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ICCS recommendations for pediatric NGB, from Campbell's

CDC recommendations for spina bifida, from Campbell's

Recommendations for inadequate storage, from Campbell's

Neural tube defects

• Obtain baseline RUS and VCUG as soon after birth as possible, decide whether to start CIC regimen based on results and PVRs
• Pre-UDS CIC: recommended for high residuals (≥ 30mL), trabeculated bladder, high grade reflux (Grade 5), and high grade hydronephrosis (SFU 3-4)
• Perform UDS within 3mo birth (usually after spinal cord closure)
• UDS findings: bladder/sphincter synergy (26%), dyssyngery (37%), or complete denervation (36%)
• Post-UDS CIC: recommended for DLPP > 40, DSD, or Grade 5 VUR, start oxybutynin 0.2mg/kg TID
• VUR: seen in 3-5% newborns, 30-40% by 5yo if untreated, treat with CIC + anticholinergics
• Occult dysraphisms: if not diagnosed by age 3yo, 92% develop UMN or LMN lesion (tethered cord, lipoma compression)
• Crede voiding: avoid, increases reflux and potential for upper tract damage
• Cystatin C: serum levels provide more accurate estimate of GFR
• Bowel function: managed with laxatives, enemas, frequent BMs, and manual stimulation
• Antegrade continence enema (ACE): administered via appendicostomy or cecostomy, 1-2L fluid 4-7x weekly
• Sexual function: 70-92% can obtain erection, 40-75% can ejaculate, 8-83% men and 23-69% women are sexually active, 70-80% able to achieve pregnancy
• Management of Myelomeningocele Study (MOMS): prenatal surgery reduces fetal/neonatal death or CSF shunting (RR 0.7), but 40% still required shunting and pregnancy complications were higher and had higher risk for spinal cord tethering

Other neuromuscular disorders

• Sacral agenesis: MRI confirms acute cutoff of conus at T12, obtain RUS/VCUG, manage based on UDS findings
• Anorectal malformations: GU abnormalities more likely if supralevator (vs infralevator) fistula insertion, often have DO + DSD, obtain RUS, obtain UDS if bony abnormalities, spinal cord defect, or RUS/VCUG abnormalities
• Post-pelvic surgery: due to splanchnic/hypogastric nerve damage, preop/postop UDS recommended if high risk for injury during surgery
• Cerebral palsy: 16-94% have GU symptoms, mainly incontinence, upper tract injury is rare, manage based on individual picture
• Spinal cord injury: acontractile bladder in acute setting, then may develop DO + DSD, minimal regain of function if cauda equina injured, protect upper tracts
• Transverse myelitis: urinary retention common in acute setting, then develops into DO + DSD, 57-73% require CIC

Management Options

• Goals (in order of priority): protect upper tracts, maintain continence, maintain independence, avoid reconstructive surgery
• Surgical management categories: increase bladder size/compliance, increase bladder outlet resistance, create catheterizable channel
• Most concerning finding is filling pressures > 40, increases risk for upper tract injury
• Anticholinergics: improves capacity/compliance, decreases incontinence and catheterization episodes, side effects (dry mouth, constipation, blurred vision, dizziness, headaches) can lead to decreased compliance, no evidence cognitive impairment in children
• Sympathomimetics: improve urethral resistance, but may cause dizziness, nausea, insomnia, headache, retention
• Botox: 65-87% dryness after failing medical management + CIC, 360U max dose
• Augmentation: for poorly compliant small capacity bladders, 0-29% require continued anticholinergics, 15% develop major complications (SBO, transfusions, perforation, UTI/pyelo), cystography diagnostic for augment perforation, 0.6% cancer risk
• Vesicostomy: used for severe reflux despite CIC, CIC non-compliance, poor augment candidates, complications include prolapse, stenosis, bladder stones
• Artificial urinary sphincter: improves outlet obstruction, continence 63-86%, cuff erosion 16-20%, worse bladder function 4-30%, device lifespan 4.6-12.7yrs
• Bladder slings (fascial and synthetic): improves outlet obstruction, continence 30-93%, new detrusor overactivity 33%
• Bladder neck reconstruction (Young-Dees-Leadbetter): excise and tighten bladder neck to maintain continence
• Catheterizable channels (Mitrofanoff, Yang-Monti): use appendix or intestine to create a continent reservoir to make CIC easier