Fetal Surgery for Myelomeningocele and the Incidence of Shunt-Dependent Hydrocephalus

• Published in: JAMA : the journal of the American Medical Association 1999-11, Vol.282 (19), p.1819-1825
• Main Authors: Bruner, Joseph P, Tulipan, Noel, Paschall, Ray L, Boehm, Frank H, Walsh, William F, Silva, Sandra R, Hernanz-Schulman, Marta, Lowe, Lisa H, Reed, George W
• Format: Article
• Language: English
• Subjects: Biological and medical sciences; Birth defects; Cerebrospinal fluid. Spinal cord. Spinal roots. Spinal nerves; Female; Fetal Diseases - surgery; Fetuses; Gestational Age; Humans; Hydrocephalus - etiology; Hydrocephalus - therapy; Infant, Newborn; Infant, Premature; Intraoperative Complications; Medical sciences; Meningomyelocele - complications; Meningomyelocele - surgery; Nervous system; Neurosurgery; Pregnancy; Pregnancy Outcome; Prenatal development; Surgery; Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases; Survival Analysis; Treatment Outcome; Ventriculoperitoneal Shunt
• ISSN: 0098-7484; 1538-3598
• DOI: 10.1001/jama.282.19.1819

CONTEXT Intrauterine closure of exposed spinal cord tissue prevents secondary neurologic injury in animals with a surgically created spinal defect; however, whether in utero repair of myelomeningocele improves neurologic outcome in infants with spina bifida is not known. OBJECTIVE To determine whether intrauterine repair of myelomeningocele improves patient outcomes compared with standard care. DESIGN Single-institution, nonrandomized observational study conducted between January 1990 and February 1999. SETTING Tertiary care medical center. PARTICIPANTS A sample of 29 study patients with isolated fetal myelomeningocele referred for intrauterine repair that was performed between 24 and 30 gestational weeks and 23 controls matched to cases for diagnosis, level of lesion, practice parameters, and calendar time. All infants were followed up for a minimum of 6 months after delivery. MAIN OUTCOME MEASURES Requirement for ventriculoperitoneal shunt placement, obstetrical complications, gestational age at delivery, and birth weight for study vs control subjects. RESULTS The requirement for ventriculoperitoneal shunt placement for decompression of hydrocephalus was significantly decreased among study infants (59% vs 91%; P = .01). The median age at shunt placement was also older among study infants (50 vs 5 days; P = .006). This may be explained by the reduced incidence of hindbrain herniation among study infants (38% vs 95%; P