Voiding Dysfunction in Children

COL John Roscelli Pediatric Nephrology San Antonio Military Pediatric Center

Perspective
 Incontinence is part of transitional phase from infantile to adult lower urinary tract function
 Wetting disorders often considered necessary nuisance & tolerated until child lags behind peers  Parental concerns about voiding are common & often supersede the child's anxiety

 However, voiding dysfunction can be a sign of underlying pathophysiology that needs Rx to prevent Renal/Urologic damage

Agenda
 How the Lower Urinary Tract Works  Voiding Dysfunction in children with no organic pathology
Definition Presentation modes Evaluation Treatment

How the Lower Urinary Tract Works

Bowl and Bladder Function

 Lower GU tract tied to lower GI tract
 Same embryogenic origin: endodermal tissue  Up to sixth week gestation urogenital sinus & the hindgut empty into common cloaca

 Problems with elimination in one usually associated with problems in the other  Proper term is Elimination Dysfunction Syndrome

Function of Lower Urinary Tract STORAGE of adequate volumes of urine at low pressure & with no leakage EMPTYING that is
Voluntary Efficient Complete Low pressure

Lower Urinary Tract is a Functionally Integrated Unit

Ureteral Vesicle Junction Bladder Sphincter Urethra Neurologic control mechanisms

Anatomy & Neurophysiology of the Lower Urinary Tract

Bladder (detrusor)
Stores urine at low pressure Compresses urine for voiding

Urethra
Conveys urine from bladder to outside world

Sphincter(s) internal & external

Controls urine flow & maintain continence between voidings

Nervous system control of Lower Tract

 CNS
 Periaqueductal gray matter receives bladder filling info  Frontal/parietal lobes & cingulate gyrus inibit lower micturation centers  Hypothalamus center initiate voluntary voiding  Pontine Micturation center excites Bladder & inhibits sphincter  Cerebellum integrates

 Spinal
 Sympathetics T10-L1 via hypogastric Nerve  S2-S4 Parasympathetic via Pelvic N Somatic via Pudental N

Nervous system control of Lower Tract

CNS micturition centers
 CNS micturition centers
 Exert voluntary control over spinal centers

 Spinal micturition centers

 T10-L1  Sympathetics via hypogastric Nerve  S2-S4  Parasympathetic via Pelvic N  Somatic via Pudental N

T10L1

S2S4

Autonomic NS receptor Distribution

Low pressure storage with continence

CNS micturition centers

Outlet obstruction:
Sympathetic E-adrenergic stimulation of bladder neck & posterior urethra from T10-L1 via Hypogastric Nerve Somatic stimulation of External Sphincter from S2-S4 via Pudental Nerve

T10 -L1 S2S4

Bladder Relaxation:
Allows continent storage of significant volumes of urine at < 20 mmHg

F-adrenergic stimulation of bladder fundus from T10-L2 via Hypogastric Nerve decreases bladder tone

Voluntary Efficient Complete Low Pressure Voiding Outlet relaxation:

CNS micturition centers CNS micturition Centers Inhibit sympathetic E-adrenergic stimulation of bladder neck/posterior urethra & somatic stimulation of External Sphincter

T10 -L1 S2S4

Bladder Contraction:
CNS micturition Centers Inhibits F-adrenergic bladder relaxation & stimulates Parasympathetic cholinergic stimulation of bladder fundus from S2-S4 via Pelvic Nerve

Allows complete emptying at pressures < 40 mm Hg

Normal Voiding Study

External Sphincter EMG Activity Bladder Neck Pressures

Bladder Pressures

Storage (cc)

Voluntary Voiding

Normal Voiding Study

Maturation of Voiding
Neonatal voiding
 Controlled by sacral spinal cord reflex
 Bladder distention sends signals to sacral spinal cord micturition center Spinal cord micturition center sends efferent signals that cause detrusor contraction & relaxation of external sphincter

 Results in frequent, complete, low pressure emptying

Newborns void 20 x/day with only a slight decrease during the 1st year of life

Maturation of Voiding
 Bladder capacity increases & voiding frequency decrease with growth
 Bladder capacity in Ounces (30ml) = Age (yrs) +2

 1-2 yrs: conscious sensation of bladder fullness develops  2-3 yrs: Ability to initiate or inhibit voiding voluntarily develops  2-4 yrs: Voiding comes under reliable voluntary control
 By 4 years of age, most children have achieved an adult pattern of micturition

Maturation of Voiding
 By age 4 Micturition spinal reflex fully modulated by CNS micturition center via a spinobulbospinal tact
As bladder fills, desire to empty occurs-child must consciously suppress this desire until he/she can get to toilet With conscious voiding, external sphincter willfully relaxed prior to initiating bladder contraction
Sphincter relaxation & bladder contraction, must occur in coordinated fashion for proper emptying

Maturation of Voiding
Initially child has better control over external sphincter than bladder
Easier to stop urination than start it Voiding inhibition done by contracting external sphincter rather than inhibiting bladder contraction
This pattern may be reinforced during toilet training Persistence of this pattern is bladder sphincter dysnergia

Usual sequence of bowel & bladder control

Nocturnal bowel control Daytime bowel control Daytime control of voiding Nocturnal control of voiding

Maturation of Voiding

 By 4 most children have adult voiding pattern Brazelton studied 1,170 children & found
26% achieved daytime continence by age 24 months 52.5% by age 27 months 85.3% by age 30 months 98% by age 36 months

Definitions
Incontinence in Children

International Children's Continence Society definitions

Enuresis: normal voiding that occurs at inappropriate time or involuntarily in socially unacceptable setting
nocturnal enuresis- nighttime wetting diurnal enuresis- daytime wetting

Definitions
 Diurnal enuresis is often interchanged with dysfunctional voiding but they are not the same
 Not all dysfunctional voiders are incontinent

 Dysfunctional voiding can be neuropathic or nonneuropathic

 Neuropathic- voiding disorders caused by neurologic conditions such as spina bifida, transverse myelitis, or spinal cord trauma  Nonneuropathic- functional voiding problems in neurologically normal children

Characterization of Voiding Dysfunction

 Storage Problem: Failure to Store normal volumes of urine at low pressure & without leakage
 Non compliant bladder  Irritable bladder  Inadequate sphincter tone during filling

 Emptying Problem: Failure to empty completely, on command, efficiently at low pressures

 Failure of neurological control of bladder  Bladder muscle failure  Failure of sphincter relaxation during voiding

Clinical Problems from Voiding Dysfunction

 Increased bladder pressures resulting in
VUR Upper tract damage Bladder hypertrophy leading to detrusor failure

 Residual Urine
UTI

 Incontinence
Social consequences

Voiding Dysfunction in Children with no organic pathology

Voiding Dysfunction in Normal Children- 3 Issues

 Clinician must 1st suspect voiding dysfunction in certain clinical circumstances in normal children  Clinician must then rule out Neurologic, Urologic & other organic (diabetes, concentrating defects) problems  Clinician must then characterize & Rx the functional voiding dysfunction

Presentations of Voiding Dysfunction in Normal Children

Urologic Presentation GI Presentation Occult Neurologic presentation

Urologic Presentation
Signs & Symptoms which suggest voiding dysfunction

 Infrequent voiding  Frequent voiding  Urgency  Dysuria  Holding maneuvers  Straining  Poor stream  Intermittent stream

 Incomplete emptying  Incontinence  Urinary tract infections  VUR

Urologic Presentation
It can not be overemphasized to the general pediatrician how important it is that they rule out voiding dysfunction in all their children with recurrent UTIs, VUR or incontinence

GI Presentation
Signs & Symptoms which suggest voiding dysfunction

 Fecal staining of undergarments  Fecal incontinence  Constipation  Encopresis  Obstipation (i.e., severe constipation causing obstruction)  Abdominal pains

Occult Neurologic Presentation

 Early detection may prevent neurologic damage and its bladder or bowel dysfunction sequelae  Complex spina bifida occulta is an important disease entity because of its prevalence in the general population (as much as 1%);
 Lower back abnormalities such as nevus, dermal sinus, or dimple  Abnormal neurologic examination, or foot or gait abnormality

Ocult Neurologic Presentation

Spinal cord tethering suggested by

 Lower back abnormalities such as nevus, dermal sinus, or dimple  Pain in the lower back during stretching of the lower extremities  Gait abnormalities  Worsening symptoms during growth spurts  Severe stool incontinence  Complex enuresis refractory to routine Rx

Types of Voiding Dysfunction Disorders in normal Children

Minor Voiding Dysfunctional Disorders

 Extraordinary daytime urinary frequency syndrome  Giggle incontinence  Stress incontinence  Post void dribbling  Vaginal voiding  Primary monosymptomatic nocturnal enuresis

Major Voiding Dysfunctional Disorders Hinman syndromenon neruogenic neurogenic bladder Ochoa (urofacial) syndrome
Hinman syndrome with Autosomal dominant inheritance & facial grimace when smiling

Myogenic detrusor failure

Moderate Voiding Dysfunctional Disorders

Overactive bladder/Urge Syndrome Bladder Sphincter Dysnergia Lazy bladder syndrome

Moderate Voiding dysfunctional disorders

Classification of Diurnal Voiding Dysfunction Term

Urge syndrome

Filling
Multiple uninhibited detrusor contractions with increased EMG activity and expressions of urgency

Voiding Post void Residual

Usually normal None

Classification of Diurnal Voiding Dysfunction Term

Bladder/ sphincter dysfunction

Filling
Usually normal

Voiding Post void Residual

Increased EMG activity causing diminution or interruption of the urinary flow

Variable

Classification of Diurnal Voiding Dysfunction Term

Lazy bladder syndrome

Filling
Abnormally capacious bladder with little or no expression of urge

Voiding Post void Residual

Prolonged, decreased flow with abdominal straining and bursts of EMG activity Always

Evaluation of Voiding Dysfunction

Purpose of evaluation
Characterize the Elimination problems to direct treatment
Storage problem Emptying problem Continence problem

Rule out Neurolgic, Urologic or other organic causes

Evaluation of Dysfunctional Voiding

 Index of suspicion  History  History  History  Physical Exam  Physical Exam  Simple Lab Tests  Imaging  Urodynamics

History
To characterize the Problem

 Evaluation of dysfunctional voiding begins with a detailed elimination history

History of current elimination problems
Detailed voiding history Detailed Stooling history

Past elimination/urologic History

UTIs Constipation Age of toilet training

Intake history- fluids and diet Family history of urologic problems

History
To characterize the Problem

Voiding symptoms & pattern of incontinence must be quantified

Urgency, frequency, straining, dysuria etc Holding maneuvers such as leg crossing, squatting, or "Vincent's curtsey" Continuous incontinence in a girl suggests ectopic ureter that inserts distal to urethral sphincter or into the vagina

Holding Maneuvers

Ectopic Ureter

3 Day Elimination DiaryYour most powerful diagnostic tool & its CHEAP & BENIGN
Determines BM problems Characterizes voiding Frequency of voids Volume of voids Accidents Associated symptoms Allows Characterization voiding disorder Storage Emptying Continence

Good time to do intake diary

Parents record liquid intake volume

History
Irritable Bladder
 Urgency & frequency as Cerebral cortex unable to inhibit reflex bladder contractions triggered during filling
 Parents need to know where every bathroom is at mall etc

 When they void, void normally although usually have a small bladder capacity  Exhibit behaviors to avoid leakage: Dancing, squatting, holding & posturing
 Classic sign of bladder instability is "Vincent's curtsy- squatting posture in girls in which the heel compresses the perineum and thereby obstructs the urethra to prevent urinary leakage
 If unsuccessful get urge incontinence of small amount of urine

 These behaviors can lead to bladder sphincter dysnergia

History
Infrequent Voider

 Typically school girls with recurrent UTI & often with history of intermittent enuresis  Postpone voiding as long as possible
 Dont like to void in public bathrooms  Use holding maneuvers to fight urge to void
 If holding maneuvers fail get incontinence- Suzy waits till the last minute to void & then its to late

 Develop large capacity bladders- void 2-3 times per day & often dont have to void on awakening

 When they void voluntarily it is large volumes, prolonged & requires straining
 Often dont take time to completely empty

History
Infrequent Voider

 Physician must uncover that the child with a wetting problem actually has abnormally few voids & a weak bladder
May wait at least an hour after waking to void May void only 2-3 to three times daily, often not at all during school Straining during urination common because detrusor is large-capacity & capable of only weak contractions

History
To Identify underlying treatable Pathology

 Identify organic pathology

Diabetes, epilepsy, obstructive sleep apnea Neurologic problems Urologic problems

 Identify functional cause that is treatable

Voiding symptoms may be sign of sexual abuse Stressful occurrence at home or school can trigger incontinence

Physical Examination
 1st step is growth, general health & vital signs including BP  2nd step is to inspect the child's underwear for evidence of wetness or soiling  3rd step is to observe or at least listen to voiding for evidence of weak, slow or intermittent stream  4th step is focused physical exam

Physical Examination
Abdomen

Renal masses Distended bladder Large stool mass suggestive of constipation

Physical Examination
Perineum & Genitalia
 Dampness at beginning of exam & with straining  Signs of erythema or irritation may be indicative of vaginal voiding  Meatal stenosis in boys & presence of labial adhesions in girls  Signs of trauma suggestive of sexual abuse  Careful examination of the introitus for an ectopic ureter  Location of anus

Focused Neurolgogic Examination

 Lumbosacral spine for lipoma, sinus, pigmentation tufts of hair- may be clue to underlying occult myelodysplasia  Perineal sensation, anal sphincter tone, lower limb function/gait/sensation & Peripheral reflexes  The bulbocavernosus reflex: squeeze glans penis or clitoris & observe or feel reflex contraction of external anal sphincter
 Checks integrity of the lower motor neuron reflex arcs  Absence suggestive of a sacral neurologic lesion

NERVE ROOTS & THEIR ASSOCIATED SENSORY & MOTOR FUNCTIONS Level
L1 L2 L3 L4 L5 S1 S2 S3, 4

Sensation
Inguinal area Anterior/medial thigh Knees, lateral thigh Anterior/medial tibia Lateral tibia Sole of the foot Heel of the foot Perineal

Motor
Thigh extension/flexion Thigh extension/flexion Lower leg flexion Lower leg extension Dorsiflexion of foot (cannot walk on heels) Plantar flexion of foot (cannot walk on toes) Dorsiflexion of big toe Plantar flexion of big toe

FOCUSED NEUROLOGIC EXAMINATION

Nerve Root S1 S2 S3

Motor Plantar flexion Big toe extension Big toe flexion

Sensory Side of foot Back of heel Perineum

Routine Labs
Urine tests best obtained on 1st AM specimen after overnight NPO
UA
Specific gravity- over 1.020 rules out significant concentrating defect pH Glucose Blood Protein Microscopic

UC

Other Studies that can be obtained prior to referral Post void residual urine by catheter Abdominal radiograph (KUB)
Identifies lumbar-sacral anomalies, bowel gas patterns & amount of stool

Renal and bladder ultrasound

Sonography
 Upper tract
Size, contour, echogenicity Hydro-nephrosis  Lower tract
 Assess bladder wall thickness (nl <3mm when full; 5 mm when empty)  Post void residual > 2 mL/kg is abnormal

Excellent correlation between residual urine by direct urethral instrumentation & noninvasive sonography

Other Studies that can be obtained prior to referral Nuclear Medicine renal scan
Cortical scan to RO scars or difference in function Functional SCAN with/without lasix to RO obstruction

Voiding cystourethrography
History of UTIs Family history of VUR

Studies requiring referral

Rarely required but simple & non invasive

Uroflometry

Very rarely required & invasive

Urodynamics with electromyography of the external urinary sphincter

Studies requiring referral

Uroflow/Flowmetry
Non invasive assessment of urine flow rates
Staccato voiding or intermittent stream
Intermittent involuntary sphincter activity during voiding

Fractionated & incomplete voiding

Abdominal straining needed to assist bladder emptying & contraction of abdominal muscles contracts the sphincter

Studies requiring referral

Urodynamics often with video fluro  Parameters used to diagnose urodynamic dysfunction
Bladder capacity of <10-15 mL/kg body weight, Postvoid residual of >2 mL/kg body weight, Detrusor hyper-reflexia, (detrusor contractions during bladder filling without urine leakage and intravesical pressure of >40 cm H2 O Voiding detrusor pressure of >70 cm H2 O Dyssynergic increase or lack of suppression of sphincteric EMG with a detrusor contraction.

Studies requiring referral

Urodynamics often with video fluro

Studies that should never be done

Cystoscopy with or without urethral dilation or meatotomy

These are rarely if ever useful and are expensive & potentially dangerous

Management of Voiding Dysfunction in Children with no treatable Neruologic, Urologic or other organic etiology

Treatment of Voiding Dysfunction

Non Pharmacological

Timed voiding is the easiest & most effective Rx & it works for irritable bladder & infrequent voider
Regular by the clock voids q 2-3 hours during day

Biofeedback Kegel exercises

Treatment of Voiding Dysfunction

Pharmacological

 Anticholinergic
Used for irritable bladder especially with urgency, frequency & urge incontinence
Oxybutinin 0.1-0.15 mg/kg per dose 3 x day
Dry mouth, constipation, drowsiness & heat intolerance

 Imipramine used primarily for nocturnal enuresis  Low dose UTI prophylaxis

Treatment of Voiding Dysfunction

TREAT STOOLING DYSFUNCTION

UTIs, VUR & Elimination Dysfunction

Strong association between the 3 Treat voiding dysfunction Treat stooling dysfunction

Approach to Voiding Dysfunction

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Summary
We have reviewed
 Function (continent storage & voluntary emptying at low pressures) & how the lower GU tract works & how it matures
 Relationship with lower GI tract

 Voiding dysfunction syndromes in normal children

 When to suspect it- UTIs, VUR, incontinence  How to evaluate (history, voiding diary)  How to RX voiding dysfunction
 Timed urination,  Treat stooling dysfunction

Voiding Dysfunction in Children with Neurogenic Bladder

Spinal cord injury (SCI) produces profound alterations in lower urinary tract function

Incontinence Neurological obstruction

Elevated intravesical pressure VUR

Increased risk of UTIs Stones

Neurogenic Bladder Made Simple

Lack of higher CNS control results in  Inability to sense fullness & voluntarily void
 Detrusor controlled by un modulated spinal reflex

 Sphincter with fixed passive resistance- Leak Point Pressure (LPP)

 Varies between patients may change in same patient  At bladder pressures < LPP no leakage  At bladder pressures > LPP leakage or urination  Sphincter may not relax when bladder contractsbladder sphincter dysnergia
 Results in high voiding pressures

Neurogenic Bladder Made Simple

 High LPP pressure is good for continence but bad for the kidney
Prolonged LPP > than 40 cm H2O have been associated with
VUR Upper tract deterioration Decreasing bladder compliance

Neurogenic Bladder Made Simple

Bladder compliance is another key variable & may change over time
Determined by neurologic reflex activity & LPP Poor bladder compliance associated with
Incontinence UTIs Upper Tract Damage

Focus is on 2 issues
Preservation of Renal function
Maintaining normal bladder pressures during filling & voiding Minimizing UTIs

Continence
Not an issue in first couple of years of life

Evaluation of Newborn with Neurogenic Bladder

 Assess upper tract for damage or evidence of high pressure (hydronephrosis)
Creatinine, lytes UA & Cultures Renal US CT urography can give more detail if US abnormal
Can do non contrast MRI if there is renal failure Some use nuclear studies

Evaluation of Newborn with Neurogenic Bladder Assess lower tract for evidence of increased voiding pressure
Bladder US for bladder hypertrophy & post void residual- obtain in newborn period VCUG for VUR & bladder hypertrophy Urodynamics for LPP & compliance

Newborn with Neurogenic Bladder

General Treatment

 Prevention
Folic Acid- 0.4 mg per day start prior to pregnancy

 Minimize spinal damage

Prenatal Diagnosis
Suspect in certain racial groups Prenatal screening
 E fetoprotein- 16-17 weeks GA Fetal sonography- 17th week GA

C Section prior to labor Proper handling post delivery

Newborn with Neurogenic Bladder

General Treatment

Latex precautions from birth

Latex allergy seen in up to 40% of spina bifida patients

Treat GI tract dysfunction Maximize orthopedic function Avoid obesity

Treatment of Neurogenic Bladder in the infant based on Evaluation

 Low LPP, normal bladder functionobservation  Flaccid bladder unable to empty- Clean Intermittent Catheterization- CIC  Hyperreflexic &/or non compliant bladder- CIC with anticholinergics  CIC if needed done every 3 hours
NO CREDE If upper tracts deteriorate- vesicostomy

Treatment of Neurogenic Bladder

Continence, Bladder Pressures & UTIs

Urologic Rx Based on bladder/sphincter physiology

Low LPP, normal bladder function
Observation for neonates CIC for continence in older children

Flaccid bladder unable to empty- CIC Hyperreflexic &/or non compliant bladder- CIC with anticholinergics
Oxybutynin 0.1 mgk/Kg per dose 3 X per day

Treatment of Neurogenic Bladder

Continence, Bladder Pressures & UTIs

 CIC is key- s bladder pressures, improves continence & eliminates residual urine
CIC in newborns
done every 3 hours NO CREDE If upper tracts deteriorate- vesicostomy

In older children CIC can be made easier with Continent Catheterizable stomas
Especially valuable in males who still have perineal sensation or children with poor coordination
Metroffanof uses appendix as conduit

Improving Continence
Continent Catherizable Stomas

 Appendix (Mitrofanoff), section of ileum or colon placed from umbilicus to bladder & tunneled into bladder to prevent reflux  Indicated in
 Wheel-chair bound patients with severe scoliosis lordosis  Poor upper extremity function  Males with intact urethral sensation

Bladder Augmentation
 Indicated when medical therapy fails to achieve adequate low-pressure capacity with continence  Variety of substances and surgical techniques used each with problems
Use of intestinal tract allows absorption or secretion of electrolytes from or into urine

 All require religious CIC to avoid rupture

Bladder Augmentation
Variety of Methods  Ileum & colon
 Hyperchloremic hypokalemia acidosis, mucous

 Stomach
 Less mucous  Can cause hyperkalemic metabolic aklalosis  Can cause hematuria and dysuria due to acid
 Rx with H2 blockers

 Dilated ureter of non functioning kidney

 None of problems seen with GI tract

 Detrusor mytomy (autoaugmentation)

 None of problems seen with GI tract

Bladder Augmentation using Segment of Ileum

Improving Continence by Increasing Sphincter Resistance

 E Adrenergic drugs (phenylpropanolamine, pseudoephedrine) increase sphincter tone

Usually only marginally effective

 Surgical techniques
Periurethral injections Bladder neck suspension & Sling procedures Artificial urinary sphincter

Vesicoureteral Reflux (VUR)

 40-65% of neurogenic bladder patients have VUR  Rx aimed at reducing bladder pressures rather than fixing the VUR
CIC Bladder Augmentation

 Prophylactic antibiotics controversial  Surgical correction of VUR indicated for

Deterioration of upper tracts Recurrent pyelonephritis

Urinary Tract Infections

Bacteruria- rule not the exception
 J Peds 126; 1995; 490

Urinary Tract Infections

Treatment of asymptomatic bacteriuria in SCI patients of no proven benefit
Do not treat cultures treat patients

Working definition of true UTI in these patients is fever with + UC

Rx of Urinary Tract Infections

Symptomatic UTIs treated with narrowest spectrum antibiotics for the shortest possible time
Same antibiotics as used for Rx of complicated UTIs in general population

Rx of Urinary Tract Infections

Prophylaxis does not decrease UTIs or asymptomatic bacteruria- (J Peds
132;1998;704)

Some still use if there is VUR  Other methods also unsuccessful

Cranberry juice- J Peds 135; 1999; 698 Single use sterile catheter Peds 108;2001;2001

Summary
We have reviewed
 Function (storage & voluntary emptying at low pressures) & how the lower GU tract works & how it matures
 Relationship with lower GI tract

 Voiding dysfunction in normal children

 When to suspect it- UTIs, VUR, incontinence  How to evaluate (voiding diary) & Rx it (timed urination)

 Evaluation & Rx of children with neurogenic bladder- focus on preserving upper tract & continence

References
 Pediatric Clinics N America 48; Dec 01 1489-1503 & 1505-1518  Fernandes; The Unstable Bladder in children; Journal Peds; 118; 1991; 831  Pediatrics in Review; Volume 21 Number 10 October 2000; 336-341