[In utero treatment of congenital diaphragmatic hernia].

Abstract

Congenital diaphragmatic hernia is a rare malformation with a prevalence of approximately 1 in 3,000 births. Pulmonary development during fetal life is variably impaired, and newborns typically present pulmonary hypoplasia, often associated with pulmonary hypertension. In France, in 2008, the creation of the Rare Diseases Reference Center (CRMR) for Congenital Diaphragmatic Hernia, affiliated with the Rare Disease Network FIMATHO (Program for Rare Abdominothoracic Diseases), enabled the establishment of a territorial network ensuring relatively homogeneous expertise across the country. After more than 10 years of evaluation and two international randomized trials, in utero surgery by fetal tracheal occlusion has become one of the therapeutic options for fetuses with severe and moderate forms of congenital diaphragmatic hernia.