Physiology & Neurophysiology of lower U.T.

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Physiology & Neurophysiology of lower U.T. Classification of voiding dysfunction Evaluation of a child with voiding dysfunction Management

Storage Ø Adequate volume of urine Ø At LOW pressure Ø With NO leakage Ø Voluntary Emptying Ø Complete Ø Efficient Ø Low pressure

Stores urine at low pressure Compresses urine for voiding Bladder (Detrusor) Conveys urine from bladder to outside Urethra Controls urine flow Maintain continence between voidings Sphincter(s)

Low Pressure Storage with Continence Outlet Obstruction - Sympathetic -adrenergic stimulation of bladder neck & post. urethra - Somatic stimulation of external sphincter Bladder Relaxation - B adrenergic stimulation of bladder fundus Voluntary Complete Voiding With Low pressure Outlet Relaxation - Sympathetic -adrenergic inhibition of bladder neck & post. urethra - Somatic inhibition of external sphincter Bladder Contraction - Muscarinic (A.Ch.) bladder receptors - B adrenergic inhibition of bladder fundus

Storage External Sphincter EMG Bladder Neck pressure Bladder pressure Emptying

Ø Neonate: bladder emptying via sacral spinal cord reflex Ø ~2 years: conscious sensation of bladder fullness >> spinal reflex gradually modified & inhibited by pontine micturition center Ø 2-4 years: the childs develops ability to control voiding Ø Balance between inhibiting voiding and initiating voiding not fully mastered 4years until ~

Voiding Frequency: - Frequent micturition 8/day - Infrequent micturition 3/day Incontinence: Normal frequency 4-7 /day Uncontrollable leakage of urine. It can be continuous or intermittent Urgency: Sudden and unexpected experience of an immediate need to void

Hesitancy: Difficulty in the initiation of voiding or that the child must wait a considerable period before voiding starts. Straining: The child applies abdominal pressure to initiate and maintain voiding Intermittency: Term applied when micturition occurs not in a continuous stream, but rather in several discrete spurts (> 3 years)

Residual urine > 20 ml

Normal voiding that occurs in an inappropriate or socially unacceptable place or time. - Nocturnal or diurnal - Diurnal enuresis vs dysfunctional voiding

Neuropathic Anatomical Spinal Supra-Spinal Peripheral Nighttime incontinence Functional Daytime incontinence Minor Moderate Major

Neurospinal Dysraphism Sacral Agenesis

Ectopic Ureter PUV

Minor Disorders No impact on upper UT Moderate Disorders Some impact on upper UT Major Disorders Overt impact on upper UT 1. Extraordinary daytime urinary frequency syndrome 2. Giggle incontinence 3. Stress incontinence 4. Post-void dribbling 1. Bladder/Bowel dysfunction 2. Underactive bladder 3. Overactive bladder 1. Transient UD dysfunction of infancy 2. Hinman Syndrome 3. Ochoa Syndrome 4. Myogenic detrusor failure

- > Boys between 3-8 years - Sudden onset of daytime-only frequency and urgency every 10 20 min - Bedwetting is rare - Etiology is unknown - Apart from urinalysis / urine culture, NO further investigations - Lasts from days to months (up to 9 mo) - TTT: reassurance Pediatr Nephrol (2009) 24:789 795

Pediatric Incontinence: Evaluation and Clinical Management, First Edition. Edited by Israel Franco - Massive unexpected detrusor contraction associated with complete bladder emptying - Episodes occur with giggling and laughter - Starts around puberty - NO UTI - TTT: Anticholinergics - Sympathomimetics Biofeedback Botulinum toxin injection in detrusor

- NOT IN CHILDREN - Starts in teenage girls - Increase incidence with high-impact sports - Involuntary loss of urine during coughing, sneezing, or physical exertion such as sport activities Curr Opin Pediatr. 2006;18(2):139

- Wetting typically occurs after micturition. - Due to trapping of urine in the vagina, more in obese girls - Presents with labiovulvar erythema/burning/itching - TTT: Postural correction J Pediatr Adolesc Gynecol. 2009;22(6):347

- Anorectal and lower urinary tract function are interrelated - Constipation is often associated with bladder dysfunction - Several theories to explain - Bladder dysfunction may be associated with encopresis - TTT: of constipation timed voiding Nat Rev Urol. 2013; 10(11): 640 8.

- 2 nd most common VD after nocturnal enuresis. - Abnormal bladder contraction during filling phase - May be due to delay in acquisition of cortical inhibition over uninhibited detrusor contractions - Symptoms include urgency, urge incontinence, enuresis and Recurrent UTI - Holding maneuvers such as leg crossing and squatting are common Clin Pediatr (Phila). 2003; 42(6): 553

- Habitual voiding postponement. - Holding maneuvers >>> low voiding frequency >>> Prolonged detrusor overdistension>>>hypoactive bladder >>> Large post-void residuals with overflow incontinence --- risk of UTI - TTT: timed voiding regimen alpha blockers - CIC Rev Urol. 2013;15(1):11 22.

- High-grade reflux with thick-walled bladders noted prenatally. - Absence of infravesical obstruction. - Spontaneous resolution noted to reach 27% even with grade V reflux. - May be associated with delay in maturation of the external urinary sphincter - TTT: vesicostomy J Urol. 1993; 150 (2 Pt 2): 617-22

- Sphincter contraction during voiding >>> high voiding pressure bladder decompensation UTI renal damage Symptoms Incontinence enuresis UTI constipation - encopresis Findings UTI hydronephrosis - VUR renal insufficiency UD diagnostic TTT - Anti-cholinergics, alpha-blockers, Botox - Re-education, biofeedback - Surgical (failure) Urology. 2011;78(6):1397 401.

- All features of Hinman $ but genetically determined (AR - HPSE2 gene) - Inversion in the muscles of facial expression that becomes more evident when smiling (grimace that gives the appearance of sobbing or crying) - Diagnosis early in life (because of facial expression) Am J Hum Genet. 2010;86(6):957 62.

BJU Int. 2005;96(1):140 3. - End-stage bladder decompensation - End-stage of neuropathic bladder - PUV - Post-void residue, recurrent UTI hydronephrosis renal damage - TTT: alpha blockers

31 32 3 When to suspect voiding dysfunction Initial evaluation: History, Examination, Simple Lab Further Investigations

Ø Daytime urinary incontinence in school-age or previously toilet-trained children. Ø Persistent urinary symptoms such as urgency, dribbling, or dysuria Ø Bladder dysfunction has been associated with conditions such as VUR, recurrent UTI, and chronic constipation or encopresis. Evaluation and diagnosis of bladder dysfunction in children. UpToDate

Voiding schedule: frequency of voids, incontinent episodes, voided volume Symptoms of bladder dysfunction: urgency, hesitancy, holding maneuvers, etc... Bowel habits: frequency, stool consistency, encopresis, stool withholding Neurodevelopmental & psychogenic disorders Family History, perinatal H. and toilet training history

Voiding and fluid intake diary Evaluation and diagnosis of bladder dysfunction in children. UpToDate

Ø Neurological examination Ø Abdominal & rectal examination Ø Observation of urine stream (if possible)

Ø Urinalysis Ø Urine culture & sensitivity

Further Evaluation ØFailure to improve following initial trial of conservative management (e.g. timed voiding & treatment of constipation) ØSuspicion of a neurologic / anatomic etiology ØConstant continuous incontinence, since these children are more likely to have an organic cause such as an ectopic ureter ØRecurrent UTI /unexplained VUR ØImpaired kidney functions in a child with voiding disorder

I. Imaging Hydronephrosis Post-voidresidual Bladderwallthickness

I. Imaging VUR PUV

I. Imaging Tetheredcord Diastematomyelia

II. Urodynamics Overactivebladder Bladderobstruction Underactivebladder Dysfunctionalvoiding

Pharamacological Ø Anticholinergics Ø DDAVP for enuresis Ø Antibiotics prophylaxis for VUR Ø Laxatives Non-pharmacological Ø TTT of constipation Ø Timed voiding Ø Biofeedback Ø Pelvic floor exercises

ü Some causes of VD may be hazardous with an overt impact on upper UT ü VD should be suspected if there is daytime urinary incontinence or persistent urinary symptoms ü Detailed voiding diary is the most important step in evaluation of any case with VD