Caroli’s disease

Caroli’s disease

• Caroli’s disease is a rare congenital dilatation of the intra- - hepatic biliary tree with an incidence of <1/100 /uni00A0 000. It is often complicated by intrahepatic stone formation and presentation is usually with abdominal pain or sepsis. Imaging is diagnostic with ultrasonography or CT demonstrating intrahepatic biliary lakes containing stones which predispose to potentially life-threatening biliary sepsis. Acute infective episodes are treated with antibiotics and obstructed and infected bile ducts may be drained radiologically , endoscopically or surgically . - Ductal dilatation is usually di ff use but if segmental may be treated by resection of the a ff ected part. Cholangiocarcinoma is more common than for other forms of biliary ectasia and treatment by liver transplantation is described.