Intrauterine myelomeningocele repair reverses preexisting hindbrain herniation

Abstract

Background: It has been reported that intrauterine myelomeningocele repair reduces the amount of hindbrain herniation normally seen in association with the Chiari type II malformation. It is not yet known, however, whether hindbrain herniation is prevented, or whether preexisting herniation is reversed. The following study was designed to elucidate this issue.

Methods: A series of 9 patients underwent intraoperative ultrasound examinations immediately prior to intrauterine myelomeningocele repair. These same patients were then evaluated postnatally using ultrasound and/or MRI. The degree of hindbrain herniation before and after repair was compared using a grading system devised by the authors.

Results: Eight patients had clear evidence of moderate to severe hindbrain herniation on intraoperative scans while one was mild. In contrast, on postnatal studies 5 of 9 patients had no evidence of hindbrain herniation, while the other 4 had only mild herniation.

Conclusion: Intra-uterine myelomeningocele repair appears to reverse preexisting hindbrain herniation. It is postulated that continuous flow of cerebrospinal fluid through the neural placode is the force responsible for inducing migration of the cerebellum and brain stem downward through the foramen magnum. By interrupting that flow during gestation, intrauterine myelomeningocele repair enables the cerebellum and brain stem to resume a normal, or nearly normal, configuration.