Objective: The objective of this study was to present our experience regarding the diagnosis and therapeutic approach in cases of congenital chylous ascites that were surgically managed in our department in a 10-year period.Background: Congenital chylous ascites is a rare condition that constitutes a challenge for physicians. This condition is often refractory to therapy, and it is responsible for serious malnutrition and immunological deficiency because of losses of proteins and lymphocytes.Patients and methods: From 2007 to 2017, four cases of congenital chylous ascites were managed at our center. All cases were treated surgically after failure of medical treatment.Results: The first patient had no definite lymphatic leaks rather than presence of a moderate amount of milky ascitic fluid with dilated lymphatics at the root of the mesentery and at the intestinal wall and enlarged regional lymph nodes. The second one had leaking chylolymphatic cyst in the greater omentum that was excised. The third patient had no definite lymphatic leaks rather than thickened greater omentum, and thus, subtotal omentectomy was done. The fourth baby had no definite lymphatic leaks and developed reaccumulation of the ascitic fluid 1 month after surgery; insertion of a peritoneovenous shunt was indicated that succeeded to resolve the ascites. All children are growing up normally with no recurrence of the ascites on long-term follow-up.Keywords: chylolymphatic cyst, chylous ascites, lymphatic leak, peritonealvenous shun
