$Unique_ID{BRK03547} $Pretitle{} $Title{Budd-Chiari Syndrome} $Subject{Budd-Chiari Syndrome Hepatic Veno-Occlusive Disease Chiari's Disease Chiari-Budd Syndrome Rokitansky's Disease Budd's Syndrome Lesions of the Hepatic Artery Lesions of the Hepatic Venous System} $Volume{} $Log{} Copyright (C) 1988, 1989 National Organization for Rare Disorders, Inc. 583: Budd-Chiari Syndrome ** IMPORTANT ** It is possible that the main title of the article (Budd-Chiari Syndrome) is not the name you expected. Please check the SYNONYM listing to find the alternate names and disorder subdivisions covered by this article. Synonyms Hepatic Veno-Occlusive Disease Chiari's Disease Chiari-Budd Syndrome Rokitansky's Disease Budd's Syndrome Information on the following diseases can be found in the Related Disorders section of this report: Lesions of the Hepatic Artery Lesions of the Hepatic Venous System General Discussion ** REMINDER ** The information contained in the Rare Disease Database is provided for educational purposes only. It should not be used for diagnostic or treatment purposes. If you wish to obtain more information about this disorder, please contact your personal physician and/or the agencies listed in the "Resources" section of this report. Budd-Chiari Syndrome is characterized by abnormal enlargement of the liver. Swelling of the abdomen and legs occurs and increased pressure in the portal vessels may result in serious damage to the liver. Symptoms Budd-Chiari Syndrome involves obstruction 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 atrium of the heart. This syndrome may begin gradually or abruptly. There may be increased pressure (portal hypertension) in the veins carrying blood from the gastrointestinal (GI) tract back to the heart through the liver. Impaired liver function also develops. Liver biopsy tests show central cell deterioration, development of fibrous growths and blockage (occlusion) of the terminal hepatic (liver) veins. When vein blockage is severe, onset of the disorder can be very sudden, and may be accompanied by severe pain. If the disease is chronic, onset may be gradual. Pain in the stomach region or right area of the abdomen, with abdominal swelling is noticed. Some patients may only have abdominal swelling while others may suddenly begin passing blood in their urine (hemoglobinuria). There may also be unusual swelling (edema) of the legs. Causes The exact cause of approximately 70% of all cases of Budd-Chiari Syndrome is not known. 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 Population Budd-Chiari Syndrome affects males and females in equal numbers. Most cases tend to affect patients between the ages of twenty and forty. Related Disorders Symptoms of the following disorders can be similar to those of Budd-Chiari Syndrome. Comparisons may be useful for a differential diagnosis: Lesions of the Hepatic (liver) Artery. Hepatic Artery Blockage (occlusion) is usually caused by clotting (thrombosis/embolism) or surgical ligation. The blockage may result in the destruction of tissue in the liver. However, the outcome is unpredictable since each individual reacts to these situations differently. Ballooning of the vessel wall (aneurysm) of the hepatic artery can occur as a result of infection, arteriosclerosis, trauma, or other disorders. Aneurysms are often multiple and tend to rupture into the abdominal cavity (peritoneum), common bile duct, or adjacent hollow organs. The ruptured hepatic artery aneurysm can cause upper abdominal colic, obstructive jaundice, or gastrointestinal (GI) tract bleeding (hemorrhage). Early surgery on the hepatic artery aneurysm is recommended to avoid complications. Lesions of the Hepatic Venous System. Veno-Occlusive Disease involves injury or disease of the terminal hepatic veins or the small hepatic vein system inside the liver. The larger branches of the hepatic vein system are not involved. Obstruction of the flow from the hepatic sinisoids which results in liver cell damage. Therapies: Standard Treatment of Budd-Chiari Syndrome is most successful if it is diagnosed early. There are many methods of identifying Budd-Chiari Syndrome. Conventional methods of X-ray and CT scanning may be useful. Magnetic Resonance Imagining (MRI) and Ultrasound are also used as diagnostic procedures. There are also various treatments. The use of balloon dilation of veins (angioplasty) may ease high pressure in the vessel walls. The use of high doses of the drug prednisone, or diverting blood flow from one vein to another (shunting), may also be recommended. Therapies: Investigational This disease entry is based upon medical information available through December 1988. Since NORD's resources are limited, it is not possible to keep every entry in the Rare Disease Database completely current and accurate. Please check with the agencies listed in the Resources section for the most current information about this disorder. Resources For more information on Budd-Chiari Syndrome, please contact: National Organization for Rare Disorders (NORD) P.O. Box 8923 New Fairfield, CT 06812-1783 (203) 746-6518 American Liver Foundation 1425 Pompton Ave. Cedar Grove, N.J. 07009 (201) 857-2626 (800) 223-0179 The United Liver Foundation 11646 West Pico Blvd. Los Angeles, CA 90064 (213) 445-4204 or 445-4200 Children's Liver Foundation 14245 Ventura Blvd. Sherman Oaks, CA 91423 (818) 906-3021 National Digestive Diseases Information Clearinghouse Box NDDIC Bethesda, MD 20892 (301) 468-6344 References INTERNAL MEDICINE, 2nd Ed.: Jay H. Stein, ed.-in-chief; Little, Brown and Co., 1987. Pp. 236, 1067. TREATMENT OF THE BUDD-CHIARI SYNDROME WITH PERCUTANEOUS TRANSLUMINAL ANGIOPLASTY. CASE REPORT AND REVIEW OF THE LITERATURE. J. Sparano, et al.; Am J Med (April, 1987, issue 82 (4)). Pp. 821-828. RESULTS OF PORTAL SYSTEMIC SHUNTS IN BUDD-CHIARI SYNDROME. C. Vons, et al.; Ann Surg (April, 1986, issue 203 (4)). Pp. 366-370. COMPARISON OF ULTRASONOGRAPHY, COMPUTED TOMOGRAPHY AND 99mTc LIVER SCAN IN DIAGNOSIS OF BUDD-CHIARI SYNDROME. S. Gupta, et al.; Gut (March, 1987, issue 28 (3)). Pp. 242-247.