1. Children with Neurogenic Bladder: Who Needs Augmentation? Warren Snodgrass P.A.R.C. Urology

2. Goals of Management in Children Protect renal function Achieve urinary continence

3. Risk Factors for Renal Damage? Reflux and febrile UTI Not bladder pressures (DLPP, compliance ∆ P/ ∆ V) Shiroyanagi et al, 2009 Ozel et al, 2007 LeLair et al, 2007 Shiroyanagi et al, 2009

4. DLPP greater than 40cm? Based on single study of 42 MM pts 20 < 40cm: 0 VUR, 10% had dilated ureter on IVP 22 > 40cm: 68% VUR, 80% had dilated ureter on IVP age not stated medical management, if any, not described <40cm, all wet despite CIC + AC >40cm, all dry with CIC + AC McGuire et al, 1981

5. What Does Augmentation Do? Increases bladder capacity Decreases bladder pressures

6. Complications from Augmentation Urinary mucous Bladder stones Metabolic acidosis Growth retardation B12 deficiency Bladder rupture Malignancy

7. Alternatives to Augmentation? CIC (intermittent catheterization) reduces bladder pressures Anticholinergics increase functional bladder capacity decrease bladder pressures Medical Therapy

8. Medical Therapy Defined CIC q3hrs (approximating normal voiding schedule) Anticholinergics oxybutynin 0.2mg/kg/dose 3-4x/ day ± intravesical oxybutynin 5-10mg 2x/ day

9. Neurogenic Bladder Phenotypes 1. High storage pressures (>40cm) trabeculation, HN, VUR most respond to medical therapy augmentation if inadequate response (to adequate therapy)

10. 2. Sphincteric Incompetency smooth bladder, no HN, VUR bladder pressures <40cm bladder capacity uncertain (leakage during filling) remain wet with CIC + AC Bladder Neck surgery Role of augmentation uncertain

11. Lessons From the Past AUS without augmentation subsequent augmentation in 0-42% detrusor overactivity hydronephrosis decreased volume ± increased pressures Churchill et al, 1987 Spiess et al, 2002 Hafez et al, 2002 Lopez-Pereira et al, 2006

12. Was Augment Needed after AUS? Medical therapy pre-AUS not well described Medical therapy post-AUS not well described UD findings not stated More that 50% AUS patients were never augmented

13. Changing trends in management shift from AUS to slings for sphincter incompetency an era of near universal augmentation with BN surgery

14. protocol 2000 - 2014 BN repair without augment Indication: sphincteric incompetency neurogenic incontinence non-contractile bladder on medical therapy DLPP <50cm Patients consecutive children all who underwent BN surgery (no selection bias)

15. 85 children with neurogenic incontinence 37 BNS45 LMS 3 BNC 13 dry (37%) 22 not dry 7 BNC 1 BNC + augment 29 dry (66%) 13 not dry 13 BNC 23 BNC 15 dry (65%) 8 not dry 1 augment 6 augments 2000-2014

16. Augments after BNC bladder pressures ≥ 80cm + persistent bilateral g3 HN (n=1) or + new Mitrofanoff leakage (n=6) 5/6 change from <25 cm (n=4) or 40cm (n=1) occurred at a median of 21 months (6-72) Snodgrass & Granberg, 2016

17. Predictors for augmentation? no differences in preop UD % capacity end filling bladder pressures compliance indications were clinical findings EFP ≥80 cm despite tx + HN or incontinence

18. UD in Those Later Augmented Kronner (Cain) et alSnodgrass et al preop % cap 60% postop % cap 40% preop compliance 8 postop compliance 4 preop % cap 60% postop % cap 40%* preop compliance 4 postop compliance 2 end fill pressure preop 28 end fill pressure postop 80 * 20% of BNC patients not augmented had % capacity ≤ 40%

19. So who needs an augmentation? high pressure bladder with VUR and/or HN despite adequate medical tx high pressure bladder after BN surgery with persistent incontinence

20. ZR 6 year old male with SB Preop end filling pressure LMS in 2008 Annual F/u for 8 years Dry on CIC, AC No febrile UTI Normal renal US, cystograms Serum Cr 0.3mg/dl (age 13) Normal DMSA