Congenital hepatic fibrosis is a part of Caroli syndrome (abnormal biliary sacculations). It is also part of the spectrum of autosomal recessive polycystic kidney disease. The fibrosis, when advanced, will lead to portal hypertension and its sequelae, including variceal bleeding and ascites.
Transforming growth factor-1 and thrombospondin-1 may play a role in the pathogenesis of liver fibrosis in patients with congenital hepatic fibrosis. Furthermore, connective tissue growth factor retained diffusely in heparan sulfate proteoglycan in the fibrous portal tracts or septa may be responsible for nonresolving hepatic fibrosis in congenital hepatic fibrosis.
In the histologic specimen above, notice the irregular bile ducts (the Caroli disease), the portal-based fibrosis (made more obvious by the paucity of periportal hepatocyte nuclei) and the relative lack of inflammation.