Conforti Andrea F, Losty Paul D
Department of Paediatric Surgery, The Royal Liverpool Children's Hospital (Alder Hey) Division of Child Health, University of Liverpool, United Kingdom.
Early Hum Dev. 2006 May;82(5):283-7. doi: 10.1016/j.earlhumdev.2006.02.008. Epub 2006 Apr 17.
Congenital diaphragmatic hernia (CDH) retains high mortality due to lung hypoplasia and pulmonary hypertension. Efforts to improve survival and outcome have included fetal intervention, delivery at specialist centres, elective operation after stabilisation of labile physiology and minimising barotrauma. Permissive hypercapnea ('gentle ventilation') represents a significant advance in therapy gaining wider acceptance in centres worldwide. Human genetic studies are underway to identify candidate genes for the birth defect. Progress in the basic sciences may uncover critical aspects of developmental biology fundamental to CDH. Clinical trends in perinatal management of CDH are highlighted, which underpin the challenges of this lethal human anomaly.
