portal portal bridging fibrosis; portal tract expanded by fibrosis Image

proliferation of duct prolifles along the periphery of the limiting plate Image

bile plugs; little to no inflammation; hepatic parenchyma shows no giant cell change or hepatocyte alterations; hepatic cholestasis is absent Image

staghorn shaped duct prolifes; buboidal epithelium; absent reactive ductular proliferation, rather these are primary malformed ducts Image

Nice gross image Image

interlacing pattern Image

Arrest of embyrologic remodeling of the ductal plate, leading to abnormally increased biliary channels forming a ring along the portal tract periphery. A centrally located bile duct is generally absent.

May be associated with polycystic kidney disease autosomoal recessive.

Clinical presentation varies but may include cytopenias, bleeding varices or splenomegaly. The liver is enlarged. The process may be patchy, making needle biopsy sometimes unreliable for diagnosis. Complications include portal hypertension, cholangitis, and cholangiocarcinoma.