Bile Duct Perforation due to Inspissated Bile Presenting as Refractory Ascites

Non hepatic origin of refractory ascites is not a rarity. Hemolytic anemias are known to cause inspissated bile and biliary obstruction. Distal biliary obstruction can lead to biliary perforation. The authors report a case of hereditary spherocytosis leading to inspissated bile causing bile duct perforation and biliary ascites. A high index of suspicion for biliary ascites should be kept in a child with refractory ascites in the setting of progressive ascites with decreasing bilirubin. Ascitic fluid bilirubin analysis will clinch the diagnosis. Surgical repair is the optimal management.