Case history

Case history #1

A 40-year-old woman presents with acute onset of upper abdominal pain and ascites. She has been on the combined oral contraceptive pill for 7 years. On exam she has tender hepatomegaly. There is no hepatojugular reflux and no lower limb edema. Cardiovascular exam revealed no medical abnormalities. She is known to have polycythemia vera of 10 years' duration.

Case history #2

A 50-year-old man with a history of essential thrombocytosis of 12 years' duration presents with mild jaundice and vague right upper quadrant pain. Physical exam reveals minimal ascites, hepatomegaly, and splenomegaly.

Other presentations

The classic triad of symptoms is abdominal pain (61%), ascites (83%), and hepatomegaly (67%).[1][6][7]​ Acute Budd-Chiari syndrome (BCS) is observed in 20% of patients.[8] These patients develop severe right upper quadrant abdominal pain, hepatomegaly, jaundice, and intractable ascites within a few weeks. Between 5% and 20% of cases are detected following investigation of slightly abnormal results in liver function tests, but have no symptoms.[8][9]​ The fulminant form of BCS is uncommon. Patients rapidly develop hepatic encephalopathy, renal failure, and coagulopathy. The liver is enlarged and tender.[10] Patients with inferior vena cava compression or thrombosis present with leg edema or venous collaterals over the trunk in addition to hepatomegaly, ascites, and abdominal pain. The symptoms are less severe than in hepatic vein thrombosis.[11][12]

Use of this content is subject to our disclaimer