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. Department of Surgery Division of Urologic Surgery ICCS Standardisation Documents

3. Department of Surgery Division of Urologic Surgery ICCS Standardisation Documents

4. Department of Surgery Division of Urologic Surgery 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. Department of Surgery Division of Urologic Surgery Objectives Neuropathic 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. Department of Surgery Division of Urologic Surgery Initial evaluation Determined by several factors: Timing of presentation or diagnosis – infancy vs. older child Etiology

7. Department of Surgery Division of Urologic Surgery Open spinal cord lesion Initial 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. Department of Surgery Division of Urologic Surgery Open spinal cord lesion Initial evaluation Renal & bladder U/S Screening for: Hydronephrosis, Ureteral dilation

9. Department of Surgery Division of Urologic Surgery Open spinal cord lesion Initial evaluation Renal & bladder U/S Screening for: Discrepancy in renal size or contour RK: 9.2 cmLK: 6.7 cm

10. Department of Surgery Division of Urologic Surgery Open spinal cord lesion Initial evaluation Renal & bladder U/S Screening for: Bladder wall thickness

11. Department of Surgery Division of Urologic Surgery Open spinal cord lesion Initial 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. Department of Surgery Division of Urologic Surgery Neuropathic bladder – Video-urodynamics

13. Department of Surgery Division of Urologic Surgery Follow-up of NBD dysfunction Newborn 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. Department of Surgery Division of Urologic Surgery Follow-up of NBD dysfunction Toddler 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. Department of Surgery Division of Urologic Surgery Follow-up of NBD dysfunction Adolescent to adult 2 nd 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. Department of Surgery Division of Urologic Surgery Follow-up of NBD dysfunction Adulthood RBUS every 3 years UDS Changes on RBUS Changes in continence Increased UTIs

17. Department of Surgery Division of Urologic Surgery Evaluation of neuropathic bowel dysfunction History 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. Department of Surgery Division of Urologic Surgery Evaluation of neuropathic bowel dysfunction History 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. Department of Surgery Division of Urologic Surgery Treatment: Neuropathic bladder & bowel

20. Department of Surgery Division of Urologic Surgery Pharmacotherapy Anticholinergics 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 M1M4M2 ACh Anticholinergics

21. Department of Surgery Division of Urologic Surgery Pre-treatmentPost-treatment Anticholinergic effects Detrusor overactivity

22. Department of Surgery Division of Urologic Surgery Anticholinergic effects Detrusor compliance Pre-treatmentPost-treatment

23. Department of Surgery Division of Urologic Surgery Pharmacotherapy Botulinum-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

24. Department of Surgery Division of Urologic Surgery Pharmacotherapy Antibiotics 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

25. Department of Surgery Division of Urologic Surgery 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

26. Department of Surgery Division of Urologic Surgery Neuromodulation therapy Intravesical electrical stimulation Labor intensive & controversial Only one randomized, placebo-controlled trial No efficacy demonstrated in children with NBD

27. Department of Surgery Division of Urologic Surgery Neuromodulation therapy Sacral nerve stimulation Primarily been reported in the treatment of patients with non-neuropathic bladder Sacral nerve stimulation is considered investigational at this time

28. Department of Surgery Division of Urologic Surgery Neuromodulation therapy Biofeedback No significant studies of biofeedback have been reported in children with NBD

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

30. Department of Surgery Division of Urologic Surgery 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. Department of Surgery Division of Urologic Surgery Bladder augmentation Achieves complete continence in children with neuropathic bladder Allows independence & self-esteem Requires patient commitment & compliance

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

33. Department of Surgery Division of Urologic Surgery Bladder augmentation Associated complications Acid-Base imbalances UTIs Stones Bladder augment perforation Cancer risk

34. Department of Surgery Division of Urologic Surgery Increasing bladder outlet resistance Variety of surgical approaches Fascial sling Artificial urinary sphincter Bladder neck reconstruction Bladder neck closure Pump Cuf f Reservoir

35. Department of Surgery Division of Urologic Surgery Treatment Neuropathic 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. Department of Surgery Division of Urologic Surgery Summary Neuropathic 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. Department of Surgery Division of Urologic Surgery Surgical reconstruction Neuropathic bladder & bowel

38. Department of Surgery Division of Urologic Surgery Bowel segments

39. Department of Surgery Division of Urologic Surgery Bowel segments

40. Department of Surgery Division of Urologic Surgery Mitrofanoff principal *

41. Department of Surgery Division of Urologic Surgery

42. Department of Surgery Division of Urologic Surgery Surgical reconstruction Neurogenic bladder & bowel

43. Department of Surgery Division of Urologic Surgery Bowel segments Preparation

44. Department of Surgery Division of Urologic Surgery Monti Catheterizable channel

45. Department of Surgery Division of Urologic Surgery Bowel segments

46. Department of Surgery Division of Urologic Surgery Catheterizable channels & augmentation

47. Department of Surgery Division of Urologic Surgery Continence mechanism How does it work?

48. Department of Surgery Division of Urologic Surgery MACE Malone Antegrade Continence Enema

49. Department of Surgery Division of Urologic Surgery Refractory constipation Neuropathic bladder & bowel Myelodysplasia Anorectal malformations

50. Department of Surgery Division of Urologic Surgery 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

51. Department of Surgery Division of Urologic Surgery Continence mechanism MACE

52. Department of Surgery Division of Urologic Surgery Appendiceal mesentery MACE

53. Department of Surgery Division of Urologic Surgery Mesenteric windows Dissection

54. Department of Surgery Division of Urologic Surgery Mesenteric windows MACE

55. Department of Surgery Division of Urologic Surgery Pre-cecal wrap MACE

56. Department of Surgery Division of Urologic Surgery Cecal wrap MACE

57. Department of Surgery Division of Urologic Surgery MACE Cecal wrap

58. Department of Surgery Division of Urologic Surgery Mitrofanoff & MACE (Appendix)

59. Department of Surgery Division of Urologic Surgery Mitrofanoff & MACE (Appendix)

60. Department of Surgery Division of Urologic Surgery Spiral Monti Casale, J Urol, 162:1743, 1999

61. Department of Surgery Division of Urologic Surgery Spiral Monti

62. Department of Surgery Division of Urologic Surgery Spiral Monti

63. Department of Surgery Division of Urologic Surgery Spiral Monti

64. Department of Surgery Division of Urologic Surgery MACE alternatives Appendectomy

65. Department of Surgery Division of Urologic Surgery Colon tube

66. Department of Surgery Division of Urologic Surgery

67. Department of Surgery Division of Urologic Surgery

68. Department of Surgery Division of Urologic Surgery

69. Department of Surgery Division of Urologic Surgery

70. Department of Surgery Division of Urologic Surgery

71. Department of Surgery Division of Urologic Surgery Appendiceal pedicle Limitations

72. Department of Surgery Division of Urologic Surgery Stoma construction V-flap

73. Department of Surgery Division of Urologic Surgery Stomas MACE & Mitrofanoff

74. Department of Surgery Division of Urologic Surgery Thank you!