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Budd-Chiari syndrome is clotting of the hepatic vein, the major vein that drains the liver of blood. Most patients with Budd-Chiari syndrome have an underlying condition that predisposes to blood clotting. About 10% have polycythemia vera, a condition in which abnormal amounts of red blood cells are. About 10% of
patients with Budd-Chiari syndrome take birth control pills, which also may predispose to blood clotting.

The most common symptoms in Budd-Chiari syndrome is ascites, or fluid accumulation in the abdomen and abdominal pain. Patients can also have abnormal bloods tests indicative of liver disease. Some individuals with Budd-Chiari syndrome may be jaundiced (yellow skin). The examining doctors often first suspect cirrhosis as a cause of the symptoms in patients with Budd-Chiari syndrome.

Patients with Budd-Chiari syndrome who have deteriorating liver function and complications usually need
to undergo liver transplantation. Other surgical procedures have been used with variable degrees of success including the fashioning of a transjugular intrahepatic portosystemic shunt (TIPSS) or portacaval shunt.
In some cases, the underlying condition that caused the syndrome excludes transplantation as a treatment
option. In many situations, patients will need to remain on blood thinners the remainer of their life to prevent further clotting.