A “C” triad: cirrhosis and cholangitis due to Caroli disease.

Abstract

Caroli disease (CD) is a rare congenital pathology of probable autosomal recessive inheritance, it is part of the fibrocystic liver diseases and is included in group V of the Todani classification. It is characterized by saccular or cystic dilations of the intrahepatic bile ducts, sometimes associated with congenital liver fibrosis, and is then called Caroli syndrome (CS). The usual clinical manifestation is recurrent abdominal pain secondary to intrahepatic or vesicular stones, and acute cholangitis crises with the consequent formation of intrahepatic abscesses.

Keywords: Caroli disease, liver cirrhosis, acute cholangitis.