What is the treatment plan for Spina Bifida?

Treatment Plan for Spina Bifida

Spina bifida requires immediate postnatal surgical closure within the first 2 days of life, or alternatively prenatal repair before 26 weeks' gestation in selected cases, followed by lifelong multidisciplinary management addressing neurological, urological, and bowel complications.

Initial Surgical Management

Timing of Defect Closure

• Postnatal repair should occur within the first 2 days of life to minimize infection risk and prevent further neurological damage 1
• Prenatal repair before 26 weeks' gestation is an alternative for selected patients meeting MOMS trial criteria, which reduces shunt-dependent hydrocephalus and reverses hindbrain herniation, though it carries significant maternal risks including preterm labor and premature rupture of membranes 2, 3, 4
• Birth at a higher-level nursery care hospital facilitates timely repair, as lower-level facilities are associated with delayed surgical intervention 1

Hydrocephalus Management

• Monitor closely for shunt-dependent hydrocephalus, which occurs frequently in myelomeningocele patients 2
• Ventricular shunting is required for most patients with hydrocephalus to prevent progressive neurological deterioration 3, 4
• Prenatal repair reduces but does not eliminate the need for shunting 2, 4

Urological Management Strategy

Proactive Surveillance Approach

• Initiate regular urodynamic testing early to detect bladder dysfunction before upper tract changes develop 2, 5
• Begin clean intermittent catheterization (CIC) and anticholinergic medications proactively rather than waiting for complications 6, 2
• Delayed CIC initiation is an independent risk factor for renal cortical loss and represents a critical pitfall to avoid 2

Medical Management

• Both expectant and proactive approaches protect against renal injury, but delayed intervention increases bladder augmentation rates 6
• The controversy between approaches is resolved by evidence showing proactive management prevents upper tract deterioration 2, 5

Surgical Intervention Indications

• Surgical reconstruction is indicated when upper tract changes or renal deterioration occur despite maximal medical management 6, 5
• Enterocystoplasty remains the gold standard for bladder augmentation in patients with hostile bladder, despite complications including infections, metabolic abnormalities, and perforation risk 6
• Catheterizable channels to the bladder and colon facilitate continence and promote independent living 6

Neurological Surveillance

Tethered Cord Monitoring

• Lifelong surveillance for tethered cord syndrome is mandatory regardless of initial repair type 2, 5
• Loss of ambulatory function is a key indicator of cord tethering requiring immediate evaluation 2
• High risk of tethering exists even after successful initial repair 2

Associated Conditions

• Monitor for Arnold-Chiari II malformation complications including brainstem dysfunction and sleep apnea 7, 8
• Assess for hydrosyringomyelia and scoliosis development throughout childhood 7

Bowel Management Algorithm

Follow a stepwise escalation approach 2:

1. Start with dietary modifications, laxatives, and suppositories
2. Progress to manual evacuation if needed
3. Advance to retrograde enemas for persistent issues
4. Consider Antegrade Continence Enema (MACE) procedure or colostomy for refractory fecal incontinence 2

Fecal incontinence significantly impacts quality of life and requires aggressive management 2

Transition to Adult Care

• Structured transition programs from pediatric to adult services are essential 2, 5
• Begin attention to independence factors early in childhood to facilitate successful transition 2
• Adult care must address spina bifida-specific complications including pressure sores, lymphedema, sexual dysfunction, and ongoing urological/renal monitoring 8
• Regular follow-up with urology, neurosurgery, and physiatry specialists is necessary for nearly all adults with spina bifida 8

Critical Pitfalls to Avoid

• Delaying urological intervention until upper tract changes occur increases morbidity and need for major reconstruction 2, 5
• Birth at lower-level care facilities delays timely surgical repair 1
• Inadequate transition planning leaves young adults without appropriate specialist care 2, 5
• Underestimating the impact of executive dysfunction and nonverbal learning disability on self-management in adults 8