Clinical and experimental aspects of lung development and injury in congenital diaphragmatic hernia

Congenital anomalies are expressions of abnormal growth and development. They presently form the second most frequent cause of death, after immaturity, in the perinatal period. I Congenital diaphragmatic hemia (CDH) is a congenital anomaly manifesting itself in about one in 3,000 total births or one in 2,700 live buihs. More than 95% of the diaphragmatic defects are posterolateral ones. For a long tilne CDH was considered as a purely anatomical defect of the diaphragm4 But it has become clear that in many cases (rangulg from 39 to 47%) it is associated with other anomalies, especially cardiac defects. Despite improved neonatal intensive care, the overall survival rate does still not exceed 55 to 58, and is even less in patients with associated anomalies. However, higher survival rates have been repOlied: Wung and coworkers reported a survival rate of 94% in a group of CDH patients who were treated with very delayed surgery and a respu'atory care strategy that avoids pulmonary overdistension.