Evaluation, treatment & intervention in the pediatric neuropathic bladder

1. Evaluation, treatment & intervention in the pediatric neuropathic bladder Paul F. Austin, MD, FAAP Professor of Urologic Surgery Department of Surgery Division of Urologic Surgery St. Louis Children’s Hospital Washington University School of Medicine

2. ICCS Standardisation Documents

3. ICCS Standardisation Documents

4. 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

5. 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

6. Initial evaluation Determined by several factors: Timing of presentation or diagnosis – infancy vs. older child Etiology

7. 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

8. Open spinal cord lesionInitial evaluation Renal & bladder U/S Screening for: Hydronephrosis, Ureteral dilation

9. 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

10. Open spinal cord lesionInitial evaluation Renal & bladder U/S Screening for: Bladder wall thickness

11. 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

12. Neuropathic bladder – Video-urodynamics

13. Follow-up of NBD dysfunctionNewborn to toddler Urodynamic studies High risk CIC +/- anticholinergics Low risk Diaper 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

14. 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

15. 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

16. Follow-up of NBD dysfunctionAdulthood RBUS every 3 years UDS Changes on RBUS Changes in continence Increased UTIs

17. 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

18. 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

19. Treatment: Neuropathic bladder & bowel

20. 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 ACh ACh M1 M4 M2 Anticholinergics M3 M2

21. Anticholinergic effectsDetrusor overactivity Pre-treatment Post-treatment

22. Anticholinergic effectsDetrusor compliance Pre-treatment Post-treatment

23. 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 forthe 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

24. PharmacotherapyAntibiotics No levelI 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

25. Catheterization Non-latex catheters are employed exclusively Cochrane Review - incidence of UTI Lackof 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

26. Neuromodulation therapyIntravesical electrical stimulation Labor intensive & controversial Only one randomized, placebo-controlled trial No efficacy demonstrated in children with NBD

27. 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

28. Neuromodulation therapyBiofeedback No significant studies of biofeedback have been reported in children with NBD

29. Surgical intervention Patients who fail medical management Goals: Attaining safe bladder storage pressures & capacity Increasing bladder outlet resistance

30. 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

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

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

33. Bladder augmentation Associated complications Acid-Base imbalances UTIs Stones Bladder augment perforation Cancer risk

34. Increasing bladder outlet resistance Variety of surgical approaches Fascial sling Artificial urinary sphincter Bladder neck reconstruction Bladder neck closure Pump Reservoir Cuff

35. 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

36. 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

37. Surgical reconstruction Neuropathic bladder & bowel

38. Bowel segments

39. Bowel segments

40. Mitrofanoff principal *

42. Surgical reconstructionNeurogenic bladder & bowel

43. Bowel segmentsPreparation

44. MontiCatheterizable channel

45. Bowel segments

46. Catheterizable channels& augmentation

47. Continence mechanism How does it work?

48. MACE Malone Antegrade Continence Enema

49. Refractory constipation Neuropathic bladder & bowel Myelodysplasia Anorectal malformations

50. 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