Diagnosis and management of congenital diaphragmatic hernia: a clinical practice guideline

The severity of congenital diaphragmatic hernia (CDH) can be estimated prenatally using observed-to-expected lung-head ratios (by ultrasound) and total fetal lung volumes (by magnetic resonance imaging), as well as fetal liver position. Infants with CDH require intensive cardiopulmonary support after birth, including immediate endotracheal intubation and "gentle ventilation," as well as judicious fluid and inotropic support. Pulmonary hypertension, as assessed by echocardiography, may require the use of pulmonary vasodilators and other medical adjuncts or, in severe cases, extracorporeal life support, if available. Open surgical repair of the diaphragmatic defect should usually be delayed until physiologic stability has been achieved, but a failure to perform surgery within the first two weeks of life should prompt a team discussion of priorities with the family. Infants with CDH should undergo long-term, multidisciplinary surveillance that includes standardized neurodevelopmental testing, especially if they are considered high risk (i.e., needing pulmonary support at 30 days, needing a patch repair or requiring extracorporeal life support).