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