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.