Fetal surgery: the ochsner experience with in utero spina bifida repair

Myelomeningocele is the most common form of congenital central nervous system defect that is compatible with life. Most patients with myelomeningocele have significant functional impairment of ambulation and bowel and bladder function, require permanent cerebrospinal fluid diversion with shunting, and have significant morbidity and mortality from hindbrain herniation (Chiari II malformation). The advent of intrauterine surgery has provided new opportunities to better address this lifelong debilitating disease.

The patient was a 19-year-old gravida 2 para 1 at 22-6/7 weeks whose fetus was diagnosed with an open neural tube defect and further demonstrated to have ventriculomegaly and hindbrain herniation. Amniocentesis confirmed normal karyotype and the presence of acetylcholinesterase. After an intrauterine procedure, the patient underwent cesarean section at 35-5/7 weeks and delivered a male infant. His spinal incision was well healed at birth without any evidence of cerebrospinal fluid leakage, and his extremities were normal in appearance, range of motion, and movement. The infant also has maintained relatively normal, age-appropriate bowel and bladder function and has no obvious neurologic deficit.

As the benefit of fetal surgery becomes more widely accepted, quality of care and patient safety must be at the forefront of any institution's effort to offer fetal surgery. Given the current prevalence of spina bifida and the amount of resources required to treat this disease effectively either in utero or postnatally, it is our opinion that the treatment of spina bifida should be regionalized to tertiary referral centers with the interdisciplinary expertise to offer comprehensive treatment for all aspects of the disease and all phases of care for the patients.