Budd-Chiari syndrome with multiple large regenerative nodules

Budd-Chiari syndrome is defined as hepatic venous outflow tract obstruction regardless of the level of the obstruction (anywhere between the small hepatic veins to the junction of the inferior vena cava and right atrium) or the cause of obstruction. It is frequently associated with hepatomegaly, abdominal pain, ascites and hepatic dysfunction, and patients with Budd-Chiari syndrome often demonstrate one or more risk factors, including an underlying hypercoagulable state, vascular injury and stasis, venous obstruction by neoplasm or cirrhosis, amebic abscess, sarcoidosis etc. Hypercoagulable states include oral contraceptive use, antiphospholipid antibody syndrome, myeloproliferative diseases, paroxysmal nocturnal hemoglobinuria, factor V Leiden mutation, prothrombin gene G20210A mutation, and deficiencies of protein C, protein S, or antithrombin III. Although marked congestion and fibrosis are the common features of this condition, the histopathological features of Budd-Chiari syndrome are actually very heterogeneous depending on the type of vascular occlusion present, its severity and the duration. Obstruction of all three hepatic veins may initially present with fulminant hepatic failure, while involvement of one or two hepatic veins may be associated with only hepatomegaly and mild or transient liver test abnormalities. Portal vein obstruction is commonly seen in severe cases of Budd-Chiari syndrome. A case of BuddChiari syndrome with multiple large regenerative nodules is described, and the histopathological features discussed.