What is the standard treatment for spina bifida?

Standard Treatment for Spina Bifida

For fetuses meeting MOMS criteria, prenatal surgical repair is the standard of care to reduce shunt-dependent hydrocephalus and improve neurological outcomes, while postnatal cases require immediate surgical closure within 48-72 hours of birth followed by lifelong multidisciplinary management of neurological, urological, and orthopedic complications. 1

Prenatal Management

Fetal Surgery Criteria and Approach

• Prenatal repair is recommended (Level I evidence) for fetuses meeting Management of Myelomeningocele Study (MOMS) criteria to reduce the risk of shunt-dependent hydrocephalus by approximately 40%. 1
• Prenatal repair improves lower neuromotor function and reduces hindbrain herniation compared to postnatal repair. 2
• Three surgical approaches exist: open maternal-fetal surgery (traditional MOMS approach), laparotomy-assisted fetoscopy, and percutaneous/mini-laparotomy fetoscopy, with the latter minimizing maternal morbidity while maintaining fetal benefits. 3

Key Maternal-Fetal Considerations

• Prenatal surgery carries maternal risks including uterine dehiscence, preterm labor, and need for cesarean delivery in current and future pregnancies. 2
• The percutaneous fetoscopic approach reduces anesthesia risk and improves maternal recovery compared to open fetal surgery, though may have higher preterm birth rates than laparotomy-assisted techniques. 3

Postnatal Surgical Management

Immediate Closure

• Postnatal closure should occur within 48-72 hours of birth to minimize infection risk and prevent further neurological deterioration. 1
• Surgical closure involves layered repair of the neural placode, dura, fascia, and skin to create a watertight seal. 2

Hydrocephalus Management

• Approximately 86% of patients require cerebrospinal fluid diversion, with 95% undergoing at least one shunt revision during their lifetime. 4
• Ventriculoperitoneal shunt placement is the standard treatment for progressive hydrocephalus, which develops in the majority of myelomeningocele patients. 1

Urological Management Algorithm

Proactive vs. Expectant Approach

• Early proactive management with regular urodynamic testing and clean intermittent catheterization (CIC) before upper tract changes develop is recommended over expectant management. 1, 5
• Baseline renal and bladder ultrasound should be performed in the neonatal period to assess for congenital anomalies. 6
• Initiation of CIC after age 1 year is an independent risk factor for renal cortical loss. 1

Medical Management Protocol

• CIC should be initiated if post-void residual volumes consistently exceed 30 ml. 6
• Antimuscarinic medications (e.g., oxybutynin) are indicated for detrusor overactivity to maintain low bladder pressures and protect upper tracts. 1, 6
• Regular urodynamic surveillance is essential, as chronic kidney disease from poor bladder dynamics remains a significant source of morbidity and mortality. 5

Surgical Reconstruction Indications

• Surgery is indicated when: (1) upper tract changes or renal deterioration occur despite maximal medical management, or (2) facilitation of continence and independence is needed in older children. 1
• Bladder augmentation (enterocystoplasty) creates a large, low-pressure reservoir but commits patients to lifetime catheterization and carries risks including infection (37%), stones (52%), perforation (6%), and malignancy. 1
• The Antegrade Continence Enema (MACE) procedure achieves complete or near-complete fecal continence in 77% of patients and significantly improves quality of life. 7

Orthopedic and Neurological Surveillance

Tethered Cord Syndrome

• 32% of patients require tethered cord release, with 97% experiencing improvement or stabilization of preoperative symptoms. 4
• Continued surveillance for tethered cord is mandatory throughout childhood and adolescence, as it can develop or recur over time. 5, 6

Scoliosis Management

• 49% develop scoliosis, with 43% eventually requiring spinal fusion. 4
• Serial spine radiographs should be obtained during growth spurts to monitor curve progression. 8

Chiari II Malformation

• Nearly all myelomeningocele patients have Chiari II malformation, which may require posterior cervical decompression if symptomatic. 4, 2

Critical Long-Term Considerations

Latex Allergy Precautions

• Approximately one-third of patients develop latex allergy, with 6% experiencing life-threatening reactions in one cohort. 4
• All spina bifida patients should be managed in latex-free environments from birth onward. 4

Mortality and Late Deterioration

• 24% mortality rate extends into young adulthood, with late deterioration being common. 4
• At least 75% of children with myelomeningocele survive to early adulthood, but establishing adult care networks remains a major challenge. 4
• 23% develop seizures requiring anticonvulsant therapy. 4

Transition to Adult Care

• Structured transition programs with joint meetings between pediatric and adult medical teams significantly improve satisfaction and continuity of care. 7
• Transition of urological care to adulthood remains a major avenue for improvement in disease management. 5

Bowel Management Algorithm

Stepwise Approach

• First-line: Dietary modifications, laxatives, suppositories, and/or manual evacuation (successful in ~50% of patients). 7
• Second-line: Retrograde enemas if medical treatment fails (75% success rate). 7
• Third-line: MACE procedure for refractory cases (77% achieve continence). 7
• Final option: Colostomy placement by pediatric or colorectal surgeon for definitive management, though carries 2% mortality risk. 7

Prevention

• Periconceptional folic acid supplementation (400-800 mcg daily) reduces spina bifida incidence by up to 70%, leading to mandatory food fortification programs in many countries. 2