marked depression of depression of the sternumthe sternum

3535

12 year old girl with pectus excavatum


From Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.

PectusPectus ExcavatumExcavatum: Treatment: Treatment

MonitoringMonitoring

Psychological support Psychological support –– if appropriateif appropriate

SternalSternal suctionsuction

SternalSternal magnetmagnet

Prosthetic insertsProsthetic inserts

Physical therapyPhysical therapy

Surgical correction Surgical correction –– moderate to severe moderate to severe deformitiesdeformities

3636Mayer OH. Pectus excavatum: Treatment. Up to Date. May 2010. Accessed on September 11th 2010.




3737


PectusPectus CarinatumCarinatumProtrusion deformity of the anterior chest wall.Protrusion deformity of the anterior chest wall.Types:Types:ChondrogladiolarChondrogladiolar prominenceprominence

Middle and lower portions of sternum prominent and arch forwardMiddle and lower portions of sternum prominent and arch forward

Most commonMost common

ChondromanubrialChondromanubrial prominenceprominence

Upper portion of sternum Upper portion of sternum anteriorlyanteriorly prominent, body of sternum prominent, body of sternum depressed depressed posteriorlyposteriorly, and a final anterior deflection of distal , and a final anterior deflection of distal sternumsternum

ZZ--shape in lateral viewshape in lateral view

Less commonLess common3838

Nuchtern JG. Mayer OH. Pectus Carinatum. Up to Date. May 2010. Accessed 11th September 2010.


PectusPectus CarinatumCarinatum: Incidence and : Incidence and PathophysiologyPathophysiology

Incidence:Incidence:

1 in 1500 live births1 in 1500 live births

Male 4:1 FemaleMale 4:1 Female

Pathophysiology:Pathophysiology: Same as Same as PectusPectus ExcavatumExcavatum

3939



PectusPectus CarinatumCarinatum: Associations and/or : Associations and/or Differential DiagnosesDifferential Diagnoses

Musculoskeletal abnormalities Musculoskeletal abnormalities

egeg scoliosisscoliosis

Connective tissue disorders Connective tissue disorders

egeg MarfanMarfan syndrome and syndrome and osteogenesisosteogenesis imperfectaimperfecta

Other genetic conditions Other genetic conditions

egeg Noonan syndrome, Noonan syndrome, cardiofaciocutaneouscardiofaciocutaneous syndrome, Poland syndrome, Coffinsyndrome, Poland syndrome, Coffin--Lowry syndrome, Lowry syndrome, and and MorquioMorquio diseasedisease

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PectusPectus CarinatumCarinatum: Symptoms: Symptoms

Symptoms worsen during puberty Symptoms worsen during puberty --Cosmetic concernsCosmetic concernsRarely (and lacking objective evidence) Rarely (and lacking objective evidence) --Exercise limitationExercise limitationExertionalExertional dyspnoeadyspnoea

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PectusPectus CarinatumCarinatum: Evaluation: Evaluation

Physical Exam Physical Exam –– sternalsternal protrusion, thoracic protrusion, thoracic abnormalities, musculoskeletal examination, abnormalities, musculoskeletal examination, respiratory function, cardiovascular respiratory function, cardiovascular examinationexamination

Exercise testing Exercise testing –– if appropriateif appropriate ImagingImaging –– detect severity, associated detect severity, associated

scoliosisscoliosis

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Companion Companion Patient 1: Patient 1:

PectusPectus CarinatumCarinatum AP ViewAP View

Ribs are Ribs are more more horizontalhorizontal

Thorax can be Thorax can be narrowed in narrowed in pectuspectus carinatumcarinatum –– not not shown hereshown here

4343Grissom LE, Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in Roentgenology. 1998; Vol XXXIII (No. 2):199-208.

PACS, BIDMC


Companion Companion Patient 1: Patient 1:

PectusPectus CarinatumCarinatum

Lateral ViewLateral View

Prominently Prominently bowed bowed sternumsternum

Increased Increased AP diameter AP diameter of the chestof the chest

BIDMC, PACS 4444

PACS, BIDMC


Grissom LE, Harcke HT. Thoracic Deformities and the Growing Lung. Seminars in Roentgenology. 1998; Vol XXXIII (No. 2):199-208.

PectusPectus CarinatumCarinatum: Haller Index: Haller Index

CT ImagingCT Imaging

Measure the Haller index (as seen in Measure the Haller index (as seen in pectuspectus excavatumexcavatum) )

Lower the index = worse deformityLower the index = worse deformity

Mean index of 260 subjects with Mean index of 260 subjects with pectuspectus carinatumcarinatum = 1.81= 1.81

BIDMC, PACS 4545Nuchtern JG, Mayer OH. Pectus Carinatum. Up to Date. May 2010. Accessed September 11th 2010.


PectusPectus CarinatumCarinatum: Treatment: Treatment

No interventionNo intervention

Psychological support Psychological support –– if appropriateif appropriate

Bracing Bracing –– for patients with a flexible mild for patients with a flexible mild to moderate deformityto moderate deformity

Surgical correction Surgical correction –– moderate to severe moderate to severe deformitiesdeformities

BIDMC, PACS 4646


Nuchtern JG, Mayer OH. Pectus Carinatum. Up to Date. May 2010. Accessed September 11th 2010.



4747


Tilting of the SternumTilting of the Sternum

Deviation of the typical horizontal positioningDeviation of the typical horizontal positioningof the sternum in the transverse axis of theof the sternum in the transverse axis of thebody.body.Imaging:Imaging:

Usually not apparent on radiographsUsually not apparent on radiographs

Secondary lateral displacement of medial Secondary lateral displacement of medial heads of the adjacent clavicles may assist heads of the adjacent clavicles may assist detectiondetection

4848


Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.

Tilting of the Sternum: Tilting of the Sternum: AssociationsAssociations

Anterior Anterior subluxationsubluxation of the adjacent of the adjacent clavicularclavicular headhead

Abnormal convexity of the adjacent rib Abnormal convexity of the adjacent rib resulting in a palpable chest wall bumpresulting in a palpable chest wall bump

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5050


SternalSternal Fusion AbnormalitiesFusion AbnormalitiesExample: Axial Example: Axial noncontrastnoncontrast CT of 1 month old boy with a bifid sternum: CT of 1 month old boy with a bifid sternum:

markedmarked separation of the separation of the clavicularclavicular headsheads and and depression of soft tissues depression of soft tissues in the location of the expected upper sternumin the location of the expected upper sternum

May be an May be an isolated isolated abnormality and abnormality and can require can require surgical surgical correction to correction to prevent prevent cardiopulmonary cardiopulmonary compromisecompromise

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SternalSternal Fusion Abnormalities: Fusion Abnormalities: AssociationsAssociations

Example: Lateral Chest Radiograph demonstrating Example: Lateral Chest Radiograph demonstrating absence absence of sternum and of sternum and sternalsternal ossification centersossification centers

Severe Severe sternalsternal fusion fusion abnormaliiesabnormaliies are are associated with associated with ––

Congenital heart Congenital heart disease disease egeg ectopiaectopia cordiscordis ((extrathoracicextrathoracic heart)heart)

PentalogyPentalogy of Cantrell of Cantrell (combination of severe (combination of severe sternum, diaphragm, heart sternum, diaphragm, heart and abdominal wall defects)and abdominal wall defects)

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5353


Rib AbnormalitiesRib AbnormalitiesTypes: Types: Agenesis, Agenesis, hypoplasiahypoplasia, and bifid configuration., and bifid configuration.

Developmental anatomic variations can present Developmental anatomic variations can present as asymptomatic palpable chest wall massesas asymptomatic palpable chest wall massesExamples Examples ––

Prominent convexity of anterior rib or costal Prominent convexity of anterior rib or costal cartilagecartilage

Prominence of Prominence of costochondralcostochondral junctionjunction

Small Small parachondralparachondral nodules of unknown originnodules of unknown origin

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Scapula Abnormalities: Scapula Abnormalities: SprengelSprengel’’ss DeformityDeformity

Failure of descent of the scapula.Failure of descent of the scapula.

Most notable scapula deformityMost notable scapula deformity

Sometimes the scapula is tethered to the spine Sometimes the scapula is tethered to the spine by an by an osteocartilaginousosteocartilaginous connection called the connection called the omohyoidomohyoid bone bone

Can cause neck stiffness and restrict Can cause neck stiffness and restrict abduction of the armabduction of the arm

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Brett-Fleegler M. Evaluation of neck stiffness in children. Up to Date. August 2009. Accessed on September 11th 2010.Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.Thacker MM. Sprengel Deformity. eMedicine. July 2009. Accessed on September 11th 2010.

Scapula Abnormalities: Scapula Abnormalities: SprengelSprengel’’ss Deformity, Associations and TreatmentDeformity, Associations and Treatment

Associated with Associated with

KlippelKlippel--FeilFeil syndromesyndrome

Osseous abnormalitiesOsseous abnormalities

Spinal cord abnormalitiesSpinal cord abnormalities

Treatment:Treatment: Physiotherapy or surgeryPhysiotherapy or surgery

5757


Brett-Fleegler M. Evaluation of neck stiffness in children. Up to Date. August 2009. Accessed on September 11th 2010.Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.Thacker MM. Sprengel Deformity. eMedicine. July 2009. Accessed on September 11th 2010.

Congenital Soft Tissue Congenital Soft Tissue AbnormalitiesAbnormalities

5858



1.1. Poland SyndromePoland Syndrome2.2. Lymphatic MalformationsLymphatic Malformations3.3. Venous MalformationsVenous Malformations

5959


Poland SyndromePoland SyndromeRare congenital malformation Rare congenital malformation of chest wall with of chest wall with hypoplasiahypoplasia ororaplasiaaplasia of the of the pectoralispectoralis majormajormuscle and adjacentmuscle and adjacentcartilaginous, osseous, and softcartilaginous, osseous, and softtissue structures.tissue structures.Clinical asymmetry of the chestClinical asymmetry of the chest

6060


Fefferman NR, Pinkney LP. Imaging Evaluation of Chest Wall Disorders in Children. Radiol Clin N Am. 2005; 43: 355 – 370.Schwartzstein RM. Diseases of the chest wall. Up to Date. May 2010. Accessed 11th September 2010.

From Habib M. Mahajan S. Kuchey GA. Gupta D. Sharma S. Poland Syndrome, a rare entity. The Internet Journal of Orthopedic Surgery. 2009; 12 (1).

Poland Syndrome: Pathophysiology Poland Syndrome: Pathophysiology and Incidence and Incidence

Pathophysiology: Pathophysiology:

Unknown Unknown

Hypothesized to occur as a Hypothesized to occur as a result of result of ipsilateralipsilateral subclaviansubclavian artery disruptionartery disruption

Incidence: Incidence:

1/30 000 live births1/30 000 live births

Usually unilateralUsually unilateral

Males > FemalesMales > Females

Right > LeftRight > Left6161



From Habib M. Mahajan S. Kuchey GA. Gupta D. Sharma S. Poland Syndrome, a rare entity. The Internet Journal of Orthopedic Surgery. 2009; 12 (1).

Poland Syndrome: Chest RadiographsPoland Syndrome: Chest Radiographs

The The hypoplasiahypoplasia of chest wall soft tissues of chest wall soft tissues results in relative lunacy of affected results in relative lunacy of affected hemithoraxhemithorax

Differential diagnoses from radiograph:Differential diagnoses from radiograph:

Pulmonary entities that cause air trappingPulmonary entities that cause air trapping

Congenital lobar emphysemaCongenital lobar emphysema

Obstruction from a foreign bodyObstruction from a foreign body

SwyerSwyer--James syndrome James syndrome

6262



Poland Syndrome: TreatmentPoland Syndrome: Treatment

Surgical correction if severe chest wall Surgical correction if severe chest wall deformitiesdeformities

CT scanning or MR imaging is useful to CT scanning or MR imaging is useful to determine the extent of the deformity for determine the extent of the deformity for presurgicalpresurgical planningplanning

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6464


Lymphatic MalformationsLymphatic MalformationsIncreased number of dilated lymphatic channelsIncreased number of dilated lymphatic channelslined by endotheliumlined by endothelium

MicrocysticMicrocystic, , macrocysticmacrocystic, or combined, or combined

Most common in: Most common in: AxillaAxilla, chest, , chest, cervicofacialcervicofacial regionregion

In the chest: In the chest:

focal or diffuse masses confined to the focal or diffuse masses confined to the subcutaneuossubcutaneuos tissues ortissues or

involve the spine and/or involve the spine and/or mediastinummediastinum

6565



From Children’s Hospital Boston. Lymphatic Malformation. Accessed 14th September 2010.

Lymphatic Malformations: MRI (1)Lymphatic Malformations: MRI (1) 2 year old boy with left lateral chest wall lymphatic

malformations

Axial (C+) T2Axial (C+) T2-- weighted MRI weighted MRI demonstrating a demonstrating a multiloculatedmultiloculated highhigh--signalsignal-- intensity left lateral intensity left lateral chest wall mass chest wall mass with with internal internal septationsseptations

6666



Lymphatic Malformations: MRI (2)Lymphatic Malformations: MRI (2) 2 year old boy with left lateral chest wall lymphatic malformations

Axial fatAxial fat--saturated, T1saturated, T1-- weighted weighted postcontrastpostcontrast MRI MRI –– low signal low signal intensity of the cystic intensity of the cystic component component and and enhancement of walls enhancement of walls and and septationsseptations

6767





6868


Venous MalformationsVenous MalformationsIsolated or multiple dilated, tortuous, thinIsolated or multiple dilated, tortuous, thin--walled (lack of smooth muscle) venous walled (lack of smooth muscle) venous structuresstructures

Grow in proportion to child growthGrow in proportion to child growth

Focal abnormalities through to diffuse Focal abnormalities through to diffuse involvement of the deeper soft tissues and involvement of the deeper soft tissues and bonebone

Affects chest wall less than lymphatic Affects chest wall less than lymphatic abnormalitiesabnormalities

6969



Venous Malformations: ImagingVenous Malformations: Imaging

Ultrasound Ultrasound –– hypoechoichypoechoic, , isoechoicisoechoic, or , or hyperechoichyperechoicPhlebolithsPhleboliths may also be identifiedmay also be identifiedColor Doppler spectral tracings demonstrate Color Doppler spectral tracings demonstrate

either loweither low--flow venous patterns or no flowflow venous patterns or no flowMRI MRI -- evaluates extent of involvement evaluates extent of involvement

and characterization of flow and characterization of flow

7070



Venous Malformations: Venous Malformations: TimeTime--resolved MR Angiography (1)resolved MR Angiography (1)

Axial (C+) MR STIR Axial (C+) MR STIR image with a image with a cluster cluster of of serpiginousserpiginous highhigh-- signal structuressignal structures

7171

10 year old boy who has left posterolateral chest wall venous malformations



Venous Malformations: Venous Malformations: TimeTime--resolved MR Angiography (2)resolved MR Angiography (2)

7272

Axial Axial postcontrastpostcontrast T1T1-- weighted MRI weighted MRI -- late late venousvenous--phase phase enhancement of enhancement of abnormal vascular abnormal vascular structuresstructures

10 year old boy who has left posterolateral chest wall venous malformations



Venous Malformations: Venous Malformations: Treatment Treatment

ObservationObservation

SclerotherapySclerotherapy

7373



Final PatientFinal Patient

7474



Companion Companion Patient 2Patient 2

WhereWhere’’s the s the congenital congenital chest wall chest wall abnormality?abnormality?

Please turn to the next Please turn to the next page to reveal thepage to reveal theabnormalityabnormality

7575PACS, BIDMC


Companion Companion Patient 2: Patient 2: Relevant Relevant FindingFinding

RibAbnormality

7676PACS, BIDMC


Companion Companion Patient 2: Patient 2: Additional Additional Findings?Findings?

Can you detect the additional findingson this radiograph?

Please turn to the nextpage to reveal thefindings

7777PACS, BIDMC


Companion Companion Patient 2: Patient 2: Additional Additional FindingsFindings

PneumomediastinumTranstracheal oxygenCathetorSubcutaneous neck EmphysemaMulti-focal linear atelectasis in bilateral mid and lower lungs

7878PACS, BIDMC


SummarySummary


Types of Chest Wall Disorders in Types of Chest Wall Disorders in ChildrenChildren

Imaging Modalities: Imaging Modalities:

Chest radiography or ultrasound then Chest radiography or ultrasound then MRI or CT if requiredMRI or CT if required

Congenital Osseous AbnormalitiesCongenital Osseous Abnormalities

Congenital Soft Tissue AbnormalitiesCongenital Soft Tissue Abnormalities

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http://emedicine.medscape.com/article/1242896-overview



AcknowledgementsAcknowledgements

Larry Larry BarbarasBarbaras

Gillian Lieberman, MDGillian Lieberman, MD

Emily HansonEmily Hanson

Pauline Bishop, MDPauline Bishop, MD

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Documents

Congenital Chest Wall Diseases