Congenital diaphragmatic hernia: where are we and where do we go from here?
The infant born with congenital diphragmatic hernia (CDH) remains one of the most complex patients to manage. Pulmonary hypoplasia and immaturity of the CDH lung are well recognized as the definitive limitation leading to the high mortality rates. Based on the knowledge that CDH is more a physiological disease than a surgical disease, we have shifted our management strategy from immediate repair to delayed repair and stabilization. The associated pulmonary hypertension and right-to-left shunt are common and rarely the cause of death and as such may be largely ignored. Extracorporeal membrane oxygen has been shown to salvage some of the most severely affected neonates. Other advanced and experimental respiratory therapies merit investigation in properly conducted prospective randomized multi-center trials. Survivors of CDH have predictable pulmonary, gastrointestinal and nutritional problems which, when identified and treated early, are correctable. CDH survival is close to 90% at most advanced centers. Uniform standards for CDH management do not exist however. Therefore a minimal set of practice standards should be developed from evidenced-based scientific review.
Muratore
C S
CS
Department of Surgery, Childrens Hospital and Harvard Medical School, Boston, MA 02115, USA.
Wilson
J M
JM
D015199:Extracorporeal Membrane Oxygenation; D005315:Fetal Diseases / Q000601:surgery; D005767:Gastrointestinal Diseases / Q000209:etiology; D006548:Hernia, Diaphragmatic / Q000196:embryology / Q000266:history / Q000628:therapy; D065630:Hernias, Diaphragmatic, Congenital; D049669:History, 16th Century; D049670:History, 17th Century; D049671:History, 18th Century; D049672:History, 19th Century; D049673:History, 20th Century; D006801:Humans; D021061:Liquid Ventilation; D008168:Lung / Q000473:pathology; D008171:Lung Diseases / Q000209:etiology; D009569:Nitric Oxide / Q000627:therapeutic use; D011663:Pulmonary Surfactants / Q000627:therapeutic use; D012121:Respiration, Artificial / Q000379:methods
D011663:Pulmonary Surfactants; D009569:Nitric Oxide