Budd Chiari Syndrome
Budd Chiari Syndrome
National Organization for Rare Disorders, Inc.
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.
- Budd's Syndrome
- Chiari's Disease
- Chiari-Budd Syndrome
- Hepatic Veno-Occlusive Disease
- Rokitansky's Disease
Related Disorders List
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
- Polycythemia Vera
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.
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.
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.
Budd-Chiari syndrome affects males and females in equal numbers. Most cases tend to affect individuals between the ages of twenty and forty.
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.
The following disorder may precede the development of Budd-Chiari syndrome. It can be useful in identifying an underlying cause of some forms of this symdrome:
Polycythemia vera is a rare chronic myeloproliferative disorder characterized by overproduction of red blood cells and elements of the bone marrow involved in the formation of red blood cells (hematopoietic elements). In most cases, affected individuals experience headaches, weakness, dizziness (vertigo), and/or a ringing noise in the ear (tinnitus). In some cases, individuals with polycythemia vera experience itching (pruritis), especially after a hot bath. Affected individuals often have an abnormally enlarged spleen (splenomegaly) and/or liver (hepatomegaly). In some cases, affected individuals may have associated conditions, including high blood pressure (hypertension), the formation of blood clots (thrombosis), rupturing of and loss of blood (hemorrhaging) from certain blood vessels, and/or Budd-Chiari syndrome. The exact cause of polycythemia vera is unknown.
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 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.
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