Transcript Voiding Dysfunction in Children

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
 Detrusor consists of meshwork of smooth m. which has the
ability to elicit maximal active tension over a wide range of
length
 This allows the bladder to be filled at low pressure
(compliance)
 Reservoir function determined by activity of detrusor m. and
bladder outlet
 Bladder sphincter plays a major role in urinary continence by
closure of bladder neck & proximal urethra
 Literature suggests that
 Immature detrusor-sphincter coordination, manifested as detrusor
hypercontractility and interrupted voiding, commonly occurs in 1st 1-2
years causing a degree of functional bladder outflow obstruction
(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.)
 Parasymp. N. fibers run in pelvic n. (S2-S4) to supply the
pelvic and vesical plexuses before entering the bladder
 Symp. n. arises from (T10-L2) to inferior mesenteric
ganglion
bladder
hypogastric n. to the pelvic plexus &
 There is also symp. innervation from T10-L2 supplying the
detrusor and urethral sphincter
 The somatic nervous system (pudendal n.) supplies the
periurethral pelvic floor muscles
 The sensory & motor n. carried by all 3 nerves innervate
the bladder and urethral sphincter
 Symp. supply causes
 Detrusor relaxation (B receptors)
 Smooth m contraction at trigone & bladder neck (alpha receptors)
 Parasymp. supply causes detrusor contraction
(muscarinic receptors)
 Pudendal supply causes striated sphincter contraction
 Within the spinal cord, information from bladder afferents
integrated with other sources and projected to brain stem
centers to coordinate with mic. centers
Development of Normal Bladder Function
and Micturition Control
 CMG studies on normal infants showed
 bladder function in young children is very
different from adults
 During the 1st 2-3 years of life
 There is progressive development from initially
indiscriminate infants voiding pattern to more
socially conscious and voluntary (adult) type of
micturition
 The natural evolution of bladder control
entails nervous system & depends on:
 Progressive increase in bladder functional
storage capacity
 Maturation of voluntary control over the urethral
striated muscle sphincter
 Development of direct volitional control over the
bladder sphincter unit, so that the child can
voluntary initiate or inhibit micturition reflex
Change in bladder function parameters
 Voiding frequency
 During the 3rd trimester, the fetus voids at rate
30 times/24 hrs
 Immediately after birth, this drops for the 1st
days only
 Increases again after 1st 1/52 to reach a peak
by 2-4 /52 to an average once/hour
 This rate declines to 10-15 times/day at 6-12
mo.
 8-10 times/day by 2-3 years
 This reduction in voiding frequency
observed during the 1st years of life related
mainly to an increase in bladder capacity
parallel to body growth
 By the age of 12, voiding pattern is very
similar to that in adult ( 4-6 voids / day )
 Bladder capacity
 An adequate reservoir function of urine storage is
necessary to meet the increase rate of urine production
and decreased voiding frequency in the growing child
Bladder capacity can be estimated
For young infants:
Bladder capacity (ml) = 38 + 2.5 * age (mo)
For children:
Bladder capacity (ml) = (age [yr] + 2) * 30 koff’s
formula
Bladder capacity (ml) = 30 + (age [yr] * 30 Hijalma’s
formula
 In parallel to increase in bladder capacity, the
mean voided volume of each micturation
increases with age
 CMG studies showed that most infants with
incomplete maturation of det.- sphin. coordination
before the age 1, are still able to have satisfactory
emptying (>80)
 There are limited studies on detr. pressure at
voiding in infants due techn. difficulties
 Limited data documented higher maximum detr.
pressure with mict. than in adults
 Male infants voided significantly higher pressure than
females (mean Pdetmax 118 vs. 75cm H20, P<.03)
 Studies showed that high detrusor pressures
noted during micturition were mainly observed
only during the 1st year of life & decreased
progressively with age
Evolution of Normal Micturition Control
 Traditionally, it has been assumed that micturition occurs
by simple spinal reflex with no mediation by higher neural
centers
 Recent studies showed that even in full term fetuses and
newborns, mict. Is modulated by higher centers
 Further extensive modulation occurs during the postnatal
period
 During the 2nd & 3rd year of life, there is a progressive
development toward a socially conscious continence and
a more voluntary (adult) type mict.
 The final steps usually achieved at 3-4 yrs
 Most develop the adult pattern of urinary control & will be dry day
& night
 The child has learned to inhibit a mict. Reflex and
postpone voiding and voluntarily initiate mict. At socially
acceptable time & place
 Neurologic control of normal mic. occurs at different levels
of CNS from the spinal cord (sacral mic. centers) to the
brain stem (pontine mic. Centers)
 Cerebellum, basal ganglion, limbic system, thalamus and
hypothalamus, and cerebral cortex
 bladder is unique among other visceral organs
 its function is under control of somatic and autonomic n. system
 Beside acetylcholine & NE, other neurotransmitters
involved during bladder stimulation
 PG substance P, Opioid, vasoactive intestinal peptide,
neuropeptide Y
Transitory Detrusor-Sphincter
Discoordination in Infancy
 Studies showed that all children may transiently
display some degree of abnormal bladder-sphincter
function
 Urodynamic findings show association of high
voiding pressures and interruption of flow BUT no
impairment of overall bladder empting
 This type of dysfunction resolved with a period of
successful toilet training, transient or intermittent,
and does not persist
Non-Neuropathic
Bladder Sphincter
Dysfunction
in Children
 Reported 15 % of 6-year old children suffer from
Non-Neuogenic B.S.Dysfunction
 Dysfunctional voiding may
 starts with detrusor instability with sphincter & pelvic floor
overactivity
 then develops gradually fractionated voiding with
increasing PVR
 Finally, develops bladder decompensation and the lazy
bladder syndrome
 Distinction between Neuropathic & Nonneuropathic bladder dysfunctions may not be clear
 The term non-neuropathic is based purely on the fact
that no obvious and identifiable neurologic lesions
can be identified
 In adults, lower urinary tract function has been well
understood and standardization of terminology has been
established
 In contrast, neural control over the bladder-sphincter unit
in children is age dependent and is much more variable
and complex
Etiologic Classification of
Bladder Dysfunction
 Derangement of Nervous Control:
 Congenital malformation of CNS, e.g.:
 myelomeningocele, spina bifida, caudal regression
synd., tethered cord
 Developmental disturbances, e.g.,:
 Mental retardation, dysfunctional voiding, urge synd.
 Acquired conditions, e.g.:
 CP, progressive degenerative diseases of CNS,
transverse myelitis, MS, vascular malformations,
trauma of spinal cord
 Disorders of Detrusor & Sphincteric Muscle function:
 Congenital conditions
 Muscular dystophy, neuronal dyplasia
 Acquired conditions
 Chronic bladder distension, fibrosis of detrusor & bladder wall
 Structural abnormalities
 Congenital conditions
 Bladder extrophy, epispadias, cloacal anomoly, uretroceles, PUV,
prune belly syndrome
 Acquired conditions
 Traumatic stricture, damage to sphincter or urethra
 Other unclassified conditions
 Giggle incontinence
 Hinman syndrome
 Ochoa syndrome (urofacial syndrome)
Functional classification of bladder
dysfunction
based on functional state of the bladdersphincter complex with respect of
detrusor activity
bladder sensation
bladder compliance and function
urethral function
during the filling & voiding phase of CMG
 During the filling phase:
 Detrusor activity
 Normal or stable
 Overactive : phasic involuntary detrusor contractions
which occur spontaneously or provoked by alteration
of posture, coughing, walking, jumping
 Unstable: contraction unrelated to underlying neurologic
disorder
 Detrusor hyperreflexia : overactivity related disturbance of
neural control mechanism
 Bladder sensation during filling phase:
 Normal / hypersensitive / hyposensitive / absent
 Bladder capacity
 normal/ / high / low
 Compliance
 Normal / high / low
 Urethral function
 Normal / incompetent
 During the voiding phase
 Detrusor activity
 Normal: Voiding achieved by voluntarily initiated
detrusor contractions that is sustained and cannot
usually suppressed voluntarily until after 4 year old
 Underactive
 Acontractile
 Urethral function
 Normal
 obstructive
Bladder-Sphincter dysfunction
during filling
 Overactive (unstable) bladder, urge
syndrome, urge incontinence
 Traditionally the infant bladder has been
described as unstable or uninhibited
 Recent studies showed that bladder is normally
quiescent and stable even in newborn
 Clinically, the condition of (unstable bladder) is
best exhibited by URGE SYNDROME with or
without urge incontinence
 Urge syndrome characterized frequent attacks of sudden
and imperative sensations of urge due to detrusor
overactivity during filling (girls>boys)
 The unstable contractions are often counteracted by
voluntary contractions in the pelvic floor muscles to
externally compress the urethra (hold maneuvers) exhibited
as squatting in many cases
 Urge incontinence consists of small quantities of urine
loss
 More in afternoon when the child plays and is not alert enough to
contract the pelvic floor in response to the urge sensation
 Functional urinary incontinence
 Defined as involuntary loss of urine due to failure of
control of bladder sphincter unit, frequent enough to
cause social or hygienic problem with the absence of
underlying anatomic causes
 Stress incontinence represents involuntary leakage
of urine occurring when the intravesical pressure
exceeds the bladder outlet or urethral pressure in the
absence of measurable detrusor contractions
 Unlike adults, true stress incont. Extremlely uncommon in
neurologically normal children and generally not associated
with abnormal CMG
 Giggle incontinence:
 Involuntary and typically unpredictable wetting during
giggling or laughter
 In contrast to stress incontinence, it produces much
larger volume of urine leak amounting to complete
bladder emptying
 CMG may be normal or occasionally demonstrate
some detrusor overactivity
 Rx is difficult bt sometimes a course of anticholinergic
drugs may help
 Some suggested that it’s centrally mediated and
hereditary disorder that may respond to CNS
stimulants as methylphenidate
Bladder-Sphincter dysfunction
during bladder emptying
 Dysfunctional voiding
 Characterized by incomplete relaxation or
overactivity of the pelvic floor muscles during
voiding
 Can manifest in different patterns depending on
the degree of outflow obstruction caused and
the status of the detrusor activity
 Staccato and fractionated voiding
 In staccato voiding the urinary stream is often delayed after
the onset of detrusor contraction and is typically interrupted
resulting in a few small squirts of urine passed in quick
sensation
 Interrupted voiding caused by periodic bursts of pelvic floor
muscle activities during micturition resulting in
characteristic abrupt elevation of voiding pressure
coinciding with paradoxical cessation of urinary flow
 Flow time usually prolonged and bladder emptying
incomplete
 Fractionated voiding is characterized by
infrequent & incomplete emptying secondary to
detrusor inactivity
 Micturition occurs in several small discontinuous fractions
due to poor detrusor contractions
 Significant PVR
 Abdominal straining usually evident to improve emptying
 Straining paradoxically counteracted by reflex increase in
pelvic floor muscles that is triggered by increase in
intravesical pressure
 Infrequent voiding and (lazy bladder) syndrome
 Described together as they represent a spectrum of
diseases that are more commonly occurs in girls
 The lazy bladder syndrome is generally regarded as the
endpoint of long standing dysfunctional voiding
occurring in a fully decompensated system
 Due to chronic functional outflow obst., there is gradual
deterioration in detrusor contractility and emptying
efficiency
 PVR & bladder capacity increase progressively with
inefficient emptying
 Bcs urge sensation is either absent or diminished,
voiding is very infrequent and occasionally the child may
not void for 8-10 hrs or longer if engaged in activity
 Typical presentation, the mother always complains that
the child never voids unless told to do so
 Other presentations
 Recurrent UTI, Overflow incontinence, constipation
 CMG findings
 Large bladder capacity, very high compliance on filling, absent
detrusor contractions, voiding associated with increased
abdominal pressures
 Hinman’s Syndrome & Occult Neuropathic
bladder
 Different names:
 Nonneurogenic neurogenic bladder / subclinical neurogenic bladder
/ Hinman syndrome / occult neuropathic bladder
 Acquired form of bladder-sphincteric dysfunction in children
 characterized by a combination of bladder
decompensation with incontinence, poor emptying, and
recurrent UTI
 Most children have significant bowel dysfunction
 Has all the clinical & CMG features of neuropathic
dysfunction but NO neuologic pathology
 CMG shows marked sphincteric overactivity with abrupt
contractions of pelvic floor
 Ochoa (urofacial) syndrome:
 Children have all classic features of dysfunctional
voiding, including urinary incontinence, recurrent
UTI, constipation, reflux, Upper tract damage +
peculiar painful or apparently crying facial
expression during smiling
 Autosomal recessive, located on chromosome 10
 CMG shows sustained contraction of external
sphincter during voiding
 Of 66 children reported by Ochoa:
 33% renal functional impairment
 26% HTN
 24% ESRD
 Bcs neural ganglion controlling the facial
muscles are situated very close to the pontine
micturition centers
 A small genetically predetermined congenital
neurologic lesion in this area may be responsible
for both the peculiar facial expression & bladder
dysfunction
 Postvoid dibbling
 Involuntary leakage of urine immediately after voiding
 Refers to post toilet trained girls who dribble soon after
standing up after a void and otherwise normal with no
other urinary symptoms
 May be result of vesicovaginal reflux where urine is
trapped in the vagina during voiding & once the child
stands, the urine dribble out
 When in doubt, can be confirmed by MCUG
 Harmless, tends to resolve with age
 Child may be taught to empty her vagina by simply
voiding with her thigh apart & leaning forward after
voiding before getting up
Dysfunctional Elimination Syndrome,
Constipation, & Bladder Dysfunction
 DES refers to broad spectrum of functional
disturbances that may affect the urinary tract
including that of functional bowel disturbances
 The close proximity of the rectum to posterior
bladder wall make it possible that
 gross distension of the rectum by impacted feces can
result in mechanical compression of the bladder &
bladder neck leading to urinary obstruction
 Classified as :
 Functional disorder of filling
 Overactive, overdisteded, insensate bladder, may be
associated with fecal impaction or rectal distension with
infrequent call to stool
 Functional disorder of emptying
 Over recruitment of pelvic floor activity during voiding causing
interrupted / incomplete emptying, with defecation difficulties
due to nonrelaxation of puborectalis or pain on defecation
 DES influenced the clinical outcome of ureteric
reimplantation surgery for VUR
 Children with constipation had the highest likehood of
developing breakthrough UTI & requires surgery
 DES had an adverse effect on the rate of spontaneous
reflux resolution requiring an average 1.6 years longer
to outgrow reflux than in children without DES
 After successful Rx of constipation
 89% of those with daytime incontinence and 63% of nighttime
incontinence became dry
Loening-Baucke et al (1997)
 Management of the underlying dysfunction should be
given priority in the treatment protocol of children with
conditions such as VUR / incontinence / UTI bsc
successful Rx may significantly improve outcome
Relationship Betw. Bladder Sphincter
Dysfunction, VUR, & Recurrent UTI
 Impairment in the function of lower tract often
coexists with recurrent UTI & VUR without
neurologic pathology
 The most common abnormalities of lower tract
coexist with VUR are
 Detrusor overactivity
 Uncoordinated detrusor sphincter during voiding
 Reflux may be worsened by detrusor instability
 Studies showed that infants with UTI & VUR
have high prevalence of high voiding detrusor
pressure
 Male refluxers have higher maximum detrusor
pressure > female
 May be due to high urethral resistance of the longer
male urethra and smaller urethral meatus with
anatomic difference in the external urethral sphincter
 Spontaneous resolution of VUR may be delayed
in presence of abnormal dynamics of the
bladder
 Successful Rx of underlying bladder dysfunction
result in marked increase in the rate of
spontaneous resolution of reflux & recurrent UTI
Evaluation of Non-Neurogenic
Bladder Sphincter Dysfunction
 History
 Majority present after toilet training with symptoms of
nighttime / daytime urinary incontinence or both
 May present earlier with UTI or VUR
 Hx should include questions to exclude neurologic &
congenital abnormalities
 Bowel dysfunction can coexist in the form of
encorpresis, constipation and fecal impaction
 Urinary Hx should include symptoms of storage &
voiding of urine
 Physical examination:





Usually normal
Careful examination is required
Occasionally, palpable bladder may be found
External genitalia examination
Abnormalities of lower spine
 Neural tubal defect
 Asymmetrical gluteal folds
 Hairy patch
 Dermovascular malformation
 Lipomatous abnormality of sacral region
 Rectal exam. may reveal impacted stool
 Laboratory
 Not routinely required
 Urine analysis may be performed to R/O
bacteruria & glucosuria
 Serum & urine osmolarity may be looked in case
of nocturnal enuresis
 Ultrasound
 1st line investigation
 Simple, reliable, available, & noninvasive tool
 Provides anatomical & functional problems
 Recently used to measure bladder parameters used in
calculating bladder volume & wall thickness index (BVWI)
 BVWI classified into normal / thick / thin
 Studies showed these classifications corresponded
closely to CMG findings of bladder dysfunctions
 This classification can act as reliable tool to guide for
further invasive investigations
 Other imaging studies:
 Radiologic examination of the spine may be
necessary to exclude neuologic causes
 MCUG may be needed to R/O VUR & to assess
the status of the urethra
 Urodynamics studies:
 To describe the physiologic parameters
involved in bladder mechanics during filling &
voiding
 Urodynamics (cont.)
 Uroflow
 In children, normal flow rate are different from adults
 Usually there is poor correlation betw. Qmax & outflow
resistance
 Bcs the detrusor is able to exert much stronger contractions to
counteract any increased resistance
 Pattern of flow curve is important
 Patterns of uroflow curve:
 Bell shape : normal
 Tower shape : produced by explosive voiding
contractions which is seen in overactive bladder
 Low plateau : representative of outlet obstruction
 Staccato pattern : seen with sphincteric overactivity
during voiding with peak and throughs throughtout
voiding
 Interrupted voiding : seen in a contractile or
underactive bladder
 Urodynamics (cont.)
 Conventional fill urodynamic studies
 Bladder catheter introduced transurethrally or
suprapubically
 The use of SPC has been suggested as a better
alternative to transurethral catheterization
 Natural fill urodynamic studies
 The child is asked to drink to allow the bladder to fill up on its
own rate
 Artificial filling may inhibit the detrusor response and attenuate
its maximum contractile potential, making detrusor instability
less pronounced & undetectable
 Natural fill cystometry is the preferred technique in children
 The combined use of artificial & natural filling CMG is helpful to
accurately delineate the underlying bladder dysfunction
Ambulatory urodynamic studies
Management of Non-Neuropathic
Bladder-Sphincter Dysfunction
 Behavior Modification & standard Urotherapy
 Urotherapy is a nonpharmacologic nonsurgical
combination of cognitive, behavioural, & physical therapy
to normalize micturiton pattern & prevent functional
disturbances of lower tract
 Children & parents education on proper voiding
mechanics
 Instructions how and when to void
 Teaching children correct positions during voiding
Behavior Modification & standard Urotherapy
(cont.)
 Teaching how to relax the pelvic floor and
avoid straining
 Modification of drinking and voiding habits to
include proper hydration with timed voiding
 Assessment of their bowel function
Urotherapy. Pelvic floor rehabilitation with real
time biofeedback monitoring
 Biofeedback and pelvic floor rehabilitation
 Biofeedback is based on the concept of building self
perception on detrusor contractions and pelvic floor
relaxation in the pt
 By combining uroflow with real time monitoring, child is
able to see how well he / she is voiding
 Biofeedback is proven to be highly effective either on
its own or in combination with standard urotherapy
Urotherapy. Biofeedback with real time uroflowmetry
 Neuromodulation
 Recent studies reported that transcutaneous
electrical nerve stimulation (TENS) is simple,
cost effective, noninvasive treatment modality
 The use of low frequency electrical current to
inhibit detrusor activity in adults is common
 Appears to modulate excitatory & inhibitory
components of bladder control
 Bowel management
 Principles include rectal emptying of impacted stool and
maintenance of regular soft stools
 Oral laxatives or rectal enemas
 Dietary modification
 Correct toilet posture & correct recruitment of
abdominal muscles in the defecation process
 Parental education
 Medications
 Antimuscarinic agents
 These agents are the gold standard in Rx of
overactive bladder
 Muscarinic receptors are found in detrusor muscle
 Bladder contractions are initiated by stimulation of
these receptors with the release of Ach from
cholinergic n.
 Main action of antimuscarinic drugs on M1 & M3
receptor subtypes, thought to be responsible of
detrusor overactivity
 Antimuscarinic agents (cont.)
 Antimuscarinic, such as Oxybutynin, act by
reducing the frequency & intensity of involuntary
contractions causing increase in functional bladder
capacity
 The nonselective pattern of activity and penetration
of BBB are known to induce systemic & central S/E
 Alpha adrenergic blockers
 The density of the noradrenergic nerves
increases markedly toward the bladder neck
particularly in males
 The normal response to NE is relaxation &
contraction of the bladder neck
 Alpha blockers used in pts with evidence of
bladder neck dysfunction for relaxation of
bladder neck
 Other medications
 Tricyclic antidepressents
 E.g. impiramine
 Effective in increasing urine storage by decreasing
detrusor contractility & increasing outlet resistance
 Precise mechanism is not clear
 Possible effect on bladder by inhibition of NE
reuptake, producing alpha adrenergic stimulation
 Associated with high S/E
 Beta-Adrenergic agonists
 Can cause significant increase in bladder
capacity
 Bt can also cause significant CVS S/E
 Parasympathicomimetics / Ca antagonists
/ K Ch. Openers / PG inhibitors
 Rarely used in children due to unfavorable S/E
or to lack of proven efficacy
 Clean intermittent Catheterization
 Necessary in pts with decompensated bladders or lazy
bladder syndrome
 Regular emptying of the bladder to achieve low
pressure emptying which improves detrusor
contractility & bladder emptying function
 Some of these children may be able to eventually be
weaned from use of this procedure
 Surgery
 Indicated when conservative management with
nonpharmacologic & pharmacologic Rx fails
 Bladder augmentation may be performed to
produce low pressure system with increased
bladder capacity
 Augmentation using intestinal segments such
as colon / ileum / stomach
 Augmentation has its metabolic complications
 Surgery (cont.)
 Surgical means has been employed to reduce
urethral / sphincteric pressure as alternative to
alpha blockers
 Ballon dilatation of bladder neck & botulinum A
toxin injection into the sphincter has been used
 Requires repeated attempts
 ? Long term effect & efficacy
Neuropathic
Dysfunction
of
Lower Urinary Tract
Neuropathic Dysfunction of Lower Urinary Tract
 Neurospinal dysraphisms
 Myelodysplasia
 Lipomeningocele & other spinal dysraphisms
 Sacral agenesis
 Central nervous system insults
 Cerebral palsy
 Traumatic injuries to the spine
Neurospinal dysraphisms
 Myelodysplasia
 The most common cause of neurogenic
bladder dysfunction in children
 Formation of spinal cord & vertebral column
begins at 18th day of gestation
 Closure of canal proceeds in caudal direction
 Closure complete at 35 days
 ? Mechanism that results in closure and wt
produces dysraphism
 Incidence reported 1 / 1000 births
 Incidence increases with more than one member
affected
 The medical Research Council Vitamin Study
Group recommends that women of childbearing
age take 4mg/d of folic acid beginning at least
2/12 before pregnancy
 Folate deficiency can lead to myelodysplastic
abnormality
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
 Myelodysplasia
 various abnormal conditions of the vertebral column
that affect spinal cord function
 Meningocele
 Occurs when just the meninges ( no neual elements )
extend beyond the confines of vertebral canal
 Myelomeningocele
 Neural tissue, either nerve root or portions of spinal
cord has evaginated with the meningocele
 Lipomyelomeningocele
 Fatty tissue has developed with the cord structures and both
extend with the protruding sac
 Myelomeningocele accounts for > 90% of all open
spinal dysraphic states
 Most spinal defects at lumber vertebrae
Location
Incidence (%)
Cervical-high thoracic
2
Low thoracic
5
Lumbar
26
Lumbosacral
47
Sacral
20
 Usually, the meningocele is made of flimsy covering
of transparent tissue
 It may be opened with CSF leak
 For this reason, urgent repair is necessary
 Sterile precautions showed be applied after birth till time of
repair
 85 % associated with Arnold-Chiari malformation
 Cerebral tonsils herniate down through the foramen
magnum
 Herniation causes obstruction of the 4th ventricle &
preventing the CSF from entering the subarchnoid space
 It’s possible that leakage of CSF from the open spinal
column accounts for herniation of posterior brain stem down
the foramen magnum
hydrocephalus
 Neurologic lesion produced by this condition varies
 Depending on what neural element have everted with the
meningocele sac
 The bony vertebral level often provides little or no clue to
the exact neurologic lesion produced
 10% of newborn with MM exhibit no abnormality in CMG
 24% of children with normal lower tract at birth, develop
upper motor neuron changes over time
 Newborn assessment
 Renal U/S with measurement of post void residual is performed as
early as possible after birth
 Before / after closure of spinal defect
 CMG is delayed until it’s safe to transport the child to the
urodynamic suit and place him on the back or side for the
test
 If the infant cannot empty the bladder after spontaneous void
or with Crede maneuver, CIC is begun even before CMG is
done
 If Crede maneuver is effective in emptying the bladder, it’s
performed at regular basis instead of CIC until lower tract is
fully evaluated
 The normal bladder capacity in newborn is 10-15ml
 Residual urine of <5 ml is acceptable
 Other tests should be performed
 Urinanalysis & culture
 Serum creatinine
 Careful neurologic examination of LL
 Once spinal closure has healed sufficiently
 Renal U/S & renal scan for reassessment of upper tract
 VCUG
 CMG
 Findings
 15-20% of newborns have abnormal urinary tract on
radiological exam. when first evaluated
 3% have hydro 2ndry to spinal shock, probably from spinal canal
closure
 15% have abnormalities that develop in utero as result of abnormal
lower tract due to outlet obstruction
 CMG in newborn showed that 63% have bladder
contractions
 A combination of bladder contractility & external sphincter
activity results in
 synergic (26%)
 Dyssynergic with / out poor detrusor compliance (37%)
 Complete denervation (36%)
 This categorization of lower tract function has been
useful bcs it reveals




Which child is at risk for urinary tract changes
Who should be treated prophylactically
Who needs close surveillence
Who can be monitored at great intervals
 It appears that outlet obstruction is a major
contributor to the development of urinary tract
deterioration
 Recommendations
 Expectant management revealed that infants with outlet
obst. in the form of DSD are at considerable risk for urinary
tract deterioration
 These pts should be treated prophylactically
 CIC alone OR in combination with anticholinergic when
 Detrusor filling pressures > 40 cm H2O
 Voiding pressures > 80-100 cm H2O
 Resulted in an incidence of urinary tract deterioration
of only 8-10%
 Oxybutynin hydrochloride administered in a
dose of 1 mg / year of age BID
 In neonates & children < 1year, dose < 1mg &
increase proportionally as the age reaches 1 year
 On rare occasions when overactive or poor
compliant bladder fails to respond, augmentation
cystoplasty may be needed
 Neurologic findings & recommendations
 Neurologic lesion in myelodysplasia is a dynamic
disease process in which changes take place
throughout childhood
 When a change is noted on neurologic, orthopedic, or
urodynamic assessment, radiologic investigation of the
CNS often reveals
 Tethering of the spinal cord
 A syrinx or hydromyelia of the cord
 Increased intracranial pressure due shunt malfunction
 Partial herniation of the brain stem and cerebellem
 MRI is the test of choice as it reveals anatomic
details of the spinal column & CNS
 Sequential urodynamics testing on yearly basis
beginning in the newborn period and continuing
until 5 yrs old
 It may be necessary to repeat CMG if upper tract
dilates 2ndry to impaired drainage from a poor
compliant detrusor
Surveillance in infants with myelodysplasia
Sphincter Activity
Intact-synergic
Intact-dyssynergic†
Partial denervation
Complete denervation
Recommended Tests
Frequency
Postvoid residual volume
q 4 mo
IVP or renal echo
q 12 mo
UDS
q 12 mo
IVP or renal echo
q 12 mo
UDS
q 12 mo
VCUG or RNC‡
q 12 mo
Postvoid residual volume
q 4 mo
IVP or renal echo
q 12 mo
UDS§
q 12 mo
VCUG or RNC‡
q 12 mo
Postvoid residual volume
q 6 mo
Renal echo
q 12 mo
 Management of VUR
 VUR occurs in 3-5 % of newborns with
myelodysplasia
 Usually associated with poor detrusor
compliance, detrusor overactivity or DSD
 If untreated, incidence of VUR in these infants at
risk increases with time until 30-40% affected by
5 years of age
 VUR grade 1-3 who void spontaneously or who have
complete lesions with little or no outlet resistance with
good bladder emptying
 Prophylactic Abx only
 High grade reflux
 CIC to ensure complete emptying
 Children who cannot empty their bladder spontaneously
regardless of the grade
 Treated with CIC
 Children with poor detrusor compliance with / out hydro
 To add anticholenergic drugs to lower intravesical pressure and
ensure adequate upper tract decompensation
 Bacteriuria occurs in 56% of children with CIC & not harmful
 Except in presence of high grade reflux
 Symptomatic UTI & renal scarring rarely occurs in lesser grades of
reflux
 Crede maneuver should be avoided in children with
reflux, especially those with reactive external sphincter
 In results in a reflex response in external sphincter that increases
urethral resistance & raises the pressure needed to expel urine from
bladder
 Aggravating the degree of reflux & accentuating its watter hammer
effect on kidneys
 Vesicostomy drainage rarely required today
but indicated in
 Infants who has severe reflux that CIC &
anticholenergic fail to improve upper tract
drainage
 Parents cannot adapt to catheterization program
 Who are not good candidates for augmentation
cystoplasty
 The indications of antireflux surgery are not very
different from those with normal bladder
 Recurrent symptomatic UTI while receiving
adequate Abx therapy & appropriate CIC techniques
 Persistent hydro despite effective emptying of the
bladder & lowering of intravesical pressure
 Severe reflux with anatomic abnormality at the UVJ
 Reflux that persists into puberty
 Presence of reflux in any child undergoing surgery
to increase outlet resistance
 Antireflux surgery can be very effective in children with
neurogenic bladder dysfunction as long as it’s combined
with measures to ensure complete bladder emptying
 Since the advent of CIC, success rate for antireflux surgery
approached 95 %
 The endoscopic injection of Deflux has altered the
management of reflux in children with MM
 Its long term effects are yet to be appreciated
 Continence
 Initial attempts at achieving continence include
CIC & drug therapy to maintain low intravesical
pressure
 Drugs
 Glycopyrrolate (Robinol) : most potent oral
anticholenergic drug available today same other S/E
 Tolteradine (Detrol): newly approved, equally effective
as oxybutynin with fewer S/E
 Hyoscyamine (levsin) : potency less, fewer S/E
 Intravesical oxybutynin : fewer S/E compared to oral
 Botulinum roxin A injected into the detrusor
muscle has been effectively used
 Paralyzes the bladder for varying period of time
 May become viable Rx in the future
 Alpha sympathomimetic agents
 If CMG reveals that urethral resistance is inadequate to
maintain continence
 Phenylpropanolamine is the most effective agent
 Surgery
 Viable option when drug therapy fails to achieve
continence
 Generally intervention is delayed till 5 yrs old
 Enterocystoplasty using sigmoid, cecum & small
intestine
 If bladder neck or urethral resistance is insufficient to
allow adequate storage, bladder neck reconstruction is
considered
 Currently, Deflux injections at the bladder neck
are being advocated
 enhances outlet resistance
 Alternative to bladder neck reconstruction
 No long term data
 Continent urinary diversion with closure of
bladder neck has been used to provide better
quality of life for intractable urethral
incompetence
 Sexuality
 In several studies, 28-40% of MM had one or more sexual
encounters
 All of them had a desire to marry & to bear children
 In one study, 72% of male subjects have erection, 2/3
were able to ejeculate
 Other studies revealed 70-80% of MM women were able to
become pregnant
 The degree of sexuality is inversely proportional
to the level of neuologic dysfunction
 Boys reach puberty at age similar to normal boys
 In MM Girls, breast development & menarche
start 2 yrs earlier than usual normal girls
 Bowel function
 The external anal sphincter in innervated by the same
nerves that modulate the external urethral sphincter
 The internal anal sphincter is influenced by more
proximal n. from sympathatic nervous system
 The internal sphincter reflexively relaxes in response to
anal distension
 Consequently, bowel incontinence is frequently
unpredictable
 Incontinence not associated with the attainment of
urinary incontinence
Lipomeningocele & other
Spinal dysraphism
 Group of congenital defects that affects the
formation of spinal column but do not result
in open vertebral canal
 Incidence of lipomeningocele in families
0.043%
 Lesion have no obvious outward signs
Types of occult spinal dysraphisms
Lipomeningocele
Intradural lipoma
Diastematomyelia
Tight filum terminale
Dermoid cyst/sinus
Aberrant nerve roots
Anterior sacral meningocele
Cauda equina tumor
 >90% have cutaneous abnormalities overlying the
spines




Small dimple
Skin tag to a tuft of hair
Dermal vascular malformation
Very noticeable subcutaneous lipoma
 Asymmetrically curving gluteal cleft
 Careful inspection of the legs may show high arched foot /
alterations in the configuration of the toes / discrepancy in
muscle size / shortness / decreased strength in one leg
typically the ankle / gait abnormality
Small lipomeningocele
Hair patch
dimple
Dermal vascular malformation
Abnormal gluteal cleft
 Absent perineal sensation / back pain /
secondary incontinence may be noted
 Abnormal lower tract function in 40-90%





Abnormality increase with age
Difficulty with toilet training
Urinary incontinence after period of dryness
Recurrent UTI
Fecal soiling
 Majority perfectly normal neurologic examination
 CMG
 Abnormal lower tract function in 1/3 of infants < 18/12
 Most likely abnormality is UMN lesion characterized by
overactive bladder &/or hyperactive sacral reflexes
 Rarely, mild form of DSD
 LMN signs occurs in 10% only
 All children > 3 yrs who have not been operated
on OR whom occult dysraphism has been lately
diagnosed
 Have upper or lower lesion or in combination on
CMG (92%) within 2 yrs
 When observed expectantly from infancy after Dx
was made, 58% deteriorate
 Pathogenesis
 Various occult spinal dysraphic lesions produces
different neuologic findings, Reasons:
 Compression on the cauda equina or sacral n. roots by
expanding lipoma or lipomeningocele
 Tension on the cord from tethering 2ndry to differential growth
rates in bony vertebrae and neural elements while the lower end
of the cord is held in place by lipoma or thickened filum terminale
 Fixation of the split lumbosacral cord by intervertebral bony
specule or fibrous band
 Normally, the conus medullaris ends just below
the L2 vertebraae at birth and recedes upward to
T12 by adulthood
 When the cord does not rise or fixed in place bcs
of these lesions, ischemic injury may ensue
 Correction of the lesion in infancy result not only
in stabilization, bt also in improvement in the
neurologic pictures in many instances
 Recommendations
 MRI
 Spinal U/S in children < 3/12
 At this age, vertebral bones have not ossified
 Useful screening tool for visualization of spinal canal
 Currently, most NS advocates laminectomy &
removal of the intraspinal process as completely
as possible, without injuring nerve roots or cord to
release the tether and prevent further injury from
subsequent growth
Sacral Agenesis
 The absence of part or all of 2 or more lower




vertebral bodies
Teratogenic factors play a role
IDDM mothers have 1% of giving birth to a child with
sacral agenesis
16% of children with sacral agenesis have a mother
with IDDM
Maternal insulin-Ab complexes noted to cross the
placenta
 Deletion of chr.7q36 has a role
 Maternal drug exposure (Minoxidil) reported to
cause sacral agenesis
 Familial cases if sacral agenesis associated with
Curarino syndrome




Presacral mass
Sacral agenesis
Anorectal malformation
Deletion in chr.7, leading to HLXB9 genetic mutation
 Association with VACTERL syndrome
reported
 Diagnosis of sacral agenesis
 Presentation bimodal
 ¾ at early infancy
 Remainder at 4-5 yrs
 can be Dx parentally due to frequent use of U/S
 If not detected prenatally or at birth, Dx is delayed
 May present with failed attempts at toilet training
 Sensation intact & lower limb function is normal
usually
 The only clue beside the high index of
suspicion is
 Flattened buttocks
 Low gluteal cleft
 Palpation of the coccyx is used to detect
absent vertebrae
 Dx confirmed with a lateral film of lower spine
 MRI is diagnostic
Gluteal crease is short
and seen only inferiorly
 Findings
 CMG
 UMNL - 35%
 LMNL - 40%
 No signs of denervation at all - 25%
UMNL
 Overactive detrusor
 Exaggerated sacral reflexes
 Absence of voluntary control over sphincter function
 DSD no sphincteric deenervation
 Bladder is thick wall (or trabeculated), with closed
bladder neck on VCUG or U/S
LMN




Acontractile detrusor
Partial or complete denervation of external sphincter
Diminished or absent sacral reflexes
Bladder smooth and small with opened bladder neck
 The presence or absence of bulbocavernous reflex is an
indicator of an UMNL / LMNL respectively
 UTI present in 75% over time
 VUR occurs in UMNL (75%) and in LMNL (40%)
 Recommendations
 CMG / Ultrasound / VCUG or nuclear
cystography
 UMNL
 Anticholinergic
 +/- CIC
 If anticholinergic ineffective in controlling
overactive detrusor, augmentation cystoplasty
required
 LMNL
 CIC & alpha sympathomimetics drugs for
those who cannot empty the bladders or stay
dry between CIC
 Injection of bulking agents
 Artificial urinary sphincter implantation
+ Rx bowel dysfunction
 Associated conditions with sacral agenesis
Imperforate anus
 Alone or part of anomalies ( VATER / VACTERL )
 Male > female 1.5:1
 Sacral agenesis occurs with spectrum of hindgut
abnormalities in Currarino syndrome
 Associattion with fistula to the lower tract is common
 Most common findings on CMG
 UMNL with overactive bladder
 And or DSD
CNS insults
 Cerebral palsy
 Nonprogressive injury of the brain occurring in
the perinatal period that produces either a
neuromuscular disability, a specific symptom
complex, or cerebral dysfunction
 Incidence is 1.5 / 1000 births
 Lesions classified according to which
extremities involved and wt kind of dysfunction
 Spastic diplegia is the most common
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
 Most children has total urinary control
 23.5% has persistent incontinence
 The presence of incontinence is often
related to the extent of physical impairment
 Abnormal bladder & urethral sphincter
function was found in almost all
Type
Upper motor neuron lesion
Number
49
%
86
Mixed upper +lower motor neuron lesion
5
9.5
Incomplete lower motor neuron lesion
1
1.5
No urodynamic lesion
2
3
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
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
-
 Recommendations
 Overactive bladder treated with anticholinergic
 +/- CIC
 Dorsal rhizotomy in selected group of children
who fail to respond to less invasive measures
Traumatic injuries to the spine
 Rarely encountered in children
 Pts with upper thoracic or cervical lesion are likely
to have autonomic dysreflexia with spontaneous
discharge of alpha1 stimulants during bladder filling
& with contraction of detrusor
 Monitoring of BP & availability of alpha blockers are
mandatory during VCUG or CMG
 Management
 If retention immediately after trauma, foley
catheter is inserted and kept in place as short
time as possible
 Until the pt is stable and aseptic CIC can be started
safely on regular basis
 The goal is balanced voiding at pressures < 40
cmH2O, which reduces the 30% risk of urinary
tract deterioration seen in poorly managed pts
 If cannot be achieved, CIC is continued
 Anticholinergic drugs (P.O. or intavesically)
added as they are effective in reducing
overactive bladder
 Alternative Rx
 External urethral sphincterotomy
 Urethral stent placement
 Injection of botulinum A toxin (Botox) into the external
sphincter
 Continent catheterizable abdominal urinary stoma in
pts with low cervical or upper thoracic lesions who
cannot easily catheterize themselves