Omphalocele: clinical outcomes in cases with normal karyotypes

OBJECTIVE: This study was undertaken to examine neonatal outcomes in karyotypically normal cases of omphalocele with respect to mode of delivery, presence of associated anomalies, presence of extracorporeal liver, and method of abdominal wall closure. STUDY DESIGN: We reviewed 36 cases of omphalocele with delivery at University of North Carolina Hospitals between 1988 and 2001. Elective terminations and pregnancies resulting in miscarriage before 20 weeks' gestation were excluded. RESULTS: Perinatal mortality rate was 19%. Rate of composite neonatal morbidity was 25%. Associated major anomalies were present in 11 (31%) cases and were associated with increased neonatal mortality. Cesarean deliveries were performed in 21 (58%) cases and were not associated with increased primary closure rates or decreased neonatal morbidity and mortality. Extracorporeal liver was present in 27 (75%) cases and was associated with decreased rates of primary closure but did not affect neonatal outcome. CONCLUSION: In cases of ongoing omphalocele, perinatal mortality rates are low in the absence of associated anomalies or genetic defects. Intracorporeal liver was not associated with increased rates of associated anomalies or was it associated with increased neonatal morbidity or mortality.