[Caroli syndrome. Isolated congenital dilatation of intrahepatic bile ducts]

Abstract

Caroli's disease is a rare, congenital autosomal-recessive condition in which the intrahepatic bile ducts show segmental cystic dilatation. In these saccular dilatations, calculi usually develop. often with subsequent cholangitis. The disease is discussed on the basis of a case, the diagnostic evaluation and treatment possibilities being discussed.