hernia, diaphragmatic

Diaphragmatic hernia is a potentially lethal birth defect in which the diaphragm does not completely form. During fetal life, abdominal organs slide up into the chest cavity, and thereby constrict the normal development of the fetal lungs. This causes no problem whatsoever in utero, but upon delivery, the baby is in grave peril, because the lungs are underdeveloped (hypoplastic). The condition is often fatal. Surgical correction is imperative, within the first few days of life. In recent years, the artificial lung device (extracorporeal membrane oxygenation - ECMO) has been used to support these babies until surgery could expand the remaining lung tissue and the baby's lung could achieve adequate oxygen exchange on its own.

An historical review of the management of this disorder in the May 1998 issue of Archives of Surgery showed that survival has improved from 42% in the early era of immediate repair to 79% in the current era of preoperative extracorporeal membrane oxygenation (ECMO) and delayed operation if at all possible. I personally think that prenatal diagnosis on routine fetal ultrasound examinations has had a great deal to do with increased survival, as well.

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