Abstract

 
 
 Congenital chylous ascites (CCA) is a rare disease that results from the maldevelopment of the intra-abdominal lymphatic system. Due to the rarity of congenital chylous ascites and the lack of standards in diagnosis and therapy, this disease constitutes a medical challenge and individual therapy seems to be extremely important. A 3-month-old girl diagnosed with congenital chylous ascites. She was managed initially with nil per oral, parenteral nutrition, medium chain triglyceride (MCT) containing oil and abdominal paracentesis, followed by octreotide. Medium chain triglyceride formula, the main stay of management was discontinued with gradual reintroduction of breast feeds. This case was selected due to the rarity of CCA and the lack of standards in the diagnosis and therapy.
 
 
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