Ease Into Prep with a USMLE^® Step 2 CK Question of the Day

The correct answer is A. Budd-Chiari syndrome is a disorder in which hepatic venous outflow is obstructed because of thrombosis of the major hepatic veins. The blood clots may extend into the inferior vena cava, causing the abdominal wall signs and edema of the legs illustrated in the case. This condition is rare and typically occurs in the setting of a coagulopathy due to hematologic disease (myeloproliferative disorders, polycythemia vera, sickle cell disease, paroxysmal nocturnal hemoglobinuria) or in disorders of the coagulation (defects in normal inhibitors, such as antithrombin III, protein C, protein S, factor V Leiden; antiphospholipid antibodies; and possibly high estrogen states, such as oral contraceptive use or pregnancy). The disorder either presents with acute hepatic failure or, more commonly, progresses over several months. Early recognition of the syndrome is important so that thrombolytics and long-term anticoagulation can be given. Some patients respond to medical management, whereas others with fulminant or end-stage disease may require liver transplantation.

Hepatic cirrhosis (choice B) develops slowly and produces a nodular liver. Hepatic vein thrombosis is not associated.

Hepatocellular carcinoma (choice C) would produce a liver mass. Ascites is a late finding and usually develops slowly. Portal vein, rather than hepatic vein, thrombosis may occur.

Primary sclerosing cholangitis (choice D) is an inflammation of the bile ducts that does not usually produce ascites unless it has progressed to cirrhosis.

Hepatic steatosis (choice E), or fatty liver, does not produce ascites.