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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Budd-Chiari syndrome


Other Names for this Disease
  • Hepatic vein obstruction
  • Hepatic veno-occlusive disease
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Your Question

My nephew has been diagnosed with Budd-Chiari syndrome. What is the treatment for this condition?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is Budd-Chiari syndrome?

Budd-Chiari syndrome is a rare disorder characterized by narrowing and obstruction of the veins of the liver.[1] This narrowing or obstruction slows or prevents blood from flowing out of the liver and back to the heart which can lead to liver damage.[2] While some people experience no symptoms, many experience fatigue, abdominal pain, nausea, and jaundice. Other associated findings include an accumulation of fluid in the abdomen (ascites), an enlarged spleen and/or liver, and severe bleeding in the esophagus.[1][3] The severity of the disorder varies from case to case, depending on the site and number of affected veins.[1] Drugs may be used to dissolve or decrease the size of the obstruction (if it is a clot). In some cases surgery is performed.[3] In most cases, the cause of Budd-Chiari syndrome is unknown.[1]
Last updated: 10/30/2010

How might Budd-Chiari syndrome be treated?

The treatment of Budd-Chiari syndrome varies, depending on the cause of the blockage. Medical treatments may include:[2]

  • Blood-thinning (anticoagulation) medications
  • Clot-busting drugs (thrombolytic treatment)
  • Treatment for the liver disease, including ascites

Surgical treatments may also be considered and include:[2]

  • 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%).[4]

You can view more detailed information regarding the medical and surgical options for treatment of Budd-Chiari syndrome by clicking here.

Last updated: 10/30/2010

References