P50.14: Massive omphalocele: management and outcome: a case report

The repair and survival rate of a fetus with a giant omphalocele, > 5 cm in diameter, is about 10%; representing a technically difficult surgery. Also with the presence of other anomalies such as intestinal, cardiac and renal, and with a poor outlook of successful results, requires much parental counseling. Respiratory failure at birth in infants with an omphalocele, is a significant predictor of mortality. A 32-year-old woman diagnosed with an omphalocele 3.7 cm in diameter in her 11th week decided to do a chorionic villus sampling after a preliminary exam by a fetal anatomy study. The karyotype was 46XX; the incidence of aneuploidy is lower in fetuses with giant omphaloceles in respect to fetuses with smaller ones. There was a serial echographic scan to exclude high complications and to evaluate the hepatic and bowel damage. The sonograph recognized the vessel's anatomy in the abdominal cavity by the progressive external compression of the herniated organs; the presence of the liver in the herniated sac was evident from the first exams. The fetus showed adequate growth; the omphalocele diameter during pregnancy was 3.7–4.8–5.3, nearly 7 cm before the surgery. The baby was born 2.560 grams at 37 weeks + 3 days after a prevention of distress syndrome by Cesarean section. The Apgar Index was 8 at 1–5 min., and no ventilatory support was required. The surgical technique used was ‘stretching’ the abdominal wall to put the liver back in the abdominal cavity with a delicate maneuver and then a primary closure. One week after surgery the baby was taken out of the Intensive Unit Care, and one month after delivery, was taken home. The parents consciously chose and relied on medical technology and equipment. The management of omphalocele is due to the presence of other anomalies, in this case the giant exomphalos being successfully managed using multiple support with the appropriate antenatal counseling, serial scan, but specifically by courageous surgery technique.