Contemporary Urologic Management of Children with Neurogenic Bladder Patricio C. Gargollo, MD Director, Pediatric Urology Minimally Invasive and Robotic Surgery Assistant Professor in Urology, UT Southwestern Medical School Department of Urology, Children's Medical Center, Dallas Who am I and how did I get here? • Baylor University Graduate • Harvard Medical School • Massachusetts General Hospital and Harvard Medical School – 2 years general surgery – 4 years urology • Children’s Hospital Boston – 3 years pediatric urology – Advanced fetal care center – Advanced Laparoscopic training Paradigm Shift • Medical Therapy and Management – – – – Less Antibiotics Less Radiation Less Screening Less Testing • Surgical therapy – Laparoscopic Surgery – Robotic Assisted Surgery • Less Pain • Less Scars • Less Time in the hospital Outline • • • • • • Urology Goals Physiology Bladder Function/Malfunction Bowel Function/Malfunction Urology Studies Surgical Treatments Spina Bifida Classification Myelomeningocele Meningocele Lipoma of the cord Occulta Etiology Risk Factors Sex Ethnic Background Diet Medications Diabetes Obesity Socioeconomic status Prevalence 166,000 affected in the US 1 in 1,000 live births Texas Scottish Rite • 500 active patients with MM • 25 newborn patients annually Spinal Defects Clinic • • • • • • Integrate care among all specialties Provide “one-stop” shopping Patient Population: 500 patients Tuesday s 14-18 patients 12:30-6 pm Patients 1 month-2 years old – Seen every 3-6 months • Patients 2 years and older – Seen every 6 months to 1 year Spinal Defects Clinic Providers • Specialists: – Physiatrist – Orthopedist – Neurosurgeon – Urologist – Occupational Therapy – Physical Therapy – Social Work – Nursing – Project Nicaragua NGB: CHILDHOOD MILESTONES birth - toilet training (3-4 yrs) continence management (TT- middle school) teenage rebellion transition to adult care Goals • Preserve renal function – No dialysis! • Achieve social continence – – – – Bladder Bowel No diapers! Independence Neural Pathway Bladder Function Bladder • Overactive • Underactive • Normal Sphincter •Overactive •Underactive •Normal Detrusor Sphincter Dyssynergia Bladder -Overactive Sphincter -Overactive Neurogenic Detrusor Overactivity Bladder -Overactive Sphincter -Underactive Areflexic Bladder Bladder Underactive Sphincter -Underactive Bowel Function Bowel Function: “Pellets” Bowel Function:Diarrhea Urology Studies • • • • Renal/Bladder ultrasound VCUG DMSA Urodynamics Urology Studies • Renal/Bladder ultrasound Urology Studies • Renal/Bladder ultrasound Urology Studies • VCUG (Voiding cystourethrogram) Urology Studies • DMSA Urology Studies • UDS (Urodynamics) Bladder Pressure Sphincter Activity Rectal/Abdominal Pressure Pressure Time Activity Time Pressure Time Management and Outcomes • No longitudinal studies of renal function, scarring • Few longitudinal studies of bladder compliance Means to Assess Need for therapy, results, determined by: • Imaging Renal US VCUG DMSA • Urodynamics Background • Goals for management: – Preserve renal function, prevent scarring – Preserve bladder compliance • No evidence that management impacts outcomes • Reported endpoints – New HN, VUR – Change in UD – Augmentation rates Management Options • 3 options for management of children with MM from birth – age 3y: – Imaging-based observation – Universal therapy (CIC + anticholinergic) – UD-based selective therapy Surrogate Outcomes of Management • Incidence of new HN, VUR does HN or VUR predict renal damage? • Development of adverse UD parameters does tx prevent changes? does tx restore compliance? • Augmentation rates management failure vs management decision? Newborns: Tx vs Observation • No evidence shows universal treatment superiority • No study shows impact of tx on care-givers • Cost catheters, oxybutynin Newborn Protocol • ≤ 6 wks age Fluoroscopic UD Renal US, DMSA • Renal US q 3mos x1y q 6mos UD, DMSA 1yr, 3yr • Tx for: high risk UD + HN, VUR new HN, VUR, ∆ DMSA • High Risk UD filling pressure> 40cm Patterns: Initial Assessment: UD • Varying Methods 5-7Fr UD catheters infusion 1.5- 15cc/min monopolar needle vs patch electrodes EMG • Varying Terminology upper, lower motor lesions detrusor hypertonicity vs overactivity • Varying Diagnoses DSD vs no DSD Results: Initial UD 71 pts, mean age 3m (2wk – 6m) Category Number of pts “Normal” 16 (23%) No detrusor contraction 22 (31%) <25 cm H2O 9 25-40 cm H2O 9 >40 cm H2O 4 Detrusor overactivity 33 (46%) <25 cm H2O 12 25-40 cm H2O 8 >40 cm H2O 13 Results: Initial UD 71 pts, mean age 3m (2wk – 6m) Category Number of pts “Normal” 16 (23%) No detrusor contraction 22 (31%) <25 cm H2O 9 25-40 cm H2O 9 >40 cm H2O 4 Detrusor overactivity 33 (46%) <25 cm H2O 12 25-40 cm H2O 8 >40 cm H2O 13 “High risk DLPP or Storage Pressure? Same risk? DLLP 50 cm Pressure during storage is more important than compliance Churchill et al, 1994 Selective Therapy (UD-based) • UD identifies high risk before deterioration • Therapy prevents renal, bladder damage • Preserve renal function, decrease augmentation Outcomes 71 pts Initial UD High risk UD 17 (24%) F/u UD F/u UD Treatment n=12 Observation n=5 EFP <40 n=12 1 new HN No new HN/VUR Low risk UD 54 (76%) 6/54* Δ to risk UD 1 new HN+VUR * UD changes at mean 9mo (4-12) 1 new HN, 2 new VUR Outcomes • Renal damage: • 25% f UTI: no data, f/u DMSA pending 9/17 (53%) high risk 9/54 (17%) low risk 10/18 (56%) CIC vs 8/53 (15%) obs , p=.001 • 18% VUR: 11/71 (15%) initially 3/60 (5%) new Renal Outcomes: Baseline DMSA 38 patients – 35 (92%) normal scan – 3 (8%) abnormal scan, congenital nephropathy? Pt DMSA finding 1 Initial UD Pattern EFP Initial u/s Initial VCUG fUTI Unilateral, CRN 20 No hydro No VUR No 2 Unilateral, focal scar 40 No hydro No VUR No 3 Unilateral, CRN 62 Unilateral SFU Gr 3 Gr 5, 3 Yes Renal Scar: Risk Factors 95 pts NGB 7±4yrs 32% DMSA renal scar MLR analysis: VUR OR 8.12 (95%CI 2.92 – 23.14) no UD parameter bladder capacity DLPP>40cm H2O [40% taking anticholinergics] DSD detrusor overactivity Leonardo et al, 2007 Renal Scar: Risk Factors DMSA, UD in sequential pts 2005-07 113pts, 64 > 10ys age studied 16 (25%) had abnormal DMSA function < 40%, or focal scar • VUR OR 2.06 (1.43 – 2.97) • f UTI OR 9.53 (2.64 – 34.34) • DLPP 44±20 vs 46±28 • Compliance 8.8±5.9 vs 12±11 ns ns Shiroyanagi et al, 2009 Renal Scar (non-NGB) 15% focal DMSA defect • 15% VUR I-III 50% VUR IV-V • Recurrent fUTI 541 consecutive pts fUTI and/or VUR Results: Initial U/S, VCUG • 14/71 (20%) abnormal HN 3 (4%) VUR 8 (11%) HN+VUR 3 (4%) Results – 18/71 (25%) had treatment by 1 year 12 initial “high risk” 6 initial “low risk” – new loss of compliance – 14/71 (19%) VUR 11/71(15%) initially 3/60 (5%) new – 18 (25%) with febrile UTI 10/18 (56%) CIC vs 8/53 (15%) obs, p=0.001 Conclusions • Majority of infants have low risk UD findings • 83% of low risk pts have no change in UD or imaging during observation • Compliance changes occurred before age 1yr • Treated -risk patients lowered bladder pressures – No data yet on renal impact • Initial management can be tailored by initial UD Conclusions • ~25% newborns have potentially adverse imaging and/or UD ~15% VUR • ~10% have potentially adverse changes during obs • Scar risk of fUTI ± VUR not known with NGB • Potentially negative impact of CIC on renal function (fUTI) Summary of Outcomes • Some pts with “normal” or “low risk” UD will convert to “high risk” • Some pts with “high risk” UD have no clinical findings Uncertain: Is high bladder pressure alone a risk factor for renal damage? Can therapy (CIC) cause renal damage, ie via febrile UTI? Management • Medical Management – Intermittent Catheterization – Anticholinergics • Surgical Management – – – – Bladder Procedures Bladder Outlet Procedures Catheterizable Channels Procedures on the ureters Neurogenic Voiding Dysfunction Good bladder Bad sphincter Good bladder Good sphincter A. B. Bad bladder Bad sphincter Bad bladder Good sphincter C. D. Goals Medical Social Surgical Intervention • Last resort when medical therapy fails: – Botox, Augmentation +/– BN procedure : injection, suspension, sling, urethral lengthening ((Piipi Salle, Kropp), AUS… last resort is BN closure – Mitrofanoff- Monti-Yang +/– Reimplant +/– Malone ACE Pediatric Reconstruction: Key Points • In children- try to preserve bladder, not divert • Detubularize & reconfigure bowel: avoid hour glass! – Intact bowel P- 60-100 cm H2O • Maintain terminal 10-20 cm distal ileum (B12 absorption – megaloblastic anemia, peripheral neuropathy, optic atrophy, dementia) • Bladder neck closure as last resort only • Consider MACE & Mitrofanoff Treatment:Bladder CIC: Clean intermittent catheterization CIC: Clean intermittent catheterization DOES NOT INCREASE INFECTIONS IF DONE CORRECTLY!!!!!!! Treatment:Bladder CIC: Clean intermittent catheterization Surgery:Bladder Bladder Botox Surgery:Bladder Augmentation Surgery:Bladder Augmentation Surgery:Bladder Augmentation Surgery:Bladder Augmentation Surgery: •Increase bladder size •Decrease high pressures to kidneys Results: •Prevent kidney damage •Continence Intra-op Intra-op Intra-op Intra-op Catheterizable Stoma Monti-Tube Appendicovesicostomy Surgery:Mitrofanoff Surgery:Mitrofanoff Post-op Care Mitrofanoff or ACE Midline/Umbilicus Suprapubic Tube RLQ or LLQ ACE Midline or RLQ Urethral Foley Post-op Care Mitrofanoff or ACE Suprapubic Tube ACE 1. Locations and origins may differ 2. Bag drainage and plugs may differ Urethral Foley Post-op Care Flushing “In” VS 1) ACE Procedure 2) Can be tap water 3) Sit patient on toilet/bedside commode 4) Serial increase in volume Irrigation “In and Out” 1) Bladder only 2) Via Mitrofanoff, SPT or urethral foley 3) Additional catheters must be closed 4) Sterile water or saline 60 cc BID 5) This can be tricky but it’s important! POD#1: AMBULATION Routine Care:FAQs 1. How far does the ACE/Mitrofanoff go in? 2. Can I hurt anything? 3. How long does it take to heal? 4. What are the outcomes? 5. What are the risks? Key Points – – – – – Short term and long term issues Behavior and diet changes Many surgeries and treatments Intense post-operative care and teaching Requires both family and nursing support Surgical Management Minimally Invasive Pediatric Surgery • Shift –Extirpative • Nephrectomy – Reconstructive • Ureteral reimplant, augmentation, complex Reconstruction Robotic Assisted Continent Catheterizable Conduit Appendicovesicostomy/ ACE 1 2 X 3 1 10 cm 2 1: 8mm working port, mid-clavicular line 1750 2: 12mm camera port, midline 3: 8mm working port, mid-clavicular line 10 cm X: 5mm port for sutures 3 Bagrodia, A., Gargollo, P.: Robot-assisted bladder neck reconstruction, bladder neck sling, and appendicovesicostomy in children: description of technique and initial results. J Endourol, 25: 1299, 2011 Complex Reconstruction Neurogenic Incontinence • Various surgical techniques • Bladder neck sling for incontinence first described in 1986 • Sling without augmentation demonstrated to be safe – Continence rates are low (36-57%) • Sling with bladder neck reconstruction safe, with 82% continence (Snodgrass J Urol 184, p 1775, 2010) Methods: Technique Results Results: Patient Characteristics Case 1 2 3 4 5 6 7 Age (years) 8 13 13 5 11 7 8 Sex F F M F F F M BMI (kg/m2) 24.5 27.1 29 16.7 31.2 14.8 20.2 Diagnosis Shunt MMC MMC MMC LMC MMC N Y N N Y N N Tranverse myelitis BMI: Body Mass Index, Shunt: Ventriculoperitoneal shunt SCI Results: Cumulative outcomes • 86% of cases completed robotically • One complication (conversion) • Two cases of de novo reflux (resolved) Efficacy, efficiency, safety of robotic APV/BNR/BNS • Efficacy: – All patients are dry – Low profile scars • Efficiency: – Operative times are longer – Hospital durations are shorter • Safety: – Acceptable complication rate Complex Reconstruction • Gargollo et. al. Comparison of Open and Robotic Assisted Appendicovesicostomy, Bladder Neck Reconstruction and Bladder Neck Sling IRUS, January 2011 • Robotic Cohort – Longer operative times – Lower Blood loss – Lower length of stay – Decreased Narcotic Use Conclusions • The present series expands the scope of robotic reconstruction in children • Preliminary data demonstrates these procedure are feasible and safe • Comparison with open APV with bladder neck reconstruction is required and ongoing Thank you for your attention