title: Fetal diaphragmatic hernia: ultrasound diagnosis and clinical outcome in 38 cases.
abstract: A previously published survey has evaluated the natural history and clinical outcome of fetal diaphragmatic hernia (CDH) in 94 cases. This study showed that the prenatal diagnosis is accurate, the mortality is high (80%), and polyhydramnios is a prenatal predictor of poor clinical outcome. As a follow-up study, 38 consecutive cases of CDH diagnosed in utero were evaluated and treated by the same surgical team. This permitted detailed assessment of prognostic factors and evaluation of the impact of extracorporeal membrane oxygenation (ECMO) on outcome. We found the following. (1) Survival is poor despite optimal postnatal therapy including ECMO. (2) Polyhydramnios is both a common prenatal marker for CDH (present in 69% of fetuses) and a predictor for poor clinical outcome (only 18% survival), but tends to occur after the second trimester. (3) Amniocentesis is indicated to rule out chromosomal abnormalities that were present in 16% of fetuses. (4) All 14 fetuses diagnosed prior to 25 weeks' gestation died. Improved postnatal therapy or surgical intervention before birth will be necessary to salvage the CDH fetus with an early gestational diagnosis or associated polyhydramnios.
authors.affiliation: Department of Surgery and Anesthesia, Children's Hospital, Boston, Massachusetts.
authors.forename: N S
authors.initials: NS
authors.lastname: Adzick
authors.forename: J P
authors.initials: JP
authors.lastname: Vacanti
authors.forename: C W
authors.initials: CW
authors.lastname: Lillehei
authors.forename: P P
authors.initials: PP
authors.lastname: O'Rourke
authors.forename: R K
authors.initials: RK
authors.lastname: Crone
authors.forename: J M
authors.initials: JM
authors.lastname: Wilson
mesh_terms: D015199:Extracorporeal Membrane Oxygenation; D005260:Female; D005315:Fetal Diseases / Q000175:diagnosis*; D006548:Hernia, Diaphragmatic / Q000175:diagnosis* / Q000628:therapy; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007231:Infant, Newborn; D011247:Pregnancy; D011296:Prenatal Diagnosis; D011379:Prognosis; D014463:Ultrasonography