Intrahepatic biliary dilatation with aortoarteritis.

Intrahepatic biliary dilatation or Caroli's disease is a rare clinical entity of unknown etiology. It consists of dilatation of intrahepatic biliary ducts which communicate with normal common bile duct system. It may manifest at any age but usual age of presentation is late childhood or early adult life. The clinical features of disease includes recurrent abdominal pain associated with fever, hepatomegaly and mild jaundice secondary to recurrent cholingitis(l). It may be associated with liver disorders such as congenital hepatic fibrosis(2) and hepatic carcinoma(3). Occasionally it may be associated with renal tubular ectasia and renal cysts(4). To our knowledge it's association with aorto arteritis has not been reported.