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Voiding Dysfunction in Children By By Dr.Turky Dr.Turky K. Al-Mouhissen K. Al-Mouhissen Urology Chief Resident - WR Urology Chief Resident - WR King Abdulaziz Medical Center - WR King Abdulaziz Medical Center - WR

Voiding Dysfunction in Children

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Voiding Dysfunction in Children. By Dr.Turky K. Al-Mouhissen Urology Chief Resident - WR King Abdulaziz Medical Center - WR. Normal Bladder Function in Infants & Children:. The bladder is an abdominal organ - PowerPoint PPT Presentation

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Page 1: Voiding Dysfunction              in Children

Voiding Dysfunction

in ChildrenByBy

Dr.TurkyDr.Turky K. Al-MouhissenK. Al-MouhissenUrology Chief Resident - WRUrology Chief Resident - WR

King Abdulaziz Medical Center - WRKing Abdulaziz Medical Center - WR

Page 2: Voiding Dysfunction              in Children

Normal Bladder Function in Infants & Children:Normal Bladder Function in Infants & Children:

The bladder is an abdominal organ The bladder is an abdominal organ

Detrusor consists of meshwork of smooth m. which has the Detrusor consists of meshwork of smooth m. which has the ability to elicit maximal active tension over a wide range of ability to elicit maximal active tension over a wide range of lengthlength

This allows the bladder to be filled at low pressure This allows the bladder to be filled at low pressure (compliance)(compliance)

Reservoir function determined by activity of detrusor m. and Reservoir function determined by activity of detrusor m. and bladder outletbladder outlet

Bladder sphincter plays a major role in urinary continence by Bladder sphincter plays a major role in urinary continence by closure of bladder neck & proximal urethraclosure of bladder neck & proximal urethra

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Literature suggests thatLiterature suggests that Immature detrusor-sphincter coordinationImmature detrusor-sphincter coordination, manifested as detrusor , manifested as detrusor

hypercontractility and interrupted hypercontractility and interrupted voiding, commonly occurs in 1, commonly occurs in 1stst 1-2 1-2 yearsyears causing a degree of functional bladder outflow obstruction causing a degree of functional bladder outflow obstruction

(Sillen et al,1992;Yeung et al,1998)(Sillen et al,1992;Yeung et al,1998)

Some found significant age related differences in the histologic structure of the sphincter compared to adults

Activation, coordination, and integration of various parts of the bladder-sphincter complex involves central, somatic & autonomic nervous system through Sacral parasympathatic (pelvic n.) Thoracolumbar symp. (hypogastric & sympathatic chain) Sacral somatic n. (pudendal n.)

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Parasymp. N. fibers run in pelvic n. Parasymp. N. fibers run in pelvic n. (S2-S4)(S2-S4) to supply the to supply the pelvic pelvic and vesical plexuses before entering the bladderand vesical plexuses before entering the bladder

Symp. n. arises from Symp. n. arises from (T10-L2)(T10-L2) to inferior mesenteric to inferior mesenteric ganglion ganglion hypogastric n.hypogastric n. to the pelvic plexus & to the pelvic plexus & bladder bladder There is also symp. innervation from T10-L2 supplying the There is also symp. innervation from T10-L2 supplying the

detrusor and urethral sphincter detrusor and urethral sphincter

The somatic nervous system The somatic nervous system (pudendal n.)(pudendal n.) supplies the supplies the periurethral pelvic floor musclesperiurethral pelvic floor muscles

The sensory & motor n. carried by all 3 nerves innervate The sensory & motor n. carried by all 3 nerves innervate the bladder and urethral sphincter the bladder and urethral sphincter

Page 5: Voiding Dysfunction              in Children

    

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Symp. supply causes Symp. supply causes Detrusor relaxation (B receptors)Detrusor relaxation (B receptors) Smooth m contraction at trigone & bladder neck (alpha receptors)Smooth m contraction at trigone & bladder neck (alpha receptors)

Parasymp. supply causes detrusor contraction Parasymp. supply causes detrusor contraction (muscarinic receptors)(muscarinic receptors)

Pudendal supply causes striated sphincter contractionPudendal supply causes striated sphincter contraction

Within the spinal cord, information from bladder afferents Within the spinal cord, information from bladder afferents integrated with other sources and projected to brain stem integrated with other sources and projected to brain stem centers to coordinate with mic. centerscenters to coordinate with mic. centers

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Development of Normal Bladder Function Development of Normal Bladder Function and Micturition Controland Micturition Control

CMG studies on normal infants showedCMG studies on normal infants showed bladder function in young children is very bladder function in young children is very

different from adultsdifferent from adults

During the 1During the 1stst 2-3 years of life 2-3 years of life There is progressive development from initially There is progressive development from initially

indiscriminate infants voiding pattern to more indiscriminate infants voiding pattern to more socially conscious and voluntary (adult) type of socially conscious and voluntary (adult) type of micturition micturition

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The natural evolution of bladder control The natural evolution of bladder control entails nervous system & depends on:entails nervous system & depends on:

Progressive increase in bladder functional Progressive increase in bladder functional storage capacity storage capacity

Maturation of voluntary control over the urethral Maturation of voluntary control over the urethral striated muscle sphincterstriated muscle sphincter

Development of direct volitional control over the Development of direct volitional control over the bladder sphincter unit, so that the child can bladder sphincter unit, so that the child can voluntary initiate or inhibit micturition reflex voluntary initiate or inhibit micturition reflex

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Change in bladder function parametersChange in bladder function parameters

Voiding frequency Voiding frequency During the 3During the 3rdrd trimester, the fetus voids at rate trimester, the fetus voids at rate

30 times/24 hrs30 times/24 hrs Immediately after birth, this Immediately after birth, this dropsdrops for the 1 for the 1stst

days only days only Increases again after 1Increases again after 1stst 1/52 to reach a peak 1/52 to reach a peak

by 2-4 /52 to an average by 2-4 /52 to an average once/houronce/hour This rate declines to This rate declines to 10-15 times/day10-15 times/day at 6-12 at 6-12

mo.mo. 8-10 times/day8-10 times/day by 2-3 years by 2-3 years

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This reduction in voiding frequency This reduction in voiding frequency observed during the 1observed during the 1stst years of life related years of life related mainly to an increase in bladder capacity mainly to an increase in bladder capacity parallel to body growth parallel to body growth

By the age of 12, voiding pattern is very By the age of 12, voiding pattern is very similar to that in adult ( 4-6 voids / day ) similar to that in adult ( 4-6 voids / day )

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Bladder capacityBladder capacity An adequate reservoir function of urine storage is An adequate reservoir function of urine storage is

necessary to meet the increase rate of urine production necessary to meet the increase rate of urine production and decreased voiding frequency in the growing childand decreased voiding frequency in the growing child

Bladder capacity can be estimatedBladder capacity can be estimatedFor young infants:For young infants:

Bladder capacity (ml) = 38 + 2.5 * age (mo)Bladder capacity (ml) = 38 + 2.5 * age (mo)

For children:For children:Bladder capacity (ml) = (age [yr] + 2) * 30 koffBladder capacity (ml) = (age [yr] + 2) * 30 koff’’s s

formulaformula

Bladder capacity (ml) = 30 + (age [yr] * 30 HijalmaBladder capacity (ml) = 30 + (age [yr] * 30 Hijalma’’s s formulaformula

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In parallel to increase in bladder capacity, the In parallel to increase in bladder capacity, the mean voided volume of each micturation mean voided volume of each micturation increases with ageincreases with age

CMG studies showed that most infants with CMG studies showed that most infants with

incomplete maturation of det.- sphin. coordination incomplete maturation of det.- sphin. coordination before the age 1, are before the age 1, are still able to have satisfactory still able to have satisfactory emptying (>80)emptying (>80)

There are limited studies on detr. pressure at There are limited studies on detr. pressure at voiding in infants due techn. difficultiesvoiding in infants due techn. difficulties

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Limited data documented Limited data documented higherhigher maximum detr. maximum detr. pressure with mict. than in adultspressure with mict. than in adults MaleMale infants voided significantly higher pressure than infants voided significantly higher pressure than

females (mean Pdetmax 118 vs. 75cm H20, P<.03) females (mean Pdetmax 118 vs. 75cm H20, P<.03)

Studies showed that Studies showed that highhigh detrusor pressures detrusor pressures noted during micturition were mainly observed noted during micturition were mainly observed only during the 1only during the 1stst year of life & decreased year of life & decreased progressively with ageprogressively with age

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Evolution of Normal Micturition ControlEvolution of Normal Micturition Control

Traditionally, it has been assumed that micturition occurs Traditionally, it has been assumed that micturition occurs by simple spinal reflex with no mediation by higher neural by simple spinal reflex with no mediation by higher neural centerscenters

Recent studies showed that even in full term fetuses and Recent studies showed that even in full term fetuses and newborns, mict. Is modulated by newborns, mict. Is modulated by higher centershigher centers

Further extensive modulation occurs during the postnatal Further extensive modulation occurs during the postnatal periodperiod

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During the 2During the 2ndnd & 3 & 3rdrd year of life, there is a progressive year of life, there is a progressive development toward a socially conscious continence and development toward a socially conscious continence and a more a more voluntary (adult) type mictvoluntary (adult) type mict. .

The final steps usually achieved at The final steps usually achieved at 3-4 yrs3-4 yrs Most develop the adult pattern of urinary control & will be dry Most develop the adult pattern of urinary control & will be dry

day & nightday & night

The child has learned to inhibit a mict. Reflex and The child has learned to inhibit a mict. Reflex and postpone voiding and voluntarily initiate mict. At socially postpone voiding and voluntarily initiate mict. At socially acceptable time & placeacceptable time & place

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Neurologic control of normal mic. occurs at different levels Neurologic control of normal mic. occurs at different levels of CNS from the spinal cord of CNS from the spinal cord ((sacral mic. centerssacral mic. centers)) to the to the brain stem (brain stem (pontine mic. Centerspontine mic. Centers))

Cerebellum, basal ganglion, limbic system, thalamus and Cerebellum, basal ganglion, limbic system, thalamus and hypothalamus, and cerebral cortexhypothalamus, and cerebral cortex

bladder is unique among other visceral organs bladder is unique among other visceral organs its function is under control of somatic and autonomic n. systemits function is under control of somatic and autonomic n. system

Beside acetylcholine & NE, other neurotransmitters Beside acetylcholine & NE, other neurotransmitters involved during bladder stimulationinvolved during bladder stimulation PG substance P, Opioid, vasoactive intestinal peptide, PG substance P, Opioid, vasoactive intestinal peptide,

neuropeptide Yneuropeptide Y

Page 17: Voiding Dysfunction              in Children

Transitory Detrusor-Sphincter Transitory Detrusor-Sphincter

Discoordination in InfancyDiscoordination in Infancy Studies showed that all children may Studies showed that all children may transiently transiently

display some degree of abnormal bladder-sphincter display some degree of abnormal bladder-sphincter functionfunction

Urodynamic findings show association of Urodynamic findings show association of high high voiding pressuresvoiding pressures and and interruption of flowinterruption of flow BUT BUT nono impairment of overall bladder emptingimpairment of overall bladder empting

This type of dysfunction resolved with a period of This type of dysfunction resolved with a period of successful toilet training, transient or intermittent, successful toilet training, transient or intermittent, and does not persistand does not persist

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Non-Neuropathic Non-Neuropathic Bladder Sphincter Bladder Sphincter

Dysfunction Dysfunction in Childrenin Children

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Reported Reported 15 %15 % of 6-year old children suffer from of 6-year old children suffer from Non-Neuogenic B.S.DysfunctionNon-Neuogenic B.S.Dysfunction

Dysfunctional voiding mayDysfunctional voiding may starts with detrusor instability with sphincter & pelvic floor starts with detrusor instability with sphincter & pelvic floor

overactivityoveractivity then develops gradually fractionated voiding with then develops gradually fractionated voiding with

increasing PVRincreasing PVR Finally, develops bladder decompensation and the lazy Finally, develops bladder decompensation and the lazy

bladder syndrome bladder syndrome

Distinction between Neuropathic & Non-Distinction between Neuropathic & Non-neuropathic bladder dysfunctions may not be clearneuropathic bladder dysfunctions may not be clear

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The term non-neuropathic is based purely on the fact The term non-neuropathic is based purely on the fact that that no obvious and identifiable neurologic lesions no obvious and identifiable neurologic lesions can be identifiedcan be identified

In adults, lower urinary tract function has been well In adults, lower urinary tract function has been well understood and standardization of terminology has been understood and standardization of terminology has been establishedestablished

In contrast, neural control over the bladder-sphincter unit In contrast, neural control over the bladder-sphincter unit in children is age dependent and is much more variable in children is age dependent and is much more variable and complex and complex

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Etiologic Classification of Etiologic Classification of Bladder DysfunctionBladder Dysfunction

Derangement of Nervous ControlDerangement of Nervous Control::

Congenital malformation of CNSCongenital malformation of CNS, e.g.: , e.g.: myelomeningocele, spina bifida, caudal regression myelomeningocele, spina bifida, caudal regression

synd., tethered cordsynd., tethered cord Developmental disturbancesDevelopmental disturbances, e.g.,:, e.g.,:

Mental retardation, dysfunctional voiding, urge synd.Mental retardation, dysfunctional voiding, urge synd. Acquired conditionsAcquired conditions, e.g.:, e.g.:

CP, progressive degenerative diseases of CNS, CP, progressive degenerative diseases of CNS, transverse myelitis, MS, vascular malformations, transverse myelitis, MS, vascular malformations, trauma of spinal cordtrauma of spinal cord

Page 22: Voiding Dysfunction              in Children

Disorders of Detrusor & Sphincteric Muscle function:Disorders of Detrusor & Sphincteric Muscle function: Congenital conditionsCongenital conditions

Muscular dystophy, neuronal dyplasiaMuscular dystophy, neuronal dyplasia Acquired conditionsAcquired conditions

Chronic bladder distension, fibrosis of detrusor & bladder wallChronic bladder distension, fibrosis of detrusor & bladder wall

Structural abnormalitiesStructural abnormalities Congenital conditionsCongenital conditions

Bladder extrophy, epispadias, cloacal anomoly, uretroceles, PUV, Bladder extrophy, epispadias, cloacal anomoly, uretroceles, PUV, prune belly syndromeprune belly syndrome

Acquired conditionsAcquired conditions Traumatic stricture, damage to sphincter or urethraTraumatic stricture, damage to sphincter or urethra

Other unclassified conditionsOther unclassified conditions Giggle incontinenceGiggle incontinence Hinman syndromeHinman syndrome Ochoa syndrome (urofacial syndrome)Ochoa syndrome (urofacial syndrome)

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Functional classification of bladder Functional classification of bladder dysfunctiondysfunction

based on functional state of the bladder-based on functional state of the bladder-sphincter complex with respect of sphincter complex with respect of detrusor activitydetrusor activity

bladder sensationbladder sensation

bladder compliance and function bladder compliance and function

urethral function urethral function

during the filling & voiding phase of CMGduring the filling & voiding phase of CMG

Page 24: Voiding Dysfunction              in Children

During the filling phase:During the filling phase:

Detrusor activityDetrusor activity Normal or stableNormal or stable

OveractiveOveractive : phasic involuntary detrusor contractions : phasic involuntary detrusor contractions which occur spontaneously or provoked by alteration which occur spontaneously or provoked by alteration of posture, coughing, walking, jumpingof posture, coughing, walking, jumping

UnstableUnstable: contraction : contraction unrelatedunrelated to underlying neurologic to underlying neurologic disorderdisorder

Detrusor hyperreflexiaDetrusor hyperreflexia : overactivity : overactivity relatedrelated disturbance of disturbance of neural control mechanismneural control mechanism

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Bladder sensation during filling phaseBladder sensation during filling phase:: Normal / hypersensitive / hyposensitive / absentNormal / hypersensitive / hyposensitive / absent

Bladder capacityBladder capacity normal/ / high / lownormal/ / high / low

ComplianceCompliance Normal / high / lowNormal / high / low

Urethral functionUrethral function Normal / incompetentNormal / incompetent

Page 26: Voiding Dysfunction              in Children

During the voiding phaseDuring the voiding phase

Detrusor activityDetrusor activity Normal: Voiding achieved by voluntarily initiated Normal: Voiding achieved by voluntarily initiated

detrusor contractions that is sustained and detrusor contractions that is sustained and cannot cannot usually suppressed voluntarily until after 4 year oldusually suppressed voluntarily until after 4 year old

UnderactiveUnderactive AcontractileAcontractile

Urethral functionUrethral function NormalNormal obstructiveobstructive

Page 27: Voiding Dysfunction              in Children

Bladder-Sphincter dysfunction Bladder-Sphincter dysfunction during fillingduring filling

Overactive (unstable) bladder, urge Overactive (unstable) bladder, urge syndrome, urge incontinencesyndrome, urge incontinence

Traditionally the infant bladder has been Traditionally the infant bladder has been described as unstable or uninhibited described as unstable or uninhibited

Recent studies showed that bladder is normally Recent studies showed that bladder is normally quiescent and stable even in newbornquiescent and stable even in newborn

Clinically, the condition of (unstable bladder) is Clinically, the condition of (unstable bladder) is best exhibited by best exhibited by URGE SYNDROMEURGE SYNDROME with or with or without urge incontinencewithout urge incontinence

Page 28: Voiding Dysfunction              in Children

Urge syndromeUrge syndrome characterized frequent attacks of sudden characterized frequent attacks of sudden and imperative sensations of urge due to detrusor and imperative sensations of urge due to detrusor overactivity during filling (girls>boys)overactivity during filling (girls>boys)

The unstable contractions are often counteracted by The unstable contractions are often counteracted by voluntary contractions in the pelvic floor muscles to voluntary contractions in the pelvic floor muscles to externally compress the urethra (hold maneuvers) exhibited externally compress the urethra (hold maneuvers) exhibited as squatting in many casesas squatting in many cases

Urge incontinenceUrge incontinence consists of small quantities of urine consists of small quantities of urine

loss loss More in afternoon when the child plays and is not alert enough to More in afternoon when the child plays and is not alert enough to

contract the pelvic floor in response to the urge sensation contract the pelvic floor in response to the urge sensation

Page 29: Voiding Dysfunction              in Children

Functional urinary incontinenceFunctional urinary incontinence

Defined as involuntary loss of urine due to failure of Defined as involuntary loss of urine due to failure of control of bladder sphincter unit, frequent enough to control of bladder sphincter unit, frequent enough to cause social or hygienic problem with the absence of cause social or hygienic problem with the absence of underlying anatomic causesunderlying anatomic causes

Stress incontinenceStress incontinence represents involuntary leakage represents involuntary leakage of urine occurring when the intravesical pressure of urine occurring when the intravesical pressure exceeds the bladder outlet or urethral pressure in the exceeds the bladder outlet or urethral pressure in the absence of measurable detrusor contractions absence of measurable detrusor contractions

Unlike adults, true stress incont. Extremlely uncommon in Unlike adults, true stress incont. Extremlely uncommon in neurologically normal children and generally not associated neurologically normal children and generally not associated with abnormal CMGwith abnormal CMG

Page 30: Voiding Dysfunction              in Children

Giggle incontinence:Giggle incontinence:

Involuntary and typically unpredictable wetting during Involuntary and typically unpredictable wetting during giggling or laughtergiggling or laughter

In contrast to stress incontinence, it produces much In contrast to stress incontinence, it produces much larger volume of urine leak amounting to complete larger volume of urine leak amounting to complete bladder emptying bladder emptying

CMG may be normal or occasionally demonstrate CMG may be normal or occasionally demonstrate some detrusor overactivitysome detrusor overactivity

Rx is difficult bt sometimes a course of anticholinergic Rx is difficult bt sometimes a course of anticholinergic drugs may help drugs may help

Some suggested that itSome suggested that it’’s centrally mediated and s centrally mediated and hereditary disorder that may respond to CNS hereditary disorder that may respond to CNS stimulants as stimulants as methylphenidate methylphenidate

Page 31: Voiding Dysfunction              in Children

Bladder-Sphincter dysfunction Bladder-Sphincter dysfunction during bladder emptyingduring bladder emptying

Dysfunctional voidingDysfunctional voiding

Characterized by incomplete relaxation or Characterized by incomplete relaxation or overactivity of the pelvic floor muscles during overactivity of the pelvic floor muscles during voiding voiding

Can manifest in different patterns depending on Can manifest in different patterns depending on the degree of outflow obstructionthe degree of outflow obstruction caused and caused and the status of the detrusor activitythe status of the detrusor activity

Page 32: Voiding Dysfunction              in Children

Staccato and fractionated voidingStaccato and fractionated voiding In staccato voiding the urinary stream is often delayed after In staccato voiding the urinary stream is often delayed after

the onset of detrusor contraction and is typically interrupted the onset of detrusor contraction and is typically interrupted resulting in a few small squirts of urine passed in quick resulting in a few small squirts of urine passed in quick sensationsensation

Interrupted voiding caused by periodic bursts of pelvic floor Interrupted voiding caused by periodic bursts of pelvic floor muscle activities during micturition resulting in muscle activities during micturition resulting in characteristic abrupt elevation of voiding pressure characteristic abrupt elevation of voiding pressure coinciding with paradoxical cessation of urinary flowcoinciding with paradoxical cessation of urinary flow

Flow time usually prolonged and bladder emptying Flow time usually prolonged and bladder emptying incomplete incomplete

Page 33: Voiding Dysfunction              in Children

Fractionated voidingFractionated voiding is characterized by is characterized by infrequent & incomplete emptying secondary to infrequent & incomplete emptying secondary to detrusor inactivity detrusor inactivity

Micturition occurs in several small discontinuous fractions Micturition occurs in several small discontinuous fractions due to poor detrusor contractionsdue to poor detrusor contractions

Significant PVRSignificant PVR

Abdominal straining usually evident to improve emptyingAbdominal straining usually evident to improve emptying

Straining paradoxically counteracted by reflex increase in Straining paradoxically counteracted by reflex increase in pelvic floor muscles that is triggered by increase in pelvic floor muscles that is triggered by increase in intravesical pressure intravesical pressure

Page 34: Voiding Dysfunction              in Children

Infrequent voiding and (lazy bladder) syndromeInfrequent voiding and (lazy bladder) syndrome Described together as they represent a spectrum of Described together as they represent a spectrum of

diseases that are more commonly occurs in diseases that are more commonly occurs in girlsgirls

The lazy bladder syndrome is generally regarded as the The lazy bladder syndrome is generally regarded as the endpoint of long standing dysfunctional voiding endpoint of long standing dysfunctional voiding occurring in a fully decompensated systemoccurring in a fully decompensated system

Due to chronic functional outflow obst., there is gradual Due to chronic functional outflow obst., there is gradual deterioration in detrusor contractility and emptying deterioration in detrusor contractility and emptying efficiencyefficiency

PVR & bladder capacity increase progressively with PVR & bladder capacity increase progressively with inefficient emptying inefficient emptying

Page 35: Voiding Dysfunction              in Children

Bcs urge sensation is either absent or diminished, Bcs urge sensation is either absent or diminished, voiding is very infrequent and occasionally the child may voiding is very infrequent and occasionally the child may not void for 8-10 hrs or longer if engaged in activity not void for 8-10 hrs or longer if engaged in activity

Typical presentation, the mother always complains that Typical presentation, the mother always complains that the child never voids unless told to do so the child never voids unless told to do so

Other presentationsOther presentations Recurrent UTI, Overflow incontinence, constipationRecurrent UTI, Overflow incontinence, constipation

CMG findingsCMG findings Large bladder capacity, very high compliance on filling, absent Large bladder capacity, very high compliance on filling, absent

detrusor contractions, voiding associated with increased detrusor contractions, voiding associated with increased abdominal pressuresabdominal pressures

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HinmanHinman’’s Syndrome & Occult Neuropathic s Syndrome & Occult Neuropathic bladderbladder Different names:Different names:

Nonneurogenic neurogenic bladder / subclinical neurogenic bladder Nonneurogenic neurogenic bladder / subclinical neurogenic bladder / Hinman syndrome / occult neuropathic bladder/ Hinman syndrome / occult neuropathic bladder

Acquired form of bladder-sphincteric dysfunction in childrenAcquired form of bladder-sphincteric dysfunction in children

characterized by a combination of characterized by a combination of bladder bladder decompensation with incontinence, poor emptying, and decompensation with incontinence, poor emptying, and recurrent UTIrecurrent UTI

Most children have significant bowel dysfunctionMost children have significant bowel dysfunction

Has all the clinical & CMG features of neuropathic Has all the clinical & CMG features of neuropathic dysfunction but NO neuologic pathologydysfunction but NO neuologic pathology

CMG shows marked sphincteric overactivity with abrupt CMG shows marked sphincteric overactivity with abrupt contractions of pelvic floor contractions of pelvic floor

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Ochoa (urofacial) syndrome:Ochoa (urofacial) syndrome: Children have all classic features of dysfunctional Children have all classic features of dysfunctional

voiding, including urinary incontinence, recurrent voiding, including urinary incontinence, recurrent UTI, constipation, reflux, Upper tract damage + UTI, constipation, reflux, Upper tract damage + peculiar painful or apparently crying facial peculiar painful or apparently crying facial expression during smilingexpression during smiling

Autosomal recessive, located on chromosome Autosomal recessive, located on chromosome 1010

CMG shows sustained contraction of external CMG shows sustained contraction of external sphincter during voidingsphincter during voiding

Page 38: Voiding Dysfunction              in Children

Of 66 children reported by Ochoa:Of 66 children reported by Ochoa: 33% renal functional impairment33% renal functional impairment 26% HTN26% HTN 24% ESRD24% ESRD

Bcs neural ganglion controlling the facial Bcs neural ganglion controlling the facial muscles are situated very close to the pontine muscles are situated very close to the pontine micturition centersmicturition centers A small genetically predetermined congenital A small genetically predetermined congenital

neurologic lesion in this area may be responsible neurologic lesion in this area may be responsible for both the peculiar facial expression & bladder for both the peculiar facial expression & bladder dysfunctiondysfunction

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Postvoid dibblingPostvoid dibbling Involuntary leakage of urine immediately after voidingInvoluntary leakage of urine immediately after voiding

Refers to post toilet trained girls who dribble soon after Refers to post toilet trained girls who dribble soon after standing up after a void and otherwise normal with no standing up after a void and otherwise normal with no other urinary symptomsother urinary symptoms

May be result of May be result of vesicovaginal refluxvesicovaginal reflux where urine is where urine is trapped in the vagina during voiding & once the child trapped in the vagina during voiding & once the child stands, the urine dribble outstands, the urine dribble out

When in doubt, can be confirmed by MCUGWhen in doubt, can be confirmed by MCUG

Harmless, tends to resolve with ageHarmless, tends to resolve with age

Child may be taught to empty her vagina by simply Child may be taught to empty her vagina by simply voiding with her thigh apart & leaning forward after voiding with her thigh apart & leaning forward after voiding before getting upvoiding before getting up

Page 40: Voiding Dysfunction              in Children

Dysfunctional Elimination Syndrome, Dysfunctional Elimination Syndrome, Constipation, & Bladder Dysfunction Constipation, & Bladder Dysfunction

DES refers to broad spectrum of functional DES refers to broad spectrum of functional disturbances that may affect the urinary tract disturbances that may affect the urinary tract including that of functional bowel disturbances including that of functional bowel disturbances

The close proximity of the rectum to posterior The close proximity of the rectum to posterior bladder wall make it possible that bladder wall make it possible that gross distension of the rectum by impacted feces can gross distension of the rectum by impacted feces can

result in mechanical compression of the bladder & result in mechanical compression of the bladder &

bladder neck leading to urinary obstructionbladder neck leading to urinary obstruction

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Classified as : Classified as :

Functional disorder of fillingFunctional disorder of filling Overactive, overdisteded, insensate bladder, may be Overactive, overdisteded, insensate bladder, may be

associated with fecal impaction or rectal distension with associated with fecal impaction or rectal distension with infrequent call to stoolinfrequent call to stool

Functional disorder of emptyingFunctional disorder of emptying Over recruitment of pelvic floor activity during voiding Over recruitment of pelvic floor activity during voiding

causing interrupted / incomplete emptying, with defecation causing interrupted / incomplete emptying, with defecation difficulties due to nonrelaxation of puborectalis or pain on difficulties due to nonrelaxation of puborectalis or pain on defecationdefecation

Page 42: Voiding Dysfunction              in Children

DES influenced the clinical outcome of ureteric DES influenced the clinical outcome of ureteric reimplantation surgery for VURreimplantation surgery for VUR

Children with constipation had the highest likehood of Children with constipation had the highest likehood of developing breakthrough UTI & requires surgerydeveloping breakthrough UTI & requires surgery

DES had an adverse effect on the rate of spontaneous DES had an adverse effect on the rate of spontaneous reflux resolution requiring an average reflux resolution requiring an average 1.6 years1.6 years longer to longer to outgrow reflux than in children without DESoutgrow reflux than in children without DES

After successful Rx of constipationAfter successful Rx of constipation 89%89% of those with daytime incontinence and of those with daytime incontinence and 63%63% of nighttime of nighttime

incontinence became dry incontinence became dry Loening-Baucke et al (1997)Loening-Baucke et al (1997)

Management of the underlying dysfunction should be Management of the underlying dysfunction should be

given priority in the treatment protocol of children with given priority in the treatment protocol of children with conditions such as VUR / incontinence / UTI bsc conditions such as VUR / incontinence / UTI bsc successful Rx may significantly improve outcomesuccessful Rx may significantly improve outcome

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Relationship Betw. Bladder Sphincter Relationship Betw. Bladder Sphincter Dysfunction, VUR, & Recurrent UTI Dysfunction, VUR, & Recurrent UTI

Impairment in the function of lower tract often Impairment in the function of lower tract often coexists with recurrent UTI & VUR without coexists with recurrent UTI & VUR without neurologic pathologyneurologic pathology

The most common abnormalities of lower tract The most common abnormalities of lower tract coexist with VUR are coexist with VUR are Detrusor overactivityDetrusor overactivity Uncoordinated detrusor sphincter during voidingUncoordinated detrusor sphincter during voiding

Reflux may be worsened by detrusor instabilityReflux may be worsened by detrusor instability

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Studies showed that infants with UTI & VUR Studies showed that infants with UTI & VUR have high prevalence of high voiding detrusor have high prevalence of high voiding detrusor pressurepressure

MaleMale refluxers have higher maximum detrusor refluxers have higher maximum detrusor pressure > femalepressure > female May be due to high urethral resistance of the longer May be due to high urethral resistance of the longer

male urethra and smaller urethral meatus with male urethra and smaller urethral meatus with anatomic difference in the external urethral sphincteranatomic difference in the external urethral sphincter

Spontaneous resolution of VUR may be delayed Spontaneous resolution of VUR may be delayed in presence of abnormal dynamics of the in presence of abnormal dynamics of the bladderbladder

Successful Rx of underlying bladder dysfunction Successful Rx of underlying bladder dysfunction result in marked increase in the rate of result in marked increase in the rate of spontaneous resolution of reflux & recurrent UTIspontaneous resolution of reflux & recurrent UTI

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Evaluation of Non-Neurogenic Evaluation of Non-Neurogenic Bladder Sphincter Dysfunction Bladder Sphincter Dysfunction

HistoryHistory Majority present after toilet training with symptoms of Majority present after toilet training with symptoms of

nighttime / daytime urinary incontinence or both nighttime / daytime urinary incontinence or both May present earlier with UTI or VURMay present earlier with UTI or VUR Hx should include questions to exclude neurologic & Hx should include questions to exclude neurologic &

congenital abnormalitiescongenital abnormalities Bowel dysfunction can coexist in the form of Bowel dysfunction can coexist in the form of

encorpresis, constipation and fecal impactionencorpresis, constipation and fecal impaction Urinary Hx should include symptoms of storage & Urinary Hx should include symptoms of storage &

voiding of urinevoiding of urine

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Physical examination:Physical examination: Usually normalUsually normal Careful examination is required Careful examination is required Occasionally, palpable bladder may be foundOccasionally, palpable bladder may be found External genitalia examinationExternal genitalia examination Abnormalities of lower spineAbnormalities of lower spine

Neural tubal defectNeural tubal defect Asymmetrical gluteal foldsAsymmetrical gluteal folds Hairy patchHairy patch Dermovascular malformationDermovascular malformation Lipomatous abnormality of sacral regionLipomatous abnormality of sacral region

Rectal exam. may reveal impacted stoolRectal exam. may reveal impacted stool

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LaboratoryLaboratory

Not routinely requiredNot routinely required

Urine analysis may be performed to R/O Urine analysis may be performed to R/O bacteruria & glucosuriabacteruria & glucosuria

Serum & urine osmolarity may be looked in case Serum & urine osmolarity may be looked in case of nocturnal enuresis of nocturnal enuresis

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UltrasoundUltrasound

11stst line investigation line investigation

Simple, reliable, available, & noninvasive toolSimple, reliable, available, & noninvasive tool

Provides anatomical & functional problemsProvides anatomical & functional problems

Recently used to measure bladder parameters used in Recently used to measure bladder parameters used in calculating bladder volume & wall thickness index (BVWI)calculating bladder volume & wall thickness index (BVWI)

BVWI classified into normal / thick / thinBVWI classified into normal / thick / thin

Studies showed these classifications corresponded Studies showed these classifications corresponded closely to CMG findings of bladder dysfunctionsclosely to CMG findings of bladder dysfunctions

This classification can act as reliable tool to guide for This classification can act as reliable tool to guide for further invasive investigationsfurther invasive investigations

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Other imaging studiesOther imaging studies:: Radiologic examination of the spine may be Radiologic examination of the spine may be

necessary to exclude neuologic causesnecessary to exclude neuologic causes MCUGMCUG may be needed to R/O VUR & to assess may be needed to R/O VUR & to assess

the status of the urethrathe status of the urethra

Urodynamics studiesUrodynamics studies:: To describe the physiologic parameters To describe the physiologic parameters

involved in bladder mechanics during filling & involved in bladder mechanics during filling & voiding voiding

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Urodynamics (cont.)Urodynamics (cont.)

UroflowUroflow

In children, normal flow rate are different from adults In children, normal flow rate are different from adults

Usually there is poor correlation betw. Qmax & outflow Usually there is poor correlation betw. Qmax & outflow resistanceresistance

Bcs the detrusor is able to exert much stronger contractions to Bcs the detrusor is able to exert much stronger contractions to counteract any increased resistancecounteract any increased resistance

Pattern of flow curve is importantPattern of flow curve is important

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Patterns of uroflow curve:Patterns of uroflow curve:

Bell shapeBell shape : normal : normal Tower shapeTower shape : produced by explosive voiding : produced by explosive voiding

contractions which is seen in overactive bladdercontractions which is seen in overactive bladder Low plateauLow plateau : representative of outlet obstruction : representative of outlet obstruction Staccato patternStaccato pattern : seen with sphincteric overactivity : seen with sphincteric overactivity

during voiding with peak and throughs throughtout during voiding with peak and throughs throughtout voiding voiding

Interrupted voidingInterrupted voiding : seen in a contractile or : seen in a contractile or underactive bladder underactive bladder

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Urodynamics (cont.)Urodynamics (cont.)

Conventional fill urodynamic studiesConventional fill urodynamic studies Bladder catheter introduced transurethrally or Bladder catheter introduced transurethrally or

suprapubicallysuprapubically The use of SPC has been suggested as a better The use of SPC has been suggested as a better

alternative to transurethral catheterization alternative to transurethral catheterization

Natural fill urodynamic studiesNatural fill urodynamic studies The child is asked to drink to allow the bladder to fill up on its The child is asked to drink to allow the bladder to fill up on its

own rateown rate Artificial filling may inhibit the detrusor response and attenuate Artificial filling may inhibit the detrusor response and attenuate

its maximum contractile potential, making detrusor instability its maximum contractile potential, making detrusor instability less pronounced & undetectableless pronounced & undetectable

Natural fill cystometry is the preferred technique in childrenNatural fill cystometry is the preferred technique in children The combined use of artificial & natural filling CMG is helpful to The combined use of artificial & natural filling CMG is helpful to

accurately delineate the underlying bladder dysfunctionaccurately delineate the underlying bladder dysfunction

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Ambulatory urodynamic studiesAmbulatory urodynamic studies

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Management of Non-Neuropathic Management of Non-Neuropathic Bladder-Sphincter DysfunctionBladder-Sphincter Dysfunction

Behavior Modification & standard UrotherapyBehavior Modification & standard Urotherapy UrotherapyUrotherapy is a nonpharmacologic nonsurgical is a nonpharmacologic nonsurgical

combination of cognitive, behavioural, & physical therapy combination of cognitive, behavioural, & physical therapy to normalize micturiton pattern & prevent functional to normalize micturiton pattern & prevent functional disturbances of lower tractdisturbances of lower tract

Children & parents education on proper voiding Children & parents education on proper voiding mechanicsmechanics

Instructions how and when to voidInstructions how and when to void

Teaching children correct positions during voidingTeaching children correct positions during voiding

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Behavior Modification & standard Urotherapy Behavior Modification & standard Urotherapy (cont.)(cont.)

Teaching how to relax the pelvic floor and Teaching how to relax the pelvic floor and avoid strainingavoid straining

Modification of drinking and voiding habits to Modification of drinking and voiding habits to include proper hydration with timed voidinginclude proper hydration with timed voiding

Assessment of their bowel function Assessment of their bowel function

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Urotherapy. Pelvic floor rehabilitation with real Urotherapy. Pelvic floor rehabilitation with real time biofeedback monitoringtime biofeedback monitoring

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Biofeedback and pelvic floor rehabilitationBiofeedback and pelvic floor rehabilitation

Biofeedback is based on the concept of building self Biofeedback is based on the concept of building self perception on detrusor contractions and pelvic floor perception on detrusor contractions and pelvic floor relaxation in the ptrelaxation in the pt

By combining uroflow with real time monitoring, child is By combining uroflow with real time monitoring, child is able to see how well he / she is voiding able to see how well he / she is voiding

Biofeedback is proven to be highly effective either on Biofeedback is proven to be highly effective either on its own or in combination with standard urotherapyits own or in combination with standard urotherapy

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Urotherapy. Biofeedback with real time uroflowmetryUrotherapy. Biofeedback with real time uroflowmetry

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NeuromodulationNeuromodulation

Recent studies reported that transcutaneous Recent studies reported that transcutaneous electrical nerve stimulation (TENS) is simple, electrical nerve stimulation (TENS) is simple, cost effective, noninvasive treatment modalitycost effective, noninvasive treatment modality

The use of low frequency electrical current to The use of low frequency electrical current to

inhibit detrusor activity in adults is commoninhibit detrusor activity in adults is common Appears to modulate excitatory & inhibitory Appears to modulate excitatory & inhibitory

components of bladder control components of bladder control

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Bowel managementBowel management

Principles include rectal emptying of impacted stool and Principles include rectal emptying of impacted stool and maintenance of regular soft stoolsmaintenance of regular soft stools

Oral laxatives or rectal enemasOral laxatives or rectal enemas

Dietary modificationDietary modification

Correct toilet posture & correct recruitment of Correct toilet posture & correct recruitment of abdominal muscles in the defecation processabdominal muscles in the defecation process

Parental education Parental education

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MedicationsMedications Antimuscarinic agentsAntimuscarinic agents

These agents are the gold standard in Rx of These agents are the gold standard in Rx of overactive bladderoveractive bladder

Muscarinic receptors are found in detrusor muscleMuscarinic receptors are found in detrusor muscle

Bladder contractions are initiated by stimulation of Bladder contractions are initiated by stimulation of these receptors with the release of Ach from these receptors with the release of Ach from cholinergic n.cholinergic n.

Main action of antimuscarinic drugs on M1 & M3 Main action of antimuscarinic drugs on M1 & M3 receptor subtypes, thought to be responsible of receptor subtypes, thought to be responsible of detrusor overactivitydetrusor overactivity

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Antimuscarinic agents (cont.)Antimuscarinic agents (cont.)

Antimuscarinic, such as Antimuscarinic, such as OxybutyninOxybutynin, act by , act by reducing the frequency & intensity of involuntary reducing the frequency & intensity of involuntary contractions causing increase in functional bladder contractions causing increase in functional bladder capacitycapacity

The nonselective pattern of activity and penetration The nonselective pattern of activity and penetration of BBB are known to induce systemic & central S/E of BBB are known to induce systemic & central S/E

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Alpha adrenergic blockersAlpha adrenergic blockers The density of the noradrenergic nerves The density of the noradrenergic nerves

increases markedly toward the bladder neck increases markedly toward the bladder neck particularly in particularly in malesmales

The normal response to NE is relaxation & The normal response to NE is relaxation & contraction of the bladder neckcontraction of the bladder neck

Alpha blockers used in pts with Alpha blockers used in pts with evidence of evidence of

bladder neck dysfunctionbladder neck dysfunction for relaxation of for relaxation of bladder neck bladder neck

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Other medicationsOther medications Tricyclic antidepressentsTricyclic antidepressents

E.g. E.g. impiramineimpiramine

Effective in increasing urine storage by decreasing Effective in increasing urine storage by decreasing detrusor contractility & increasing outlet resistancedetrusor contractility & increasing outlet resistance

Precise mechanism is not clearPrecise mechanism is not clear

Possible effect on bladder by inhibition of NE Possible effect on bladder by inhibition of NE reuptake, producing alpha adrenergic stimulationreuptake, producing alpha adrenergic stimulation

Associated with high S/E Associated with high S/E

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Beta-Adrenergic agonists Beta-Adrenergic agonists Can cause significant increase in bladder Can cause significant increase in bladder

capacity capacity Bt can also cause significant CVS S/EBt can also cause significant CVS S/E

Parasympathicomimetics / Ca Parasympathicomimetics / Ca antagonists / K Ch. Openers / PG antagonists / K Ch. Openers / PG inhibitorsinhibitors Rarely used in children due to unfavorable S/E Rarely used in children due to unfavorable S/E

or to lack of proven efficacyor to lack of proven efficacy

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Clean intermittent CatheterizationClean intermittent Catheterization

Necessary in pts with decompensated bladders or lazy Necessary in pts with decompensated bladders or lazy bladder syndromebladder syndrome

Regular emptying of the bladder to achieve low Regular emptying of the bladder to achieve low pressure emptying which improves detrusor pressure emptying which improves detrusor contractility & bladder emptying functioncontractility & bladder emptying function

Some of these children may be able to eventually be Some of these children may be able to eventually be weaned from use of this procedure weaned from use of this procedure

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SurgerySurgery Indicated when conservative management with Indicated when conservative management with

nonpharmacologic & pharmacologic Rx failsnonpharmacologic & pharmacologic Rx fails

Bladder augmentation may be performed to Bladder augmentation may be performed to produce low pressure system with increased produce low pressure system with increased bladder capacity bladder capacity

Augmentation using intestinal segments such Augmentation using intestinal segments such as colon / ileum / stomachas colon / ileum / stomach

Augmentation has its metabolic complications Augmentation has its metabolic complications

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Surgery (cont.)Surgery (cont.)

Surgical means has been employed to reduce Surgical means has been employed to reduce urethral / sphincteric pressure as alternative to urethral / sphincteric pressure as alternative to alpha blockersalpha blockers

Ballon dilatation of bladder neck & botulinum A Ballon dilatation of bladder neck & botulinum A toxin injection into the sphinctertoxin injection into the sphincter has been used has been used Requires repeated attempts Requires repeated attempts ? Long term effect & efficacy ? Long term effect & efficacy

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Neuropathic Neuropathic Dysfunction Dysfunction

of of Lower Urinary Tract Lower Urinary Tract

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Neuropathic Dysfunction of Lower Urinary TractNeuropathic Dysfunction of Lower Urinary Tract

Neurospinal dysraphismsNeurospinal dysraphisms MyelodysplasiaMyelodysplasia Lipomeningocele & other spinal dysraphismsLipomeningocele & other spinal dysraphisms

Sacral agenesis Sacral agenesis Central nervous system insultsCentral nervous system insults

Cerebral palsyCerebral palsy

Traumatic injuries to the spineTraumatic injuries to the spine

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Neurospinal dysraphismsNeurospinal dysraphisms

MyelodysplasiaMyelodysplasia The most common cause of neurogenic The most common cause of neurogenic

bladder dysfunction in childrenbladder dysfunction in children Formation of spinal cord & vertebral column Formation of spinal cord & vertebral column

begins at 18begins at 18thth day of gestation day of gestation Closure of canal proceeds in caudal direction Closure of canal proceeds in caudal direction Closure complete at 35 daysClosure complete at 35 days ? Mechanism that results in closure and wt ? Mechanism that results in closure and wt

produces dysraphism produces dysraphism

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Incidence reported 1 / 1000 birthsIncidence reported 1 / 1000 births Incidence increases with more than one member Incidence increases with more than one member

affectedaffected The medical Research Council Vitamin Study The medical Research Council Vitamin Study

Group recommends that women of childbearing Group recommends that women of childbearing age take 4mg/d of folic acid beginning at least age take 4mg/d of folic acid beginning at least 2/12 before pregnancy2/12 before pregnancy

Folate deficiency can lead to myelodysplastic Folate deficiency can lead to myelodysplastic abnormality abnormality

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Relationship Incidence

General population 0.7-1.0

Mother with one affected child 20-50

Mother with two affected children 100

Patient with myelodysplasia 40

Mother older than 35 years 30

Sister of mother with affected child 10

Sister of father with affected child 3

Nephew who is affected 2

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Myelodysplasia Myelodysplasia various abnormal conditions of the vertebral column various abnormal conditions of the vertebral column

that affect spinal cord functionthat affect spinal cord function Meningocele Meningocele

Occurs when just the meninges ( no neual elements ) Occurs when just the meninges ( no neual elements ) extend beyond the confines of vertebral canalextend beyond the confines of vertebral canal

Myelomeningocele Myelomeningocele Neural tissue, either nerve root or portions of spinal Neural tissue, either nerve root or portions of spinal

cord has evaginated with the meningocelecord has evaginated with the meningocele LipomyelomeningoceleLipomyelomeningocele

Fatty tissue has developed with the cord structures and both Fatty tissue has developed with the cord structures and both extend with the protruding sac extend with the protruding sac

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Myelomeningocele accounts for > 90% of all open Myelomeningocele accounts for > 90% of all open spinal dysraphic statesspinal dysraphic states

Most spinal defects at lumber vertebraeMost spinal defects at lumber vertebrae

Location Incidence (%)

Cervical-high thoracic 2

Low thoracic 5

Lumbar 26

Lumbosacral 47

Sacral 20

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Page 78: Voiding Dysfunction              in Children

Usually, the meningocele is made of flimsy covering Usually, the meningocele is made of flimsy covering of transparent tissueof transparent tissue

It may be opened with CSF leakIt may be opened with CSF leak For this reason, urgent repair is necessary For this reason, urgent repair is necessary Sterile precautions showed be applied after birth till time of Sterile precautions showed be applied after birth till time of

repairrepair

85 %85 % associated with Arnold-Chiari malformation associated with Arnold-Chiari malformation Cerebral tonsils herniate down through the foramen Cerebral tonsils herniate down through the foramen

magnummagnum Herniation causes obstruction of the 4Herniation causes obstruction of the 4thth ventricle & ventricle &

preventing the CSF from entering the subarchnoid spacepreventing the CSF from entering the subarchnoid space

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ItIt’’s possible that leakage of CSF from the open spinal s possible that leakage of CSF from the open spinal column accounts for herniation of posterior brain stem down column accounts for herniation of posterior brain stem down the foramen magnum hydrocephalusthe foramen magnum hydrocephalus

Neurologic lesion produced by this condition variesNeurologic lesion produced by this condition varies Depending on what neural element have everted with the Depending on what neural element have everted with the

meningocele sacmeningocele sac

The bony vertebral level often provides little or no clue to The bony vertebral level often provides little or no clue to the exact neurologic lesion producedthe exact neurologic lesion produced

10%10% of newborn with MM exhibit no abnormality in CMG of newborn with MM exhibit no abnormality in CMG

24%24% of children with normal lower tract at birth, develop of children with normal lower tract at birth, develop upper motor neuron changes over time upper motor neuron changes over time

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Newborn assessmentNewborn assessment Renal U/SRenal U/S with measurement of post void residual is performed as with measurement of post void residual is performed as

early as possible after birth early as possible after birth Before / after closure of spinal defect Before / after closure of spinal defect

CMGCMG is delayed until it is delayed until it’’s safe to transport the child to the s safe to transport the child to the urodynamic suit and place him on the back or side for the urodynamic suit and place him on the back or side for the testtest

If the infant cannot empty the bladder after spontaneous void If the infant cannot empty the bladder after spontaneous void or with Crede maneuver, or with Crede maneuver, CICCIC is begun even before CMG is is begun even before CMG is donedone

If If Crede maneuverCrede maneuver is effective in emptying the bladder, it is effective in emptying the bladder, it’’s s performed at regular basis instead of CIC until lower tract is performed at regular basis instead of CIC until lower tract is

fully evaluatedfully evaluated

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The normal bladder capacity in newborn is The normal bladder capacity in newborn is 10-15ml10-15ml Residual urine of <5 ml is acceptableResidual urine of <5 ml is acceptable

Other tests should be performed Other tests should be performed UrinanalysisUrinanalysis & & cultureculture Serum Serum creatininecreatinine Careful Careful neurologic examination of LLneurologic examination of LL

Once spinal closure has healed sufficientlyOnce spinal closure has healed sufficiently Renal U/S & renal scanRenal U/S & renal scan for reassessment of upper tract for reassessment of upper tract VCUGVCUG CMGCMG

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FindingsFindings 15-20%15-20% of newborns have abnormal urinary tract on of newborns have abnormal urinary tract on

radiological exam. when first evaluatedradiological exam. when first evaluated 3%3% have hydro 2ndry to spinal shock, probably from spinal canal have hydro 2ndry to spinal shock, probably from spinal canal

closureclosure 15%15% have abnormalities that develop in utero as result of abnormal have abnormalities that develop in utero as result of abnormal

lower tract due to outlet obstructionlower tract due to outlet obstruction

CMG in newborn showed that CMG in newborn showed that 63%63% have bladder have bladder contractionscontractions

A combination of bladder contractility & external sphincter A combination of bladder contractility & external sphincter activity results in activity results in synergic synergic (26%)(26%) Dyssynergic with / out poor detrusor complianceDyssynergic with / out poor detrusor compliance (37%) (37%) Complete denervation Complete denervation (36%)(36%)

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This categorization of lower tract function has been This categorization of lower tract function has been useful bcs it reveals useful bcs it reveals Which child is at risk for urinary tract changesWhich child is at risk for urinary tract changes Who should be treated prophylacticallyWho should be treated prophylactically Who needs close surveillenceWho needs close surveillence Who can be monitored at great intervalsWho can be monitored at great intervals

It appears that outlet obstruction is a major It appears that outlet obstruction is a major contributor to the development of urinary tract contributor to the development of urinary tract deteriorationdeterioration

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RecommendationsRecommendations

Expectant management revealed that infants with outlet Expectant management revealed that infants with outlet obst. in the form of DSD are at considerable risk for urinary obst. in the form of DSD are at considerable risk for urinary tract deteriorationtract deterioration

These pts should be treated prophylacticallyThese pts should be treated prophylactically

CIC alone OR in combination with anticholinergic when CIC alone OR in combination with anticholinergic when Detrusor filling pressures > 40 cm H2ODetrusor filling pressures > 40 cm H2O Voiding pressures > 80-100 cm H2OVoiding pressures > 80-100 cm H2O Resulted in an incidence of urinary tract deterioration Resulted in an incidence of urinary tract deterioration

of only 8-10% of only 8-10%

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Oxybutynin hydrochlorideOxybutynin hydrochloride administered in a administered in a dose of 1 mg / year of age BID dose of 1 mg / year of age BID

In neonates & children < 1year, dose < 1mg & In neonates & children < 1year, dose < 1mg & increase proportionally as the age reaches 1 yearincrease proportionally as the age reaches 1 year

On rare occasions when overactive or poor On rare occasions when overactive or poor compliant bladder fails to respond, augmentation compliant bladder fails to respond, augmentation cystoplasty may be neededcystoplasty may be needed

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Neurologic findings & recommendationsNeurologic findings & recommendations

Neurologic lesion in myelodysplasia is a dynamic Neurologic lesion in myelodysplasia is a dynamic disease process in which changes take place disease process in which changes take place throughout childhoodthroughout childhood

When a change is noted on neurologic, orthopedic, or When a change is noted on neurologic, orthopedic, or urodynamic assessment, radiologic investigation of the urodynamic assessment, radiologic investigation of the CNS often revealsCNS often reveals Tethering of the spinal cord Tethering of the spinal cord A syrinx or hydromyelia of the cordA syrinx or hydromyelia of the cord Increased intracranial pressure due shunt malfunctionIncreased intracranial pressure due shunt malfunction Partial herniation of the brain stem and cerebellem Partial herniation of the brain stem and cerebellem

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MRIMRI is the test of choice as it reveals anatomic is the test of choice as it reveals anatomic details of the spinal column & CNSdetails of the spinal column & CNS

Sequential urodynamicsSequential urodynamics testing on yearly basis testing on yearly basis beginning in the newborn period and continuing beginning in the newborn period and continuing until until 5 yrs old 5 yrs old

It may be necessary to repeat CMG if upper tract It may be necessary to repeat CMG if upper tract dilates 2ndry to impaired drainage from a poor dilates 2ndry to impaired drainage from a poor compliant detrusor compliant detrusor

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Sphincter Activity Recommended Tests Frequency

Intact-synergic Postvoid residual volume q 4 mo

  IVP or renal echo q 12 mo

  UDS q 12 mo

Intact-dyssynergic† IVP or renal echo q 12 mo

  UDS q 12 mo

  VCUG or RNC‡ q 12 mo

Partial denervation Postvoid residual volume q 4 mo

  IVP or renal echo q 12 mo

  UDS§ q 12 mo

  VCUG or RNC‡ q 12 mo

Complete denervation Postvoid residual volume q 6 mo

  Renal echo q 12 mo

Surveillance in infants with myelodysplasia Surveillance in infants with myelodysplasia

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Management of VURManagement of VUR

VUR occurs in VUR occurs in 3-5 %3-5 % of newborns with of newborns with myelodysplasiamyelodysplasia

Usually associated with poor detrusor Usually associated with poor detrusor compliance, detrusor overactivity or DSDcompliance, detrusor overactivity or DSD

If untreated, incidence of VUR in these infants at If untreated, incidence of VUR in these infants at risk increases with time until risk increases with time until 30-40%30-40% affected by affected by 5 years of age5 years of age

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VUR grade 1-3 who void spontaneously or who have VUR grade 1-3 who void spontaneously or who have complete lesions with little or no outlet resistance with complete lesions with little or no outlet resistance with good bladder emptyinggood bladder emptying Prophylactic Abx onlyProphylactic Abx only

High grade refluxHigh grade reflux CIC CIC to ensure complete emptyingto ensure complete emptying

Children who cannot empty their bladder spontaneously Children who cannot empty their bladder spontaneously regardless of the graderegardless of the grade Treated with Treated with CICCIC

Children with poor detrusor compliance with / out hydroChildren with poor detrusor compliance with / out hydro To add To add anticholenergic drugsanticholenergic drugs to lower intravesical pressure and to lower intravesical pressure and

ensure adequate upper tract decompensation ensure adequate upper tract decompensation

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Bacteriuria occurs in 56% of children with CIC & Bacteriuria occurs in 56% of children with CIC & not harmfulnot harmful

Except in presence of high grade reflux Except in presence of high grade reflux

Symptomatic UTI & renal scarring rarely occurs in lesser grades of Symptomatic UTI & renal scarring rarely occurs in lesser grades of refluxreflux

Crede maneuver should be avoided in children with Crede maneuver should be avoided in children with refluxreflux, especially those with reactive external sphincter, especially those with reactive external sphincter

In results in a reflex response in external sphincter that increases In results in a reflex response in external sphincter that increases urethral resistance & raises the pressure needed to expel urine from urethral resistance & raises the pressure needed to expel urine from bladderbladder

Aggravating the degree of reflux & accentuating its watter hammer Aggravating the degree of reflux & accentuating its watter hammer effect on kidneys effect on kidneys

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VesicostomyVesicostomy drainage rarely required today drainage rarely required today but indicated in but indicated in

Infants who has severe reflux that CIC & Infants who has severe reflux that CIC & anticholenergic fail to improve upper tract anticholenergic fail to improve upper tract drainage drainage

Parents cannot adapt to catheterization programParents cannot adapt to catheterization program

Who are not good candidates for augmentation Who are not good candidates for augmentation cystoplastycystoplasty

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The indications of antireflux surgery are not very The indications of antireflux surgery are not very different from those with normal bladderdifferent from those with normal bladder

Recurrent symptomatic UTI while receiving Recurrent symptomatic UTI while receiving adequate Abx therapy & appropriate CIC techniquesadequate Abx therapy & appropriate CIC techniques

Persistent hydro despite effective emptying of the Persistent hydro despite effective emptying of the bladder & lowering of intravesical pressurebladder & lowering of intravesical pressure

Severe reflux with anatomic abnormality at the UVJSevere reflux with anatomic abnormality at the UVJ

Reflux that persists into puberty Reflux that persists into puberty

Presence of reflux in any child undergoing surgery Presence of reflux in any child undergoing surgery to increase outlet resistanceto increase outlet resistance

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Antireflux surgery can be very effective in children with Antireflux surgery can be very effective in children with neurogenic bladder dysfunction as long as itneurogenic bladder dysfunction as long as it’’s combined s combined with measures to ensure complete bladder emptying with measures to ensure complete bladder emptying

Since the advent of CIC, success rate for antireflux surgery Since the advent of CIC, success rate for antireflux surgery approached 95 %approached 95 %

The endoscopic injection of Deflux has altered the The endoscopic injection of Deflux has altered the management of reflux in children with MMmanagement of reflux in children with MM

Its long term effects are yet to be appreciated Its long term effects are yet to be appreciated

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ContinenceContinence Initial attempts at achieving continence include Initial attempts at achieving continence include

CIC & drug therapy to maintain low intravesical CIC & drug therapy to maintain low intravesical pressure pressure

DrugsDrugs Glycopyrrolate (Robinol)Glycopyrrolate (Robinol) : most potent oral : most potent oral

anticholenergic drug available today same other S/Eanticholenergic drug available today same other S/E Tolteradine (Detrol):Tolteradine (Detrol): newly approved, equally effective newly approved, equally effective

as oxybutynin with fewer S/Eas oxybutynin with fewer S/E Hyoscyamine (levsin)Hyoscyamine (levsin) : potency less, fewer S/E : potency less, fewer S/E Intravesical oxybutyninIntravesical oxybutynin : fewer S/E compared to oral : fewer S/E compared to oral

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Botulinum roxin ABotulinum roxin A injected into the detrusor injected into the detrusor muscle has been effectively used muscle has been effectively used Paralyzes the bladder for varying period of time Paralyzes the bladder for varying period of time May become viable Rx in the futureMay become viable Rx in the future

Alpha sympathomimetic agentsAlpha sympathomimetic agents If CMG reveals that urethral resistance is inadequate to If CMG reveals that urethral resistance is inadequate to

maintain continencemaintain continence PhenylpropanolaminePhenylpropanolamine is the most effective agent is the most effective agent

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SurgerySurgery

Viable option when drug therapy fails to achieve Viable option when drug therapy fails to achieve continencecontinence

Generally intervention is delayed till 5 yrs oldGenerally intervention is delayed till 5 yrs old

Enterocystoplasty using sigmoid, cecum & small Enterocystoplasty using sigmoid, cecum & small intestineintestine

If bladder neck or urethral resistance is insufficient to If bladder neck or urethral resistance is insufficient to allow adequate storage, bladder neck reconstruction is allow adequate storage, bladder neck reconstruction is consideredconsidered

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Currently, Deflux injections at the bladder neck Currently, Deflux injections at the bladder neck are being advocated are being advocated enhances outlet resistanceenhances outlet resistance Alternative to bladder neck reconstructionAlternative to bladder neck reconstruction No long term dataNo long term data

Continent urinary diversion with closure of Continent urinary diversion with closure of bladder neck has been used to provide better bladder neck has been used to provide better quality of life for intractable urethral quality of life for intractable urethral incompetenceincompetence

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SexualitySexuality

In several studies, In several studies, 28-40%28-40% of MM had one or more sexual of MM had one or more sexual encountersencounters

All of them had a desire to marry & to bear childrenAll of them had a desire to marry & to bear children

In one study, In one study, 72%72% of male subjects have erection, 2/3 of male subjects have erection, 2/3 were able to ejeculatewere able to ejeculate

Other studies revealed Other studies revealed 70-80%70-80% of MM women were able to of MM women were able to become pregnantbecome pregnant

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The degree of sexuality is inversely proportional The degree of sexuality is inversely proportional to the level of neuologic dysfunctionto the level of neuologic dysfunction

Boys reach puberty at age similar to normal boysBoys reach puberty at age similar to normal boys

In MM Girls, breast development & menarche In MM Girls, breast development & menarche start 2 yrs earlier than usual normal girlsstart 2 yrs earlier than usual normal girls

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Bowel functionBowel function The external anal sphincter in innervated by the same The external anal sphincter in innervated by the same

nerves that modulate the external urethral sphincternerves that modulate the external urethral sphincter

The internal anal sphincter is influenced by more The internal anal sphincter is influenced by more proximal n. from sympathatic nervous systemproximal n. from sympathatic nervous system

The internal sphincter reflexively relaxes in response to The internal sphincter reflexively relaxes in response to anal distensionanal distension

Consequently, bowel incontinence is frequently Consequently, bowel incontinence is frequently unpredictableunpredictable

Incontinence not associated with the attainment of Incontinence not associated with the attainment of urinary incontinenceurinary incontinence

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Lipomeningocele & other Lipomeningocele & other Spinal dysraphismSpinal dysraphism

Group of congenital defects that affects the Group of congenital defects that affects the formation of spinal column but formation of spinal column but do not result do not result in open vertebral canalin open vertebral canal

Incidence of lipomeningocele in families Incidence of lipomeningocele in families 0.043%0.043%

Lesion have no obvious outward signsLesion have no obvious outward signs

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Lipomeningocele

Intradural lipoma

Diastematomyelia

Tight filum terminale

Dermoid cyst/sinus

Aberrant nerve roots

Anterior sacral meningocele

Cauda equina tumor

Types of occult spinal dysraphisms

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>90%>90% have cutaneous abnormalities overlying the have cutaneous abnormalities overlying the spinesspines Small dimpleSmall dimple Skin tag to a tuft of hairSkin tag to a tuft of hair Dermal vascular malformationDermal vascular malformation Very noticeable subcutaneous lipomaVery noticeable subcutaneous lipoma

Asymmetrically curving gluteal cleftAsymmetrically curving gluteal cleft

Careful inspection of the legs may show high arched foot / Careful inspection of the legs may show high arched foot / alterations in the configuration of the toes / discrepancy in alterations in the configuration of the toes / discrepancy in muscle size / shortness / decreased strength in one leg muscle size / shortness / decreased strength in one leg typically the ankle / gait abnormalitytypically the ankle / gait abnormality

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Small lipomeningocele Hair patch

Dermal vascular malformation

dimple

Abnormal gluteal cleft

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Absent perineal sensation / back pain / Absent perineal sensation / back pain / secondary incontinence may be notedsecondary incontinence may be noted

Abnormal lower tract function in 40-90%Abnormal lower tract function in 40-90% Abnormality increase with ageAbnormality increase with age Difficulty with toilet trainingDifficulty with toilet training Urinary incontinence after period of drynessUrinary incontinence after period of dryness Recurrent UTIRecurrent UTI Fecal soilingFecal soiling

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Majority perfectly normal neurologic examinationMajority perfectly normal neurologic examination

CMGCMG

Abnormal lower tract function in 1/3 of infants < 18/12Abnormal lower tract function in 1/3 of infants < 18/12

Most likely abnormality is UMN lesion characterized by Most likely abnormality is UMN lesion characterized by overactive bladder &/or hyperactive sacral reflexesoveractive bladder &/or hyperactive sacral reflexes

Rarely, mild form of DSDRarely, mild form of DSD

LMN signs occurs in 10% onlyLMN signs occurs in 10% only

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All children > 3 yrs who have not been operated All children > 3 yrs who have not been operated on OR whom occult dysraphism has been lately on OR whom occult dysraphism has been lately diagnosed diagnosed

Have upper or lower lesion or in combination on Have upper or lower lesion or in combination on CMG (92%) within 2 yrsCMG (92%) within 2 yrs

When observed expectantly from infancy after Dx When observed expectantly from infancy after Dx was made, 58% deteriorate was made, 58% deteriorate

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Pathogenesis Pathogenesis

Various occult spinal dysraphic lesions produces different Various occult spinal dysraphic lesions produces different neuologic findings, Reasons:neuologic findings, Reasons:

Compression on the cauda equina or sacral n. roots by Compression on the cauda equina or sacral n. roots by expanding lipoma or lipomeningoceleexpanding lipoma or lipomeningocele

Tension on the cord from tethering 2ndry to differential growth Tension on the cord from tethering 2ndry to differential growth rates in bony vertebrae and neural elements while the lower end rates in bony vertebrae and neural elements while the lower end of the cord is held in place by lipoma or thickened filum terminaleof the cord is held in place by lipoma or thickened filum terminale

Fixation of the split lumbosacral cord by intervertebral bony Fixation of the split lumbosacral cord by intervertebral bony specule or fibrous bandspecule or fibrous band

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Normally, the conus medullaris ends just below Normally, the conus medullaris ends just below the L2 vertebraae at birth and recedes upward to the L2 vertebraae at birth and recedes upward to T12 by adulthoodT12 by adulthood

When the cord does not rise or fixed in place bcs When the cord does not rise or fixed in place bcs of these lesions, ischemic injury may ensueof these lesions, ischemic injury may ensue

Correction of the lesion in infancy result not only Correction of the lesion in infancy result not only in stabilization, bt also in improvement in the in stabilization, bt also in improvement in the neurologic pictures in many instancesneurologic pictures in many instances

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RecommendationsRecommendations MRI MRI Spinal U/S in children < 3/12Spinal U/S in children < 3/12

At this age, vertebral bones have not ossified At this age, vertebral bones have not ossified Useful screening tool for visualization of spinal canalUseful screening tool for visualization of spinal canal

Currently, most NS advocates laminectomy & Currently, most NS advocates laminectomy & removal of the intraspinal process as completely removal of the intraspinal process as completely as possible, without injuring nerve roots or cord to as possible, without injuring nerve roots or cord to release the tether and prevent further injury from release the tether and prevent further injury from subsequent growth subsequent growth

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Sacral AgenesisSacral Agenesis

The absence of part or all of 2 or more lower The absence of part or all of 2 or more lower vertebral bodiesvertebral bodies

Teratogenic factors play a role Teratogenic factors play a role IDDM mothers have 1% of giving birth to a child with IDDM mothers have 1% of giving birth to a child with

sacral agenesissacral agenesis 16% of children with sacral agenesis have a mother 16% of children with sacral agenesis have a mother

with IDDM with IDDM Maternal insulin-Ab complexes noted to cross the Maternal insulin-Ab complexes noted to cross the

placenta placenta

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Deletion of chr.7q36 has a role Deletion of chr.7q36 has a role Maternal drug exposure (Minoxidil) reported to Maternal drug exposure (Minoxidil) reported to

cause sacral agenesiscause sacral agenesis Familial cases if sacral agenesis associated with Familial cases if sacral agenesis associated with

Curarino syndrome Curarino syndrome Presacral massPresacral mass Sacral agenesisSacral agenesis Anorectal malformationAnorectal malformation Deletion in chr.7, leading to HLXB9 genetic mutationDeletion in chr.7, leading to HLXB9 genetic mutation

Association with VACTERL syndrome Association with VACTERL syndrome reportedreported

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Diagnosis of sacral agenesisDiagnosis of sacral agenesis

Presentation bimodal Presentation bimodal ¾ at early infancy ¾ at early infancy Remainder at 4-5 yrs Remainder at 4-5 yrs

can be Dx parentally due to frequent use of U/Scan be Dx parentally due to frequent use of U/S

If not detected prenatally or at birth, Dx is delayedIf not detected prenatally or at birth, Dx is delayed

May present with failed attempts at toilet training May present with failed attempts at toilet training

Sensation intact & lower limb function is normal Sensation intact & lower limb function is normal usuallyusually

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The only clue beside the high index of The only clue beside the high index of suspicion issuspicion is Flattened buttocksFlattened buttocks Low gluteal cleft Low gluteal cleft

Palpation of the coccyx is used to detect Palpation of the coccyx is used to detect absent vertebraeabsent vertebrae

Dx confirmed with a lateral film of lower spineDx confirmed with a lateral film of lower spine

MRI is diagnosticMRI is diagnostic

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Gluteal crease is short and seen only inferiorly

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FindingsFindings CMGCMG

UMNL - 35%UMNL - 35% LMNL - 40%LMNL - 40% No signs of denervation at all - 25%No signs of denervation at all - 25%

UMNLUMNL Overactive detrusorOveractive detrusor Exaggerated sacral reflexesExaggerated sacral reflexes Absence of voluntary control over sphincter functionAbsence of voluntary control over sphincter function DSD no sphincteric deenervationDSD no sphincteric deenervation Bladder is thick wall (or trabeculated), with closed Bladder is thick wall (or trabeculated), with closed

bladder neck on VCUG or U/Sbladder neck on VCUG or U/S

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LMN LMN Acontractile detrusor Acontractile detrusor Partial or complete denervation of external sphincter Partial or complete denervation of external sphincter Diminished or absent sacral reflexesDiminished or absent sacral reflexes Bladder smooth and small with opened bladder neck Bladder smooth and small with opened bladder neck

The presence or absence of bulbocavernous reflex is an The presence or absence of bulbocavernous reflex is an indicator of an UMNL / LMNL respectively indicator of an UMNL / LMNL respectively

UTI present in 75% over timeUTI present in 75% over time

VUR occurs in UMNL (75%) and in LMNL (40%)VUR occurs in UMNL (75%) and in LMNL (40%)

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RecommendationsRecommendations

CMG / Ultrasound / VCUG or nuclear CMG / Ultrasound / VCUG or nuclear

cystographycystography

UMNLUMNL Anticholinergic Anticholinergic +/- CIC+/- CIC If anticholinergic ineffective in controlling If anticholinergic ineffective in controlling

overactive detrusor, augmentation cystoplasty overactive detrusor, augmentation cystoplasty required required

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LMNLLMNL CIC & alpha sympathomimetics drugs for CIC & alpha sympathomimetics drugs for

those who cannot empty the bladders or stay those who cannot empty the bladders or stay dry between CICdry between CIC

Injection of bulking agents Injection of bulking agents Artificial urinary sphincter implantationArtificial urinary sphincter implantation

+ Rx bowel dysfunction+ Rx bowel dysfunction

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Associated conditions with sacral agenesisAssociated conditions with sacral agenesis

Imperforate anusImperforate anus Alone or part of anomalies ( VATER / VACTERL )Alone or part of anomalies ( VATER / VACTERL ) Male > female 1.5:1Male > female 1.5:1 Sacral agenesis occurs with spectrum of hindgut Sacral agenesis occurs with spectrum of hindgut

abnormalities in Currarino syndromeabnormalities in Currarino syndrome Associattion with fistula to the lower tract is commonAssociattion with fistula to the lower tract is common Most common findings on CMGMost common findings on CMG

UMNL with overactive bladderUMNL with overactive bladder And or DSD And or DSD

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CNS insultsCNS insults

Cerebral palsyCerebral palsy Nonprogressive injury of the brain occurring in Nonprogressive injury of the brain occurring in

the perinatal period that produces either a the perinatal period that produces either a neuromuscular disability, a specific symptom neuromuscular disability, a specific symptom complex, or cerebral dysfunctioncomplex, or cerebral dysfunction

Incidence is 1.5 / 1000 birthsIncidence is 1.5 / 1000 births Lesions classified according to which Lesions classified according to which

extremities involved and wt kind of dysfunctionextremities involved and wt kind of dysfunction Spastic diplegia is the most commonSpastic diplegia is the most common

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Female Male

High High

  Anorectal agenesis   Anorectal agenesis

    With rectovaginal fistula    Without fistula

    With rectourethral (prostatic) fistula

  Rectal atresia     Without fistula

Intermediate   Rectal atresia

  Rectovestibular fistula Intermediate

  Rectovaginal fistula   Rectovestibular urethral fistula

  Anal agenesis without fistula   Anal agenesis without fistula

Low Low

  Anovestibular fistula   Anocutaneous fistula

  Anocutaneous fistula   Anal stenosis

  Anal stenosis Rare malformation

Cloacal malformation  

Rare malformation  

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Most children has total urinary controlMost children has total urinary control

23.5% has persistent incontinence 23.5% has persistent incontinence

The presence of incontinence is often The presence of incontinence is often related to the extent of physical impairmentrelated to the extent of physical impairment

Abnormal bladder & urethral sphincter Abnormal bladder & urethral sphincter

function was found in almost all function was found in almost all

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Type Number %

Upper motor neuron lesion 49 86

Mixed upper +lower motor neuron lesion 5 9.5

Incomplete lower motor neuron lesion 1 1.5

No urodynamic lesion 2 3

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Type of Lesion No. of Patients

Upper motor neuron  

  Uninhibited contractions 35

  Detrusor sphincter dyssynergy 7

  Hyperactive sacral reflexes 6

  No voluntary control 3

  Small-capacity bladder 2

  Hypertonia 2

Lower motor neuron  

  Excessive polyphasia 5

  ↑ Amplitude +↑ duration potentials 4

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Factor UMN (No. of Patients) LMN (No. of Patients)

Prematurity 10 1

Respiratory distress/arrest/apnea

9 2

Neonatal seizures 5 -

Infection 5 -

Traumatic birth 5 -

Congenital hydrocephalus 3 -

Placenta previa/abruption 2 2

Hypoglycemia ± seizures 2 -

Intracranial hemorrhage 2 -

Cyanosis at birth 1 3

No specific factor noted 15 -

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RecommendationsRecommendations

Overactive bladder treated with anticholinergicOveractive bladder treated with anticholinergic

+/- CIC +/- CIC

Dorsal rhizotomy in selected group of children Dorsal rhizotomy in selected group of children who fail to respond to less invasive measureswho fail to respond to less invasive measures

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Traumatic injuries to the spineTraumatic injuries to the spine

Rarely encountered in childrenRarely encountered in children

Pts with upper thoracic or cervical lesion are likely Pts with upper thoracic or cervical lesion are likely to have autonomic dysreflexia with spontaneous to have autonomic dysreflexia with spontaneous discharge of alpha1 stimulants during bladder filling discharge of alpha1 stimulants during bladder filling & with contraction of detrusor& with contraction of detrusor

Monitoring of BP & availability of alpha blockers are Monitoring of BP & availability of alpha blockers are mandatory during VCUG or CMG mandatory during VCUG or CMG

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ManagementManagement If retention immediately after trauma, foley If retention immediately after trauma, foley

catheter is inserted and kept in place as short catheter is inserted and kept in place as short time as possibletime as possible Until the pt is stable and aseptic CIC can be started Until the pt is stable and aseptic CIC can be started

safely on regular basis safely on regular basis

The goal is balanced voiding at pressures < 40 The goal is balanced voiding at pressures < 40 cmH2O, which reduces the 30% risk of urinary cmH2O, which reduces the 30% risk of urinary tract deterioration seen in poorly managed ptstract deterioration seen in poorly managed pts

If cannot be achieved, CIC is continuedIf cannot be achieved, CIC is continued

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Anticholinergic drugs (P.O. or intavesically) Anticholinergic drugs (P.O. or intavesically) added as they are effective in reducing added as they are effective in reducing overactive bladderoveractive bladder

Alternative RxAlternative Rx

External urethral sphincterotomyExternal urethral sphincterotomy Urethral stent placementUrethral stent placement Injection of botulinum A toxin (Botox) into the external Injection of botulinum A toxin (Botox) into the external

sphincter sphincter Continent catheterizable abdominal urinary stoma in Continent catheterizable abdominal urinary stoma in

pts with low cervical or upper thoracic lesions who pts with low cervical or upper thoracic lesions who cannot easily catheterize themselvescannot easily catheterize themselves

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