The syndrome is caused by occlusion of the hepatic veins by thrombosis or tumor. It may occur in individuals prone to thrombosis, including those with cancer or a myeloproliferative disorder. It may also occur with hepatocellular carcinoma or extensive liver metastases. Venous obstruction of the liver leads to hepatomegaly and portal hypertension, which in turn may lead to splenomegaly, ascites and esophageal varices.
It presents with the classical triad of abdominal pain, ascites and hepatomegaly. Diagnosis is based on clinical evaluation, liver function tests and ultrasonography.
Budd-Chiari syndrome is suspected in patients with hepatomegaly, ascites, liver failure, or cirrhosis when there is no obvious cause (eg, alcohol abuse, hepatitis) or when the cause is unexplained.
In the palliative care setting, it is one of the differential diagnosis of ascites and hepatomegaly.
Treatment includes supportive medical therapy and measures to establish and maintain venous patency, such as thrombolysis, decompression with shunts, and long-term anticoagulation.
The syndrome is named after an English physician, George Budd (1808-1882) who described the clinical syndrome in 1845; and the Austrian pathologist Hans Chiari (1851-1916) who later described the pathology.
See reference for more information.
Adapted from the Merck Manual, Professional Version. Budd-Chiari's Syndrome. Internet. Accessed on September 20, 2016.