Caroli's disease. A rarely recognized entity

Caroli's disease, well known in the European literature, has been recognized infrequently in the USA. The disease is characterized by cystic dilation of intrahepatic bile ducts and is complicated by formation of gallstones, cholangitis, and hepatic abscesses. Surgical intervention and therapy with antibiotics is often unsuccessful and Gram-negative septicemia is a common cause of death. Cysts may be present in other organs, such as kidney, pancreas, and spleen, and may be helpful in the early diagnosis of this rare disease.