Fetal Surgery for Congenital Diaphragmatic Hernia: The North American Experience

Abstract

The role for fetal surgery in treating fetuses with congenital diaphragmatic hernia (CDH) is unclear. Two decades of investigation have improved our understanding of the prenatal natural history, pathophysiology, and outcomes of these patients. During this same period, there have been advances in fetal surgery techniques including improvements in fetal monitoring, maternal-fetal anesthesia, tocolysis, and improved instrumentation to permit increased application of videoscopic approaches. Because of technical challenges, open fetal repair of CDH has been abandoned. Fetal tracheal ligation has shown promise, but a recently published prospective, randomized trial failed to show a benefit of fetoscopic tracheal ligation compared with expert postnatal treatment. Although there is evidence that postnatal outcomes for infants with this disease have improved with the adoption of gentilation ventilator management, high-frequency ventilation, and ECMO, there continues to be a subset of infants with severe CDH that die or suffer serious long-term morbidity despite advanced surgical care. The purpose of this article is to review issues related to prenatal diagnosis, patient selection, and outcomes for fetal surgery; and ultimately to assess whether there is a role for fetal surgery in treating fetuses with CDH.