Spinal Dysraphia, Chiari 2 Malformation, Unified Theory, and Advances in Fetoscopic Repair.

Abstract

Fetal spina bifida, the most common nonlethal birth defect of the central nervous system, results in substantial neurologic morbidity. The unified theory describes the complex relationship between local spinal lesions and development of Chiari 2 malformation, contributing to hydrocephalus. Prenatal ultrasonography reliably allows diagnosis, but fetal MR imaging is an important complement to identify additional brain abnormalities. Fetal surgery improves neurologic and motor outcomes, but various approaches, either open hysterotomy or minimally invasive to the uterus, carry substantial obstetric risks. Optimization of the fetoscopic approach aims to minimize maternal and obstetric risks, but data regarding longer-term outcomes are awaited.