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What Is Budd-Chiari Syndrome?

Blockage of the hepatic vein, which is the major vein that leaves the liver, leads to a condition in which blood enters but has difficulty leaving the liver. This rare condition is called the Budd-Chiari syndrome. It occurs equally in men and women, usually in people 30 to 50 years old.

What Causes Budd-Chiari Syndrome?

Any condition that can lead to excessive blood clotting can cause this syndrome. Some such diseases are blood disorders such as polycythemia vera and antiphospholipid syndrome. Inherited protein deficiencies can lead to a greater tendency of blood to clot (hypercoagulable state) and can affect the liver circulation. Oral contraceptives, pregnancy, infections, collagen vascular disorders, and liver, kidney, and adrenal tumors can increase the risk of this syndrome. Infections include tuberculosis and amebic abscess. Collagen vascular disorders include systemic lupus erythematosus, sarcoidosis, and inflammatory bowel disease.

This syndrome isn’t contagious or passed on from parents to children.

What Are the Symptoms of Budd-Chiari Syndrome?

Symptoms may include pain in the abdomen (belly), ascites (fluid in the abdomen), and a large liver. Ankle swelling, icterus (yellow color of whites of the eyes), and enlarged spleen occur. Untreated people develop liver damage and complications from liver failure. These complications are hepatic encephalopathy, bleeding from varicose veins in the esophagus, and kidney failure.

How Is Budd-Chiari Syndrome Diagnosed?

The health care provider makes a diagnosis from the medical history, physical examination, blood tests, and imaging studies. These studies include ultrasonography and magnetic resonance imaging (MRI). In some cases, paracentesis or liver biopsy is needed. In paracentesis, the doctor puts a needle into the abdomen to take out fluid for study. In a liver biopsy, the doctor takes a piece of liver tissue to check abnormalities with a microscope.

How Is Budd-Chiari Syndrome Treated?

Treatment is surgery to take blood around the blocked hepatic vein into the larger vein called the inferior vena cava. Sometimes, a balloon catheter may be put into the hepatic vein to open it. People with end-stage liver failure may be candidates for liver transplantation.

Medical treatment can give people short-term relief. A blood thinner, warfarin, helps prevent clotting in other veins. In some cases, balloon angioplasty and clot-busting medicines such as urokinase can be tried to remove a clot from the hepatic vein.

DOs and DON’Ts in Managing Budd-Chiari Syndrome:

  • DO understand that in about one-third of cases, no cause can be found.
  • DO realize that if the syndrome isn’t treated, less than one-third of people survive 1 year.
  • DO realize that specialists (hematologist, gastroenterologist, surgeon) can be involved in care.
  • DO call your health care provider if you have abdominal pain and bloating, ankle swelling, or jaundice (yellow skin).
  • DO call your health care provider if you need referrals to specialists.
  • DO call your health care provider if you see blood in your stool (bowel movement) or black, tarry stool.
  • DO call your health care provider if you notice confusion, lethargy, or slurred speech.
  • DON’T forget that the earlier the diagnosis, the better the prognosis.
  • DON’T forget to take your medicines, especially warfarin. It prevents more blood clots.
  • DON’T use any medicines (including over-the-counter and herbal products) without first asking your health care provider.
FOR MORE INFORMATION

Contact the following sources:

  • American College of Gastroenterologists
    Tel: (703) 820-7400
    Website: http://www.acg.gi.org
  • American Liver Foundation
    Tel: 800-465-4837
    Website: http://www.liverfoundation.org

Copyright © 2016 by Saunders, an imprint of Elsevier, Inc.

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