Congenital diaphragmatic hernia.
Congenital diaphragmatic hernia (CDH) is a congenital anomaly consisting of a posterolateral defect in the diaphragm also known as a Bochdalek hernia. It occurs in 1 in 2000 to 3000 newborns and is associated with a variable degree of pulmonary hypoplasia (PH) and persistent pulmonary hypertension (PPH). Despite remarkable advances in neonatal resuscitation and intensive care and the new postnatal treatment strategies, many newborns with CDH continue to have high rates of mortality and morbidity as the result of severe respiratory failure secondary to PH and PPH. The pathogenesis of CDH and associated PH and PPH is poorly understood. Herein, we aim to review diaphragm and pulmonary development and correlate this to the abnormalities found in CDH.
Keijzer
Richard
R
Department of Pediatric Surgery, Erasmusmc-Sophia, Rotterdam, The Netherlands.
Puri
Prem
P
D005260:Female; D018662:Fundoplication / Q000379:methods; D006548:Hernia, Diaphragmatic / Q000150:complications* / Q000601:surgery; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D006976:Hypertension, Pulmonary / Q000453:epidemiology / Q000209:etiology / Q000503:physiopathology; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities* / Q000196:embryology; D008171:Lung Diseases / Q000209:etiology / Q000473:pathology* / Q000503:physiopathology; D008297:Male; D011379:Prognosis; D018570:Risk Assessment; D012720:Severity of Illness Index; D016896:Treatment Outcome