1. Spina bifida Stephen D Mark Christchurch Hospital Christchurch

2. Disability not Inability Major effect on Quality of Life

3. Expectations Survival is a given Options available Resources limited Specialized care Community based

4. Objectives Incidence/prevention Decision making Multidisciplinary team Urologic management Future prospects….

5. Incidence/prevention Reduced incidence Improved nutrition Folate supplement Theraputic abortion

6. Early management Back closure: improved surgery reduced disability Multi disciplinary inputs…. Renal US: Kidneys, voiding function Antibiotic prophylaxis Goals: Quality and Length of Life Renal Preservation Prevent UTI Continence ( Bowels) Latex precautions

7. Decision making in Urologic therapy Timing and Type is controversial Motivation and mobility Support IQ may be reduced Hand function Local expertise

8. Early vs Delayed Early Ab Prophylaxis Anti cholinergics CIC Renal US ( baseline) MCU UDS Manage to make bladder Safe (Pdet < 30 cm H20) Proactive treatment – Late – Ab Prophylaxis – Renal US / MCU – Interval US – CIC for emptying – Anticholinergics prn – UDS prior to surgery – Reactive treatment WHAT I DO……….

9. Urologic management Emptying: CIC ( Lapides 1970) Urethral / Mitrofanoff Storage Bladder Anticholinergics Botox Augmentation Outlet Sling Injectable AUS Closure BN Note: Bowel management first

10. Bowel management In parallel with urinary tract Regular complete emptying Laxative Enema ACE : Issues: Fluid type and volume Caecum vs Lt Colon

11. CIC : Outlet / Mitrofanoff Lapides 1970’s Safe bladder emptying Urethra is not sensate Mitrofanoff in wheel chair patient Complications rare… stricture, incontinence Goal: Competent outlet that can be emptied

12. Bladder storage Goal: Low pressure high volume bladder Voluntary voids: ( n=10/80) Anticholinergics ( Oxybutinin ) ( n= 40/80 ) Botox ( n=1/80 ) Ileal augmentation Other bladder reconstruction

13. Bladder augmentation 34/80 2 died in F/U (UTI) 32 stable renal function 1 re-augmentation 10 Mitrofanoff 4 re-operation Complications: 8 bladder calculi 1 perforation 1 laparotomy 1 Latex allergy Bladder Storage : Ileal augmentation Ileal conduit : rare but still a place

14. Bladder storage: Outlet procedure Static resistance: Injectable, Sling ( skin ), reconstruction Dynamic resistance: Artificial sphincter N = 6 ( 1 bulbar ) 1 erosion : dry after removal 1 malfunction 4 success

15. Skin sling harvest

16. Bladder neck dissection + Bi valve bladder

17. Skin Sling sutured

18. Skin sling wrapped round bladder neck

19. Cinch bladder neck wrap

20. Skin sling outcomes 19 patients, 11 boys. Age mean 10 yrs Follow up: 8 years, Sx, Renal US and UDS 14/19 dry on CIC Durable outcome No long term complications

21. My Preference Goal: Independence, QoL, Renal preservation, prevent UTI and Continent Early renal dilatation: CIC, Anticholinergics +/- augmentation Bowels OK, supportive situation. RUS follow up Incontinence stable kidney growth: CIC, Anticholinergics. Review… Persistant wetting skin sling and Ileal augmentation. Mitrofanoff if in wheelchair

22. Adolescent Transition Transfer chronic paediatric condition to adult care Independence Complex psychological / physical issues Different personnel, decision making Traumatic Formal handover beneficial with local practitioners

23. Expensive: Wellington study NZMJ 2012, Mar 125 : Bowkett et al Reviewed 6 consecutive spina bifida patients, 10 – 21 yrs. 124 operations ( Av 20 ) 8.5 Days Av LOS ( 1066 total ) 7.5 CT scans $ 944,000/ patient

24. Future Issues Reduced incidence Antenatal intervention study ( MOMS ) 2018 Tissue engineering, Xiao procedure Increased patient expectation Adolescent transition/ Pregnancy, / Adult life Long term follow up unknown ( Malignancy risk ) Remember: ….a simple intervention can make a substantial difference…

25. Adam Hall : Gold medallist Ref: 1: Bowkett et al NZMJ 2012 Mar P 125 2: Wilson RD et al J Obstet Gynaecol Can 2007 Mar 193 3: Bauer J Urol 2011 1437 4: Mt Sainai J Med 2011 Sept 759