Refractory congenital chylous ascites—Case report of a successful surgical management

Abstract

Congenital chylous ascites (CCA) is a rare condition caused by maldevelopment of the intra-abdominal lymphatic system, resulting in chyle accumulation in the peritoneal cavity. Diagnosis involves analyzing milky-white ascitic fluid with elevated triglycerides and T-lymphocyte predominance, with lymphangiography or lymphoscintigraphy as the gold standard. Initial treatment focuses on nutrition and medical therapies like medium-chain triglycerides (MCT) and total parenteral nutrition (TPN). Surgery is considered if conservative measures fail. A neonate with CCA required TPN, MCT formula, octreotide, and paracentesis. Lymphoscintigraphy showed bilateral lymphatic leakage. Despite medical management, the condition remained refractory, and exploratory surgery was performed, revealing a mesothelial cyst and peritoneal defect, which was treated with fibrin glue. Post-surgery, the infant's ascites resolved, highlighting the need for surgery in refractory cases.