Current and emerging treatment for congenital diaphragmatic hernia.

Management of congenital diaphragmatic hernia has changed dramatically over the past couple of decades. Until the early 1980s, it was felt that the abdominal contents should be returned to the abdomen as soon as possible to allow the lungs to expand. It is now known that it is not the defect that causes respiratory distress, but the infant's hypoplastic lungs and accompanying pulmonary hypertension. Advances in treatment and technology have contributed to changes in management. Ultrasonography now allows for early prenatal detection. Prenatal treatment modalities include in utero tracheal ligation and maternal antenatal steroids. Postnatal modalities have expanded to include permissive hypercapnia, high-frequency ventilation, inhaled nitric oxide, pharmacologic support, exogenous surfactant, and extracorporeal membrane oxygenation. Liquid ventilation and lobar lung transplantation have also been tried. In spite of these advances, the overall survival rate remains about 63 percent.