title: Correction of congenital diaphragmatic hernia in utero: VI. Hard-earned lessons.
abstract: Extensive experimental work suggests that repair of congenital diaphragmatic hernia (CDH) in utero may salvage severely affected fetuses who otherwise have a high expected mortality despite optimal postnatal care including extracorporeal membrane oxygenation (ECMO). We have reported that repair of CDH in utero is physiologically sound and safe for the mother, but technically difficult especially when the liver is herniated into the fetal chest. In the 3 years since our last report (1989 to 1991), 61 additional patients were referred for consideration of in utero repair. Fetal repair was attempted in 14 with severe isolated left CDH diagnosed before 24 weeks gestation. Five fetuses died intraoperatively, from technical problems related to reduction of incarcerated liver and uterine contractions--problems which have subsequently been surmounted. Nine patients were successfully repaired. Four babies survived, two delivered prematurely and died, and three died in utero within 48 hours of repair. Intraoperative technical problems have been overcome; the factors limiting successful outcome are postoperative physiologic management of the maternal-fetal unit and effective tocolysis to control preterm labor.
authors.affiliation: Fetal Treatment Center, University of California, San Francisco 94143-0570.
authors.forename: M R
authors.initials: MR
authors.lastname: Harrison
authors.forename: N S
authors.initials: NS
authors.lastname: Adzick
authors.forename: A W
authors.initials: AW
authors.lastname: Flake
authors.forename: R W
authors.initials: RW
authors.lastname: Jennings
authors.forename: J M
authors.initials: JM
authors.lastname: Estes
authors.forename: T E
authors.initials: TE
authors.lastname: MacGillivray
authors.forename: J T
authors.initials: JT
authors.lastname: Chueh
authors.forename: J D
authors.initials: JD
authors.lastname: Goldberg
authors.forename: R A
authors.initials: RA
authors.lastname: Filly
authors.forename: R B
authors.initials: RB
authors.lastname: Goldstein
mesh_terms: D005313:Fetal Death / Q000453:epidemiology / Q000209:etiology; D005315:Fetal Diseases / Q000401:mortality / Q000601:surgery*; D006548:Hernia, Diaphragmatic / Q000150:complications / Q000401:mortality / Q000601:surgery*; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007431:Intraoperative Complications / Q000401:mortality; D008107:Liver Diseases / Q000150:complications / Q000401:mortality / Q000601:surgery; D008297:Male; D011183:Postoperative Complications / Q000401:mortality; D012495:San Francisco / Q000453:epidemiology; D016896:Treatment Outcome