What is the definition of Budd-Chiari Syndrome?

Budd-Chiari syndrome is a rare disorder characterized by obstruction of the veins of the liver that carry the blood flow from the liver. When the blood flow out of the liver is impeded, blood backs up in the liver, causing it to enlarge (hepatomegaly). The spleen may also enlarge (splenomegaly). This backup of blood increases blood pressure in the portal vein, which carries blood to the liver from the intestines (portal hypertension), and result in  dilated, twisted veins in the esophagus (esophageal varices). Portal hypertension, leads to fluid accumulating in the abdomen (called ascites). The clot may extend to also block the inferior vena cava (the large vein that carries blood from the lower parts of the body to the heart). Varicose veins in the abdomen near the skin’s surface may develop and become visible. In some cases,  scarring of the liver (cirrhosis) occurs. Other symptoms may include fatigue, abdominal pain, nausea, jaundice and bleeding in the esophagus. The severity of the disorder varies from case to case, depending on the site and number of affected veins. It most often occurs in patients which have a disorder that makes blood more likely to clot, such as those who are pregnant or who have a tumor, a chronic inflammatory disease, a clotting disorder, an infection, or a myeloproliferative disorder. In about one third of the cases, the cause of Budd-Chiari syndrome is unknown. Drugs or surgical interventions may be used to dissolve or decrease the size of the obstruction (if it is a clot). In some cases liver transplantation is needed.  Budd-Chiari syndrome should be considered separate from veno-occlusive disease (VOD).

What are the alternative names for Budd-Chiari Syndrome?

  • Membranous obstruction of the inferior vena cava

What are the current treatments for Budd-Chiari Syndrome?

The treatment of Budd-Chiari syndrome varies, depending on the cause of the blockage. Medical treatments may include:
  • Blood-thinning (anticoagulation) medications
  • Clot-busting drugs (thrombolytic treatment)
  • Treatment for the liver disease, including ascites

Surgical treatments may also be considered and include:

  • Angioplasty and stent placement
  • Transjugular intrahepatic portosystemic shunt (TIPS)
  • Venous shunt surgery

While medical therapy can be instituted for short-term, symptomatic benefit, medical therapy alone has been associated with a high 2-year mortality rate (80-85%).

In cases of severe cirrhosis liver transplant may be needed. You can view more detailed information regarding the medical and surgical options for treatment of Budd-Chiari syndrome by visiting the Medscape Reference website on Budd-Chiari syndrome treatment & managment. You may need to register to view the article, but registration is free.