Congenital diaphragmatic hernia: the surgeon's perspective.

Abstract

1. Erik D. Skarsgard, MD* 2. Michael R. Harrison, MD† 1. 2. *Assistant Professor of Surgery and Pediatrics, Division of Pediatric Surgery, Lucile Packard Children’s Hospital, Stanford University School of Medicine, Palo Alto, CA. 3. 4. †Professor of Surgery and Pediatrics, Division of Pediatric Surgery, UC San Francisco, San Francisco, CA. After completing this article, readers should be able to: 1. Describe other conditions that should be sought following prenatal diagnosis of congenital diaphragmatic hernia (CDH). 2. Describe the procedures that optimize the outcome for CDH upon delivery of the infant. 3. Delineate the two most significant prenatal ultrasonographic predictors of postnatal mortality from CDH. 4. Describe the standard of care for those who have CDH that can be managed without extracorporeal membrane oxygenation. 5. Delineate the overall survival rate associated with CDH diagnosed antenatally. Congenital diaphragmatic hernia (CDH) is a simple anatomic defect in which a hole in the diaphragm allows abdominal viscera to herniate into the thorax. The physiologic consequences of this defect may be mild and minimally symptomatic at birth, but often they are severe and may result in neonatal fatality, usually from irreversible pulmonary hypoplasia or severe persistent pulmonary hypertension. Prenatal diagnosis has facilitated the early recognition of this condition, and much has been learned about the “natural history” of fetal diaphragmatic herna that has allowed recent identification of accurate fetal prognostic indicators of neonatal outcome. The incidence of CDH is estimated to be 1 per 2,000 to 5,000 births. Its incidence in stillborns is less well studied, but it is important in understanding the contribution of associated congenital defects to the so-called “hidden mortality” that is associated with prenatal diagnosis. Population-based studies of CDH among liveborn, stillborn, and spontaneously aborted fetuses suggests that approximately 30% of fetuses who have CDH will die before birth, usually from chromosomal or lethal nonpulmonary malformations. Even among those fetuses who have CDH and survive to birth, the incidence of associated life-threatening malformations is higher in those in whom the diagnosis is made antenatally, especially if it is made before 25 weeks’ gestation. The reason for this observation is unclear, but an earlier gestational event that …