Voiding Dysfunction in Children COL John Roscelli Pediatric Nephrology San Antonio Military Pediatric Center.
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Voiding Dysfunction in Children COL John Roscelli Pediatric Nephrology San Antonio Military Pediatric Center 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 micturition centers CNS micturition centers Exert voluntary control over spinal centers Spinal micturition centers T10L1 S2S4 T10-L1 Sympathetics via hypogastric Nerve S2-S4 Parasympathetic via Pelvic N Somatic via Pudental N Low pressure storage with continence CNS micturition centers T10 -L1 S2S4 Outlet obstruction: Sympathetic -adrenergic stimulation of bladder neck & posterior urethra from T10-L1 via Hypogastric Nerve Somatic stimulation of External Sphincter from S2-S4 via Pudental Nerve Bladder Relaxation: Allows continent storage of significant volumes of urine at < 20 mmHg -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 T10 -L1 S2S4 Allows complete emptying at pressures < 40 mm Hg CNS micturition Centers Inhibit sympathetic -adrenergic stimulation of bladder neck/posterior urethra & somatic stimulation of External Sphincter Bladder Contraction: CNS micturition Centers Inhibits -adrenergic bladder relaxation & stimulates Parasympathetic cholinergic stimulation of bladder fundus from S2-S4 via Pelvic Nerve Normal Voiding Study External Sphincter EMG Activity Bladder Neck Pressures Bladder Pressures Storage (cc) Voluntary Voiding 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 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 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 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 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 3 Day Elimination DiaryYour most powerful diagnostic tool & its CHEAP & BENIGN Good time to do intake diary Parents record liquid intake volume Determines BM problems Characterizes voiding Frequency of voids Volume of voids Accidents Associated symptoms Allows Characterization voiding disorder Storage Emptying Continence 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 Don’t 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 don’t have to void on awakening When they void voluntarily it is large volumes, prolonged & requires straining Often don’t take time to completely empty 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 FOCUSED NEUROLOGIC EXAMINATION Nerve Root Motor Sensory S1 Plantar flexion Side of foot S2 Big toe extension Back of heel S3 Big toe flexion 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 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 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 Approach to Voiding Dysfunction Child with Suspected Voiding Dysfunction Day Time wetting/Incontinence Recurrent UTIs Persistant or worsening VUR Rule out Organic Pathology History, physical UA/Culture Renal Bladder US, VUCG if UTIs Improved Follow up Elimination Diary Treat Constipation Consider Antibiotic Prophylaxis for UTIs Intiate timed voiding plan Continued Problems Urology referal Flowmetry Urodynamic Studies Urge Syndrome Bladder/Sphincter Dysfunction Lazy Bladder Normal Study Anticholinergic Timed Voiding Biofeedback Timed Voiding Check Compliance with Timed Voiding Program Assess motivation Consider psychological evluation 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 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 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 using Segment of Ileum Improving Continence by Increasing Sphincter Resistance 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