Caroli syndrome occurs in the presence of polycystic kidney disease; it includes a polycystic liver, biliary sacculations and congenital hepatic fibrosis. The biliary pathology may promote bile stasis, recurrent cholangitis, secondary sclerosing cholangitis and neoplasia. The sacculations are contiguous with the biliary tree.
The congenital hepatic fibrosis may lead to portal hypertension and its sequelae, including ascites and variceal bleeding. In some cases, liver synthetic function is preserved for many years and the CHF goes unnoticed.
In the histology above, the portal field is expanded by fibrosis and contains numerous dilated, interlobular bile ducts. Notice the demarcation between portal fibrosis and the surrounding liver parechyma. There is no inflammatory reaction.