title: [Therapeutic strategy and clinical outcome in congenital diaphragmatic hernia].
abstract: Congenital diaphragmatic hernia (CDH) continues to carry a high mortality rate 40%-60% mainly due to severe pulmonary hypoplasia. At our institute, thirty-seven neonates were treated for CDH diagnosed within the first 24 hours of life. Eleven of thirty-seven patients died and the mortality rate was 30%. Deaths in ten patients were due to severe pulmonary hypoplasia. The other one patient died from barotrauma at three months of age. All twenty-six patients who responded to ventilatory management and pharmacological therapies underwent surgical repair of CDH and all except one survived. Eight of eleven patients who did not respond to treatment also underwent surgery, but all died. These cases were all treated fufore ECMO introduction. Two of the other three in whom ECMO support was instituted died postoperatively. Among the parameters PH < 7, PaO2 < 60, PaCO2 > 60, AaDO2 > or = 600 and OI > or = 40 recorded on admission and examined retrospectively, OI > or = 40 was the most reliable as a predictor for poor outcome (sensitivity: 100%, specificity: 81%) and probably entry criteria for ECMO. The mean lung/body weight ratio of nonsurviving neonatal cases was as low as 0.49 +/- 0.18% while at least 1% is the critical value for survival. To salvage the CDH patient with severe pulmonary hypoplasia, surgical intervention before birth is inevitably necessary based on accurate prenatal diagnosis and established surgical techniques.
authors.affiliation: Department of Pediatric Surgery, Akita University School of Medicine, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Kato
authors.forename: H
authors.initials: H
authors.lastname: Yoshino
authors.forename: T
authors.initials: T
authors.lastname: Hebiguchi
authors.forename: M
authors.initials: M
authors.lastname: Mizuno
mesh_terms: D000818:Animals; D015199:Extracorporeal Membrane Oxygenation; D006548:Hernia, Diaphragmatic / Q000401:mortality / Q000601:surgery*; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007231:Infant, Newborn; D008168:Lung / Q000002:abnormalities; D016896:Treatment Outcome