What is the recommended management for mild spina bifida?

Management of Mild Spina Bifida

For mild spina bifida (myelomeningocele), implement proactive urological surveillance with early clean intermittent catheterization (CIC) based on urodynamic findings to preserve renal function, while monitoring for upper tract changes with regular ultrasound and urodynamic testing starting in the newborn period. 1

Initial Newborn Management

Bladder drainage and catheterization protocol:

• Drain the bladder initially via indwelling Foley catheter or intermittent catheterization postnatally 1
• Once the infant can be moved from prone position, initiate CIC every 6 hours to determine residual bladder volumes 1
• Continue catheterization until bladder volumes are less than 30 ml on the majority of catheterizations for 3 consecutive days, then decrease frequency 1
• If residual volumes remain adequately low, discontinue CIC; if not, continue every 4 hours while awake 1
• Teach all parents/caregivers intermittent catheterization techniques regardless of initial bladder status, as 80% of individuals with myelomeningocele ultimately require long-term CIC 1

Timing of neurosurgical closure:

• Close the spinal defect within 24 hours of birth to achieve improved bladder capacities and decreased detrusor leak point pressures 1
• Delaying closure past 72 hours increases the incidence of febrile UTIs, vesicoureteral reflux, hydronephrosis, and worse urodynamic parameters 1

Proactive vs. Expectant Management Approach

Proactive management (recommended for mild cases):

• Perform early and regular urodynamic testing 1
• Start CIC with or without pharmacotherapy early based on urodynamic findings, before development of upper tract changes 1
• This approach prioritizes renal preservation by addressing hostile bladder dynamics before irreversible damage occurs 1

Key urodynamic findings requiring intervention:

• Detrusor overactivity on urodynamic evaluation warrants oxybutynin 0.2 mg/kg orally three times daily 1
• Monitor for high detrusor leak point pressures that threaten upper tract integrity 1

Surveillance Protocol Throughout Childhood

Regular monitoring schedule:

• Perform periodic ultrasound to detect upper tract changes (hydronephrosis) 1
• Conduct serial urodynamic studies to assess bladder compliance and detrusor pressures 1
• Monitor serum creatinine and renal function to detect early chronic kidney disease 1

Age-specific treatment goals:

• Newborn period: Focus primarily on preserving renal function 1
• School age: Add urinary and fecal continence as treatment goals alongside kidney preservation 1
• Adolescence/emerging adulthood: Incorporate independence and sexual function as relevant goals 1

Medical Management

Anticholinergic therapy:

• Use oxybutynin 0.2 mg/kg orally three times daily for detrusor overactivity identified on urodynamics 1
• Note that anticholinergics do not address the underlying neurological problem, and detrusor overactivity returns after discontinuation 1

Conservative continence management:

• Up to 50% of patients followed conservatively with CIC with or without pharmacotherapy through puberty spontaneously achieve continence 1
• This represents a significant proportion who may avoid reconstructive surgery with advancing age 1

Bowel Management Considerations

Stepwise approach to fecal incontinence:

• First-line: Dietary modifications, laxatives, suppositories, and/or manual evacuation (successful in approximately 50% of cases) 2
• Second-line: Retrograde enemas (75% success rate for achieving continence) 2
• Third-line: Antegrade Continence Enema (MACE) procedure (77% achieve complete or near-complete fecal continence) 2
• Fourth-line: Colostomy when other approaches fail (84% of patients would choose the procedure again despite 2% mortality risk) 2, 3

Long-Term Follow-Up

Multidisciplinary care requirements:

• Almost all adults with spina bifida benefit from regular follow-up with specialists in urology, neurosurgery, and physiatry 4
• Annual follow-up care appointments at specialized spina bifida clinics are recommended 5
• Monitor for secondary health conditions including hypertension, hyperlipidemia, pressure sores, lymphedema, and sexual dysfunction 4, 6

Common adult complications requiring surveillance:

• Urinary tract infections (46% prevalence) 7
• Scoliosis (30% prevalence) 7
• Chronic pain (28% prevalence) 7
• Metabolic syndrome and obesity 6

Transition to Adult Care

Structured transition program components:

• Build trust and personal relationship with pediatric urologist as the most important factor for successful transition 1
• Arrange meetings with both adult and pediatric medical and nursing staff together 3
• Address independence, self-care, and sexual function proactively 1
• Recognize that only 24% of emerging adults with spina bifida achieve independent living 1
• Discuss sexual function (erectile dysfunction treatable with sildenafil in 80% of males) despite only 23-39% currently receiving counseling 1