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A newborn with chylous ascites caused by intestinal malrotation associated with heterotaxia syndrome.

Chylous ascites is a rare entity in infants. An uncommon cause of chylous ascites is intestinal malrotation. We report a case of a neonate with heterotaxia-polysplenia syndrome and intestinal malrotation who presented with chylous ascites and subsequently developed duodenal obstruction from midgut volvulus. He was successfully treated with peritoneal drainage, surgical intervention, and nutritional support. A brief overview of the association of chylous ascites, intestinal rotational anomalies and heterotaxia syndromes is discussed.

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