Improved survival for congenital diaphragmatic hernia, based on prenatal ultrasound diagnosis and referral to a combined obstetric-pediatric surgical center

Abstract

Between January 1990 and January 1993, 36 patients with antenatal ultrasound (US) diagnosis and/or postnatal diagnosis of congenital diaphragmatic hernia (CDH) were referred to the authors' high-risk obstetric and pediatric hospital. Among the 36, there were four spontaneous abortions (11%), five deaths after live births (14%), one false-positive US examination, and 26 patients who underwent surgery, 23 of whom survived (66% overall, 74% of live births, and 89% postoperatively). Only one survivor had extracorporeal membrane oxygenation (ECMO). Thirty-five ultrasound examinations were performed in 24 patients; there were 18 true-positives results (51%), one false-positive (3%), and 16 false-negatives (46%). In this series, there were 25 left-sided CDHs, eight right-sided, one bilateral, and one central. Of the four right-sided hernias having antenatal US, only one was diagnosed prenatally; 15 of the 17 left-sided CDHs were diagnosed correctly (88%). All 19 babies with the prenatal diagnosis were born at the authors' institution. US diagnosis before 25 weeks' gestation and polyhydramnios separately resulted in a mortality rate of only 50%. Patients born at this institution tend to be sicker than those transferred from elsewhere, as reflected by the lower 1- and 5-minute Apgar scores (3.7 v 6.9, P < .001, and 5.4 v 6.9, P < .16, respectively), lower gestational ages (37.0 v 39.2 weeks, P < .007), and lower birth weights (2,525 v 3,049 g, P < .02). Nevertheless, transferred patients had a mortality rate (3 of 15 patients, 20%) similar to that of nontransferred patients (5 of 20 patients, 25%). The authors conclude that (1) systematic prenatal US can diagnose CDH and enable referral to a center that provides high-risk obstetric and pediatric surgical care, thus providing optimal postnatal treatment, and (2) contrary to other reports, antenatal diagnosis of CDH did not presage a high mortality rate.