evaluation, treatment & intervention in the pediatric neuropathic bladder

Evaluation, treatment & intervention in the pediatric

neuropathic bladderPaul F. Austin, MD, FAAP

Professor of Urologic Surgery

Department of SurgeryDivision of Urologic SurgerySt. Louis Children’s Hospital

Washington University School of Medicine

Department of SurgeryDivision of Urologic Surgery

ICCS Standardisation Documents


Disclaimers and limitations• Not a systematic literature review• There is a paucity of level I or level II ‘‘levels of evidence’’ publications• These recommendations are a consensus of a compilation of best practices

• Review of the literature• Relevant research• Expert opinion• Current understanding on the pathophysiology of neuropathic

bladder and bowel• Draft review document was open to all the ICCS members via the ICCS web

site • Feedback was considered by the core authors and by agreement,

amendments were made as necessary


ObjectivesNeuropathic bladder & bowel documents• To create an educational reference document that will guide healthcare

providers in the evaluation and management of children with neuropathic bladder & bowel dysfunction

• To provide a consensus view of the members of the ICCS in the evaluation and management of children with neuropathic bladder & bowel dysfunction


Initial evaluation• Determined by several factors:

• Timing of presentation or diagnosis – infancy vs. older child• Etiology


Open spinal cord lesionInitial evaluation• Check PVR

• Ultrasound or catheter• Urodynamics

• Usually 2 -3 months of age• Screening for:

High pressure DO contractions

Elevated detrusor filling &/or voiding pressures


Open spinal cord lesionInitial evaluation

• Renal & bladder U/S• Screening for:

• Hydronephrosis, • Ureteral dilation


Open spinal cord lesionInitial evaluation

• Renal & bladder U/S• Screening for:

• Discrepancy in renal size or contour

RK: 9.2 cm LK: 6.7 cm


Open spinal cord lesionInitial evaluation• Renal & bladder U/S

• Screening for:• Bladder wall thickness


Open spinal cord lesionInitial evaluation• VCUG

• Not routine• Indicated when:

• Abnormal U/S imaging of kidneys

• Bladder urodynamic studies reveal high risk• Detrusor overactivity• Poor detrusor compliance• Elevated leak point pressure

and DSD


Neuropathic bladder – Video-urodynamics


Follow-up of NBD dysfunctionNewborn to toddler

Urodynamic studies

High riskCIC +/- anticholinergics

Low riskDiaper voiding

• Repeat UDS (with RBUS) in 2 – 3 months after initiating therapeutic interventions

• RBUS every 6 months for child with DO• UDS yearly unless changes seen on RBUS or with lower extremities

Rationale: Elevated risk of developing tethered cord


Follow-up of NBD dysfunctionToddler to adolescent• Cord tethering risk lessens• RBUS yearly or every 6 months• UDS

• Changes on RBUS• Changes in ambulation or lower extremity function• Changes in continence• Increased UTIs


Follow-up of NBD dysfunctionAdolescent to adult• 2nd time period of growth spurt and increased risk of tethering• RBUS yearly

• May consider every 2 years after growth velocity diminishes• UDS

• Changes on RBUS• Changes in ambulation or lower extremity function• Changes in continence• Increased UTIs


Follow-up of NBD dysfunctionAdulthood• RBUS every 3 years• UDS

• Changes on RBUS• Changes in continence• Increased UTIs


Evaluation of neuropathic bowel dysfunctionHistory• Frequency of bowel movements• Consistency of feces:

• Hard• Soft• Watery

• Current use of laxatives• Frequency of fecal incontinence• Child’s ability:

• To feel the urge to defecate• To sit on the toilet• To cooperate with bowel regimen or

program• Determine the child’s response to prior

treatments• Dietary measures• Digital rectal stimulation• Enemas• Suppositories


Evaluation of neuropathic bowel dysfunctionHistory• 2-week bowel diary

• Validated assessment of a child’s defecation habits• Although not mandatory, it is an excellent supplement to history taking

http://i-c-c-s.org/members/Clinical-Tools.cgi


Treatment: Neuropathic bladder & bowel


PharmacotherapyAnticholinergics

• Mainstay of drug therapy• Level I evidence

• Target muscarinic receptors• M2 & M3

• Systemic implications• M1-M5

• Improve bladder wall compliance• Diminish storage pressures• Convert NGB from high to low risk

• Abolishes detrusor overactivity• Provides time for CIC• Provides urinary continence M3 M2

M1M4 M2ACh

AChAnticholinergics


Pre-treatment Post-treatment

Anticholinergic effectsDetrusor overactivity


Anticholinergic effectsDetrusor compliance

Pre-treatment Post-treatment


PharmacotherapyBotulinum-A-Toxin• Inhibits ACh release at NMJ• Botox may modulate both sensory & motor pathways• Small, uncontrolled studies in children with NGB

• Improved clinical and urodynamic parameters: • Improved continence• Reduced max detrusor pressure • Increased detrusor compliance

• Not approved by FDA or the EMEA for the treatment of NBD• BTX-A use is off-label requiring informed consent

• FDA approval in adults 2011• Treatment of urinary incontinence due to DO associated with a neurologic

condition in adults who have an inadequate response to or are intolerant of an anticholinergic medication

• Spinal cord injury• Multiple sclerosis

• Adult Max dose = 200 U


PharmacotherapyAntibiotics• No level I evidence of medical benefit to using antibiotic

prophylaxis in children with NBD who perform CIC. • No difference in the rate of symptomatic or total UTIs• Alters the normal skin and bladder flora

• Increased selection of virulent bacterial isolates • Klebsiella and Pseudomonas

• Antibiotic prophylaxis – selective and individualized• Focus on better emptying with CIC


Catheterization• Non-latex catheters are employed exclusively• Cochrane Review - incidence of UTI

• Lack of evidence that one catheter type, technique, or strategy is better • Modification of catheters and catheter regimens should be made on an

individual basis for children with NBD


Neuromodulation therapyIntravesical electrical stimulation• Labor intensive & controversial• Only one randomized, placebo-controlled trial

• No efficacy demonstrated in children with NBD


Neuromodulation therapySacral nerve stimulation • Primarily been reported in the treatment of patients with

non-neuropathic bladder• Sacral nerve stimulation is considered investigational at

this time


Neuromodulation therapyBiofeedback• No significant studies of biofeedback have been reported in

children with NBD


Surgical intervention• Patients who fail medical management• Goals:

• Attaining safe bladder storage pressures & capacity• Increasing bladder outlet resistance


Attaining safe bladder storage pressures & capacity

• Urethral dilation• Mixed efficacy • Selected patients• Technically easiest

in females

• Vesicostomy• Excellent

temporizing procedure

• Ideal in infants and toddlers


Bladder augmentation• Achieves complete continence in children with neuropathic bladder• Allows independence & self-esteem• Requires patient commitment & compliance


Bladder augmentation•Definitive method of creating a safe, low-pressure storage•Small bowel•Most commonly employed•Large bowel•Ureter•Auto-augmentation


Bladder augmentation• Associated complications

• Acid-Base imbalances• UTIs• Stones• Bladder augment perforation• Cancer risk


Increasing bladder outlet resistance

• Variety of surgical approaches• Fascial sling• Artificial urinary sphincter• Bladder neck reconstruction• Bladder neck closure

Pump

Cuff

Reservoir


TreatmentNeuropathic bowel• High fiber diet• Digital stimulation / glycerin suppositories• Laxatives• Transanal irrigation – e.g. cone enema• Colonic irrigation

• ACE or MACE• Chait tube / Cecostomy tube


SummaryNeuropathic bladder & bowel documents• Provide a guideline for appropriate evaluation and timely surveillance of

the various neuro-urologic conditions that affect children• Underscore the variability and complexity of patients with NBD & bowel • Non-surgical intervention is promoted before undertaking major surgery

• CIC +/- anticholinergics are mainstay interventions• Dietary fiber, laxatives and enemas are common in bowel

management • Surgical intervention

• After failure of medical therapy• Requires patient commitment and compliance


Surgical reconstructionNeuropathic bladder & bowel


Bowel segments


Mitrofanoff principal

*


Surgical reconstructionNeurogenic bladder & bowel


Bowel segments Preparation


MontiCatheterizable channel


Bowel segments


Catheterizable channels & augmentation


Continence mechanism

How does it work?


MACE

• Malone• Antegrade• Continence• Enema


Refractory constipation• Neuropathic

bladder & bowel• Myelodysplasia

• Anorectal malformations


Patient selection• Refractory constipation

• Failed all “conservative measures”• Underlying pathology

• Chronic idiopathic constipation = poorly• Neuropathic bowel & anorectal malformations = good

• Age• > 5 yo = good results

• Compliance & Motivation


Continence mechanismMACE


Appendiceal mesenteryMACE


Mesenteric windowsDissection


Mesenteric windowsMACE


Pre-cecal wrapMACE


Cecal wrapMACE


MACE Cecal wrap


Mitrofanoff & MACE(Appendix)


Spiral Monti

Casale, J Urol, 162:1743, 1999


Spiral Monti


MACE alternativesAppendectomy


Colon tube


Appendiceal pedicleLimitations


Stoma constructionV-flap


StomasMACE & Mitrofanoff


Thank you!

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