Giant Omphalocele

Omphalocele is the second most common congenital abdominal wall defect, affecting approximately 1 to 2 per 10 000 live births, and it requires intensive neonatal care at birth. Omphalocele is characterized by herniation of abdominal viscera into a membranous sac through a defect at the umbilical ring. Giant omphalocele (GO) is a subset of this condition and is generally defined as a defect measuring at least 5 cm in diameter or one that contains evisceration of at least 50% of the liver into the sac. The pathophysiology of GO is thought to involve a complex interplay of failure of the lateral abdominal folds to close during embryogenesis and of bowel loops to return to the abdominal cavity. This results in persistent herniation of the abdominal contents into the base of the umbilical cord between the 6th and 11th weeks of development. GO is typically diagnosed prenatally by fetal ultrasonography and requires multidisciplinary care between high-risk obstetrics, neonatology, and pediatric surgery. In this review, we discuss the prenatal, neonatal, and surgical management, as well as long-term outcomes of patients with GO.