Improved survival of infants with omphalocele

Background The surgical management of patients with omphalocele has evolved over the past 4 decades. Despite many improvements in care, the reported mortality has been over 10%. Methods This study reviewed the characteristics, management, and outcome of 31 patients with omphalocele who underwent surgical care between 1980 and 1995 at a single hospital. Results Twenty-five patients had additional congenital anomalies. All but 1 patient underwent operative repair; 13 of these patients with large defects had a silo chimney constructed initially. Fourteen patients underwent primary fascial and skin closure at the initial operation. Only 1 patient died perioperatively; another patient died without operation due to other major malformations. Conclusions The results following surgical repair of omphalocele defects depend on the degree of visceroabdominal disproportion and on the severity of associated anomalies. The operative mortality for staged omphalocele repair with limited elevation of intraabdominal pressure is low, and the long-term quality of life of these patients is good.