Budd-Chiari Syndrome

Budd-Chiari syndrome is defined as noncardiogenic hepatic venous outflow obstruction of various causes, leading to progressive liver damage and portal hypertension.
Budd-Chiari syndrome recognizes different etiopathological conditions, differently distributed among population. A classification of Budd-Chiari syndrome on the basis of  morphological features, site of vein occlusion, and cause has been proposed &. Clinical presentation of the disease is usually gradual and the symptoms are related to liver congestion with subsequent hepatocyte necrosis and fibrosis. Rarely does Budd-Chiari syndrome occur with acute or fulminant liver failure &. If Budd-Chiari syndrome is suspected an appropriate work-up should be performed in order to diagnose common associated hematological disorders. Liver biopsy, if performed, shows contrilobular congestion and pressure necrosis; heart-type cirrhosis may be present in advanced stages of the disease.
Medical treatment of Budd-Chiari syndrome with diuretics and anticoagulant is not effective &. Surgical treatment is effective if irreversible liver injury has not yet developed. Several surgical procedures aimed at reducing the high venous pressure in the liver, inferior vena cava and portal vein and to restore normal liver hemodynamics, have been proposed.
Good results in the treatment of Budd-Chiari syndrome with liver transplantation have been reported. No accepted standards in deciding which patient should be treated with liver transplantation versus surgical decompression are available, even if several studies suggest that patients with fulminant or chronic forms of Budd-Chiari syndrome should undergo liver transplantation, while acute or subacute forms should be treated with portosystemic shunts &.