voiding dysfunction in children by dr.turky k. al-mouhissen urology chief resident - wr king...
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Voiding Dysfunction
in ChildrenBy
Dr.Turky K. Al-MouhissenUrology 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 hypogastric n. to the pelvic plexus & bladder 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 estimatedFor 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 & Non-neuropathic 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
General 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 bladder-sphincter 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
Urinanalysis 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 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 should 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
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
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
It 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 water 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 i 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
Lipomeningocele
Intradural lipoma
Diastematomyelia
Tight filum terminale
Dermoid cyst/sinus
Aberrant nerve roots
Anterior sacral meningocele
Cauda equina tumor
Types of occult spinal dysraphisms
>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
Dermal vascular malformation
dimple
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
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 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
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
Table 123-9. Perinatal Risk Factors in Cerebral Palsy
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