Congenital Diaphragmatic Hernia

Abstract

Congenital diaphragmatic hernia (CDH) is a congenital anomaly defined by incomplete diaphragmatic formation with associated pulmonary parenchymal hypoplasia and pulmonary vascular hypertension and an overall survival of 70%. The diaphragmatic defect that is most commonly identified is posterolateral (Bochdalek) and much more commonly occurs on the left side. The less common antero-medial (Morgagni) defect is pathophysiologically distinct and easier to manage. CDH is a disease in which morbidity and mortality result from the hypoplastic lung and abnormal pulmonary vasculature. Infants who survive face ongoing pulmonary, nutritional, gastrointestinal, and developmental challenges. Contemporary treatment begins in the prenatal period and may include fetal intervention in a highly select group. Postnatal management focuses on initial physiologic stabilization using pressure-limited ventilation strategies, pulmonary anti-hypertensive pharmacologic agents, and extracorporeal membrane oxygenation. The timing and technique for surgical repair are variable and the importance of long-term multidisciplinary care has recently been appreciated.