It now is feasible to close exposed spinal cord tissuein uterowith the goal of preventing secondary neurological compromise in animals with a surgical spinal defect. The present study was conducted to determine whether clinical intrauterine repair of myelomeningocele enhances the neurological outcome in infants with spina bifida. A nonrandomized observational study was undertaken of 29 infants with isolated fetal myelomeningocele who were seen at a tertiary-care center and underwent repair between 24 and 30 weeksrsquo; gestation. The 23 control infants, having repair within 48 hours after delivery, were matched with study cases for level of the lesion, practice parameters, and calendar time. The infants were followed up for at least 6 months. Surgery was performed after anesthetic was administered to the mother and under extended-spectrum antibiotic coverage. Intrauterine access was gained using electrocautery or a special hollow trocar. An 8-cm hysterotomy was made with an autostapling device, and the defect was repaired in the same way as in postnatal life. After the neural placode was dissected from arachnoidal tissue, the dura was freed, reflected over the placode, and closed. At present, no drain is left in place.Half of the study mothers and only 9 percent of control women were admitted with preterm uterine contractions, despite the lack of significant group differences in preexisting obstetrical risk factors. Nearly 40 percent of study women gave birth while still receiving tocolysis, compared with 4 percent of control women. The estimated gestational age at delivery was 33 weeks for the study group and 37 weeks for the control group, a difference significant at the .001 level. Five study infants were born before 30 weeksrsquo; gestation. Birth weights were significantly lower in the study group because of earlier gestational age at delivery. Ventriculoperitoneal shunt placement was necessary to decompress hydrocephalus in 59 percent of study infants, compared with 91 percent of infants who underwent repair after delivery. The long-term rates of shunt placement were 62 percent and 91 percent, respectively, still a significant difference. Only 2 of 29 study infants, compared with 10 of 22 control infants having postdelivery repair, had significant cerebellar herniation through the foramen magnum. Study infants less often had talipes (28 percent vs. 70 percent). One study infant required mechanical ventilation for longer than 72 hours. The average Bayley Scale developmental score in 26 study infants, assessed at ages 2 to 18 months, was 100. Although intrauterine repair of myelomeningocele makes premature delivery more likely, it does lessen the risk of both hindbrain herniation and shunt-dependent hydrocephalus.JAMA 1999;282colon;1819ndash;1825
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