What is the standard treatment for spina bifida?

Standard Treatment for Spina Bifida

Surgical closure of the spinal defect should be performed within 24-72 hours of birth, with immediate closure (within 24 hours) providing optimal outcomes for bladder function and decreased risk of complications. 1

Initial Management

Prenatal Management

• Prenatal diagnosis and intervention:
  • Prenatal repair of myelomeningocele is recommended for fetuses meeting Management of Myelomeningocele Study (MOMS) criteria to reduce the risk of shunt-dependent hydrocephalus (Level I evidence) 2
  • Prenatal closure has shown decreased need for ventriculoperitoneal shunting and improved lower extremity motor outcomes 2
  • However, these benefits must be weighed against increased risks of preterm delivery and uterine dehiscence 2

Postnatal Surgical Management

• Timing of surgical closure:

  • Closure within 24-72 hours of birth is critical
  • Delaying beyond 72 hours increases risk of febrile UTIs, vesicoureteral reflux, hydronephrosis, and worse urodynamic parameters 2, 1
  • Immediate closure (within 24 hours) results in improved bladder capacities and decreased detrusor leak point pressures 1

• Surgical goals:

  • Close the defect to prevent infection and CSF leakage
  • Preserve neural function
  • Untether the spinal cord
  • Address cosmetic concerns 1

Multidisciplinary Follow-up Care

Neurological Management

• Regular monitoring for hydrocephalus and Chiari II malformation
• Ventriculoperitoneal shunting if needed (86% of patients require CSF diversion) 3
• Monitoring for tethered cord syndrome (32% of patients require release) 3
• Yearly neurological assessment for high-risk cases 1

Urological Management

• Two main approaches exist: proactive and expectant management 2

  • Proactive approach: Early and regular urodynamic testing with clean intermittent catheterization (CIC) and/or pharmacotherapy before upper tract changes develop
  • Expectant approach: Monitoring with periodic ultrasound, implementing urodynamics and CIC only when clinical deterioration or hydronephrosis develops

• Regular urodynamic studies to assess bladder function

• Clean intermittent catheterization (CIC) for neurogenic bladder

• Anticholinergic medications for detrusor overactivity 1

Orthopedic Management

• Monitoring for scoliosis (49% of patients develop scoliosis, with 43% requiring spinal fusion) 3
• Management of lower limb deformities and mobility issues

Long-term Care Considerations

Transition to Adulthood

• Comprehensive transition planning from pediatric to adult care
• Focus on independence, sexual health, and development of support systems 2
• Educational and vocational planning (85% attend or graduate from high school/college) 3

Complication Prevention

• Latex-safe environment for all medical-surgical-dental procedures due to high risk of latex allergy (approximately 1/3 of patients) 1, 3
• Monitoring for secondary tethered cord syndrome
• Regular follow-up for shunt function (95% require at least one shunt revision) 3

Prognosis

• Approximately 75% of children born with myelomeningocele reach early adulthood 3
• Late deterioration is common, emphasizing the need for lifelong multidisciplinary care
• More than 80% achieve social bladder continence 3

Emerging Therapies

• Human umbilical cord-derived mesenchymal stromal cell-derived extracellular vesicle (UC-MSC-EV) therapy shows promise in reducing intrathecal scarring following spina bifida surgery 4
• Further research is needed to evaluate long-term impact of in-utero intervention and refine techniques using tissue engineering technology 5

Common Pitfalls in Management

• Delayed surgical closure beyond 72 hours significantly increases complications
• Inadequate transition from pediatric to adult care
• Failure to maintain latex-safe environments during procedures
• Insufficient monitoring for late-developing complications like tethered cord syndrome
• Fragmentation of care, especially following prenatal repair 6