Spina bifida Stephen D Mark Christchurch Hospital Christchurch

Disability not Inability Major effect on Quality of Life

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

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

Incidence/prevention Reduced incidence Improved nutrition Folate supplement Theraputic abortion

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

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

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……….

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

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

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

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

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

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

Skin sling harvest

Bladder neck dissection + Bi valve bladder

Skin Sling sutured

Skin sling wrapped round bladder neck

Cinch bladder neck wrap

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

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

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

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

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…

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 : Mt Sainai J Med 2011 Sept 759