A Rare Congenital Disorder of Intrahepatic Bile Ducts

Abstract

A34-year-old male presented with chief complaints of jaundice for last 4 weeks and abdominal distension and swelling of the legs for 3 weeks. There was no associated abdominal pain, pale stools, or itching. He is a known diabetic on insulin therapy, and occasionally consumes alcohol. There was no family history of any liver-related diseases. His initial workup showed hemoglobin of 8.8 g%, total counts of 9800 cells/cu mm, and platelets counts of 58000 cells/cu mm. Liver function tests showed total bilirubin of 13.5, direct bilirubin of 10.4,