Budd Chiari Syndrome

Budd Chiari Syndrome

National Organization for Rare Disorders, Inc.

Important

It is possible that the main title of the report Budd Chiari Syndrome is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report.

Synonyms

  • Budd's Syndrome
  • Chiari's Disease
  • Chiari-Budd Syndrome
  • Hepatic Veno-Occlusive Disease
  • Rokitansky's Disease

Disorder Subdivisions

  • None

General Discussion

Budd-Chiari syndrome is a rare disorder characterized by narrowing and obstruction (occlusion) of the veins of the liver (hepatic veins). Symptoms associated with Budd Chiari syndrome include pain in the upper right part of the abdomen, an abnormally large liver (hepatomegaly), and/or accumulation of fluid in the space (peritoneal cavity) between the two layers of the membrane that lines the stomach (ascites). Additional findings that may be associated with the disorder include nausea, vomiting, and/or an abnormally large spleen (splenomegaly). The severity of the disorder varies from case to case, depending upon the site and number of affected veins. In some cases, if the major hepatic veins are involved, high blood pressure in the veins carrying blood from the gastrointestinal (GI) tract back to the heart through the liver (portal hypertension) may be present. In most cases, the exact cause of Budd-Chiari syndrome is unknown.

Symptoms

Budd-Chiari syndrome involves obstruction or narrowing (occlusion) of the outflowing veins from either large regions of the liver or the entire liver. Blood clots or congenital webbing occur at the junction of these vessels with the large vein that carries the blood from the lower part of the body (inferior vena cava) to the right upper chamber (atrium) of the heart. This syndrome may begin gradually or abruptly.



Symptoms associated with Budd-Chiari syndrome include pain in the upper right part of the abdomen, an abnormally enlarged liver (hepatomegaly), yellowing of the skin and the whites of the eyes (jaundice), and/or accumulation of fluid in the space (peritoneal cavity) between the two layers of the membrane that lines the stomach (ascites). In some cases, there may be increased blood pressure in the veins carrying blood from the gastrointestinal (GI) tract back to the heart through the liver (portal hypertension). In some cases, impaired liver function may also develop. Liver biopsy tests show central cell deterioration, development of fibrous growths and blockage (occlusion) of the terminal liver veins. When vein blockage is severe, onset of the disorder can be sudden, and may be accompanied by severe pain. If the disease is chronic, onset may be gradual. In some cases, there may also be unusual swelling due to abnormal accumulation of fluid (edema) within the tissues of the legs.



In some cases, affected individuals may have scarring (fibrosis) and impaired functioning of the liver (cirrhosis) before or following the development of Budd-Chiari syndrome.

Causes

The exact cause of approximately 70% of all cases of Budd-Chiari syndrome is unknown. Approximately 10% of individuals with Budd-Chiari syndrome have polycythemia vera. (For more information on this disorder, see the Related Disorders section of this report.)



Symptoms develop due to blockage of the major veins that carry blood from the liver to the heart. The blockage is usually due to clotting or overgrowth of fibrous tissue in the veins. The blockage may, in some cases, be caused by Pyrrolidizine plant alkaloids (bush tea) used in some parts of the world as a drink. Other identified causes may be exposure to radiation, arsenic, trauma, blood poisoning (sepsis), vinyl chloride monomer, cancer, or some cancer chemotherapy drugs, as well as the use of birth control pills.

Affected Populations

Budd-Chiari syndrome affects males and females in equal numbers. Most cases tend to affect individuals between the ages of twenty and forty.

Standard Therapies

Diagnosis

A diagnosis of Budd-Chiari syndrome is made based upon a thorough clinical evaluation, a detailed patient history, and a variety of specialized tests. A procedure in which a radiographic dye is administered into the body to allow for x-rays of the blood vessels (angiography) is often used to aid diagnosis. Magnetic resonance imagining (MRI) and ultrasound are also used as diagnostic procedures. During MRI, a magnetic field and radio waves are used to create cross-sectional images of organs and structures in the body. Surgical removal and microscopic evaluation of liver tissue (biopsy) may be helpful in diagnosis of Budd-Chiari syndrome.



Treatment

Treatment of Budd-Chiari syndrome is most successful if the disorder is diagnosed early. There are also various methods of treatment. The use of high doses of the corticosteroid drug, prednisone, may also be administered. Drugs that hinder blood clotting (anticoagulants) such as heparin may be beneficial for treatment of individuals with Budd-Chiari syndrome.



Surgical widening (dilation) of affected veins (angioplasty) may ease high pressure in the vessel walls. In some cases, Budd-Chiari syndrome may be treated surgically by diverting blood flow from one vein to another (shunting). In other cases, a blocked vein may be cleared out and then a slender rod (stent) may be inserted into the vein to maintain blood flow. In serious cases of Budd-Chiari syndrome, liver transplantation may be necessary.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.



For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:



Tollfree: (800) 411-1222

TTY: (866) 411-1010

Email: prpl@cc.nih.gov



For information about clinical trials sponsored by private sources, contact:

www.centerwatch.com

References

TEXTBOOKS

Internal Medicine, 4th Ed.: Jay H. Stein, Editor-In-Chief; Mosby-Year Book, Inc., 1994. Pp. 632-33.



Leevy CM, et al, eds. Diseases of the Liver and Biliary Tract: Standardization of Nomenclature, Diagnostic Criteria and Prognosis. New York, NY: Raven Press; 1994:161-62.



JOURNAL ARTICLE

Orloff MJ, et al. A 27-year experience with surgical treatment of Budd-Chiari syndrome. Ann Surg. 2000;232:340-52.



Singh V, et al. Budd-Chiari syndrome: our experience of 71 patients. J Gastroenterol Hepatol. 2000;15:550-54.



Zhou H, et al. Multiple macroregenerative nodules in liver cirrhosis due to Budd-Chiari syndrome. Case reports and review of the literature. Hepatogastroenterology. 2000;47:522-27.



Michl P, et al. Successful treatment of chronic Budd-Chiari syndrome with a transjugular intrahepatic portosystemic shunt. J Hepatol. 2000;32:516-20.



Chunqing Z, et al. Ultrasonically guided percutaneous transhepatic vein stent placement for Budd-Chiari syndrome. J Vasc Interv Radiol. 1999;10:933-40.



Dumortier J, et al. The Budd-Chiari syndrome (hepatic vein obstruction). The diagnostic and therapeutic management of acute and subacute forms. Presse Med. 1999;28:802-08.



Fisher NC, et al. Managing Budd-Chiari syndrome: a retrospective study of percutaneous hepatic vein angioplasty and surgical shunting. Gut. 1999;44:568-74.



Knoop M, et al. Treatment of advanced Budd-Chiari syndrome by liver transplantation. Chirurg. 1998;69:1362-68.



Gupta S, et al. Comparison of ultrasonography, computed tomography and 99mtc liver scan in diagnosis of Budd-Chiari syndrome. Gut. 1987;28:242-47.



Sparano J, et al. Treatment of the Budd-Chiari syndrome with percutaneous transluminal angioplasty. Case report and review of the literature. Am J Med. 1987;82:821-28.



Vons C, et al. Results of portal systemic shunts in Budd-Chiari syndrome. Ann Surg. 1986;203:366-70.

Resources

American Liver Foundation

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New York, NY 10006

USA

Fax: (212)483-8179

Tel: (800)465-4837

Email: http://www.liverfoundation.org/contact/

Internet: http://www.liverfoundation.org



NIH/National Institute of Diabetes, Digestive & Kidney Diseases

Office of Communications & Public Liaison

Bldg 31, Rm 9A06

31 Center Drive, MSC 2560

Bethesda, MD 20892-2560

Tel: (301)496-3583

Email: NDDIC@info.niddk.nih.gov

Internet: http://www2.niddk.nih.gov/



Genetic and Rare Diseases (GARD) Information Center

PO Box 8126

Gaithersburg, MD 20898-8126

Tel: (301)251-4925

Fax: (301)251-4911

Tel: (888)205-2311

TDD: (888)205-3223

Internet: http://rarediseases.info.nih.gov/GARD/



For a Complete Report

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