Congenital Diaphragmatic Hernia: Maximizing Survival

Congenital diaphragmatic hernia occurs when a portion of the fetal diaphragm is absent, allowing abdominal contents to enter the thorax, and is associated with impaired pulmonary development. Although overall mortality is near 30%, a mortality rate less than 15% may be possible by following a standardized multidisciplinary care plan. Fetal diagnosis and evaluation can improve coordination of care, but there is no clear role for fetal intervention. After birth, gentle ventilation with permissive hypercapnia supports the infant while minimizing lung injury. Appropriate cardiovascular support, treatment of pulmonary hypertension, and extracorporeal membrane oxygenation may benefit some patients. Timing of surgical repair depends on disease severity. All patients should have close follow-up after discharge.