A novel approach for the closure of challenging giant omphalocele

Omphalocele is a congenital condition characterized by midline defect of the anterolateral abdominal wall whereby intra-abdominal contents covered by peritoneal sac are found herniated. It can be defined by size of defect or visceral contents. There are various ways of surgical management of omphalocele. Giant omphalocele presents particular challenge in reconstructing the abdominal wall. In this regard the use of tissues expansion as an adjunct in an attempt to recreate functional abdominal wall domain has been emerging. We are presenting a case of giant omphalocele that was closed with the help of extra peritoneal inter muscular tissue expanders.The patient was born at 37 weeks to a 33 year old healthy gravida 3, para 0 G3P0 mother via planned cesarean section. There were no prenatal, natal or postnatal complications. The giant omphalocele was diagnosed prenatally on screening Ultrasound. The patient remained in the neonatal intensive care unit for 6 weeks. The sac was large with abdominal contents including the entire liver. The sac was allowed to epithelialize completely over the course of two years. Due to the small size of the abdominal wall defect contents of the sac, loss of abdominal domain and inability of the abdominal cavity to accommodate the liver, we chose for rectangular tissue expander placement at age 30 months. 3 months later the patient was planned for tissue expander removal and abdominal wall closure. The tissue expanders were removed and omphalocele closure was performed. The patient has been followed up at 6 months and without recurrence of hernia.The management of giant omphalocele is technically challenging. Use of tissue expansion helps in recreating functional abdominal wall domain. There is growing evidence in the literature supporting the use of intra-abdominal tissue expansion in the management of giant omphalocele however because of hemodynamic consequences we think that the use of rectangular shaped, extra peritoneal, inter muscular tissue expanders is more desirable and has additional advantage of facilitating component separation.