Giant omphaloceles: surgical management and perinatal outcomes

Abstract Background The purpose of this study was to describe the current management and outcomes of infants with omphalocele. Methods The medical records of all patients treated for omphalocele at a large children's hospital from January, 2003–February, 2014 were reviewed. Patients were classified as having an isolated omphalocele or omphalocele with minor or major associated anomalies. Prenatal data collected included fetal magnetic resonance imaging-based observed-to-expected total fetal lung volumes. Giant omphalocele (GO) was defined as >50% of liver in the omphalocele sac. Results Of 95 patients, 59 presented prenatally and had comprehensive fetal center evaluation. Of 82 live-born infants, 21 had chromosomal and 25 had major associated anomalies. No live-born baby with an isolated defect ( n = 19) died, whereas mortality was 41% and 17% for those with major and minor anomalies, respectively ( P = 0.006). Infants with major anomalies had significantly longer median length of intubation (36 versus 0 versus 0 d; P = 0.04) and hospital stay (157 versus 28.5 versus 18 d; P