Variant (most frequent) of the Caroli disease characterized by the dilation of the large bile ducts in association with congenital hepatic fibrosis and hepatosplenomegaly ending in portal hypertension. Autosomal recessive transmission. Possible association with polycystic renal disease (autosomal recessive transmission), polycystic kidney disease (autosomal dominant), Cacchi-Ricci disease and nephronophthisis (autosomal dominant).

Anesthetic implications: 

check hepatic and renal functions. Portal hypertension. Rare indication of liver transplantation.

Updated: November 2019