Bappal et al. (1998) reported a three-week old non-dysmorphic female infant (weight of 3.8 kg) who presented with rapidly increasing abdominal distension of a week's duration (abdominal girth was 45 cm). Examination revealed dyspnea but her cardiovascular and respiratory systems were normal. Her baseline investigations were normal, and her total protein was 50 g/l, serum albumin was 35 g/l (urine albumin was 0.1 g/l), globulin was 24 g/l, total bilirubin was 9mmol/l, and alkaline phosphatase was 15 IU/l. Ultrasound of the abdomen revealed large amount of fluid in the peritoneal cavity with normal inferior vena cava and hepatic veins and no organomegaly. No abnormality was detected with CT-abdomen with barium contrast. Analysis of ascitic fluid revealed total protein of 20 g/l, glucose of 4 mmol/l, triglycerides more than 100 mmol/l, cholesterol of 18 mmol/l, and lactic dehydrogenase of 160 IU/l. Microscopy revealed a large number of lymphocytes. Initial conservative management technique with total parenteral nutrition (TPN) for 12 weeks succeeded in relieving the ascites as documented by an abdominal ultrasound scan after four weeks, but it recurred as the baby was fed with high medium chain triglyceride milk formula. At the end of the 12th week of TPN, laparotomy was performed which revealed leaking of fluid around the esophageal hiatus, which was managed by ligation of the leaky lympahtics. Post-surgery, she was exclusively fed with the medium chain triglyceride milk formula for four months followed by gradual introduction of a normal diet. The patient was found to be doing well by the age of two years.

Machmouchi et al. (2000) described the occurrence of four cases with congenital chylous ascites.