Abstract

Abstract Congenital chylous ascites (CCA) is a rare disease that results from mal development of the intra-abdominal lymphatic system and no gold standard treatment described so far. It is defined as the accumulation of triglyceride-rich milky fluid into peritoneal cavity in infant younger than three months. Medium chain triglyceride (MCT)-based diet or total parental nutrition (TPN) with rest to bowel and abdominal paracentesis is the time honoured conservative management. Due to rare incidence of this disorder, the existing literature includes mainly case reports where TPN was used with or without MCT based formulas along with octreotide. This condition is often refractory to therapy and is responsible for serious malnutrition and immunological deficiency because of loss of proteins and lymphocytes. Its treatment is often frustrating and challenge to physician. We report an infant with chylous ascites which was refractory to medical treatment in the form of prolonged bowel rest with TPN, Octreotide and repeated paracentesis. Surgical treatment with application of fibrin glue in the area of paradudenal retroperitoneal lymphatics sealed the lymphatic leak and cured the patient.

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