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What

Budd-Chiari syndrome is a disease in which the flow of blood leaving the liver is blocked. The liver becomes enlarged because of congestion—blood is able to flow into the liver but is not able to leave. This leads to liver damage and can ultimately cause liver failure.

Who

Budd-Chiari syndrome is usually found in adults, but is occasionally found in children. It is a rare disease with unknown prevalence. It affects all races, and is more commonly found in females than in males. The syndrome is often found in association with other diseases that predispose someone to the obstructed blood flow from the liver. People with these diseases are much more likely to develop Budd-Chiari syndrome than other people.

Signs and Symptoms

There are three main symptoms of Budd-Chiari syndrome, however, these symptoms can be found in other diseases as well. Their presence indicates that there is liver damage, but does not indicate the cause.

• Enlarged liver
  
• Ascites – a swollen abdomen or belly due to the accumulation of fluid within the abdominal cavity
  
• Abdominal pain Additionally, people diagnosed with Budd-Chiari may have a few of the following symptoms.
  
• Enlarged spleen
  
• jaundice – yellowing of the skin and sclera (the white part of the eyes)
  
• Swollen ankles
  
• The appearance of veins on the abdomen or back

Possible Causes

There are several health conditions that explain the development of Budd-Chiari syndrome. About 20-25% of people diagnosed with Budd-Chiari have a myeloproliferative disorder, which means that they produce an excess amount of red blood cells, white blood cells, and platelets. Because of the increased viscosity or thickness of the blood, there is a greater chance of developing blood clots. These clots can lodge in the veins of the liver or the inferior vena cava, which produces the symptoms of Budd-Chiari syndrome. About 10% of people diagnosed with this disorder have a tumor that is obstructing the outflow of blood from the liver. Another 10% either has an infection or a tumor growing inside the liver that compresses the veins. About 20% are either pregnant or taking oral contraceptives. These women are in a hypercoagulable state, which means that blood clots are more easily formed. So, there are several health conditions that may lead to Budd-Chiari syndrome, but in many cases the cause is unknown. . As many as 40% of the people diagnosed with this disorder in the United States do not have an identifiable cause.

Diagnosis

Diagnosis of Budd-Chiari syndrome is made through imaging of the liver and the surrounding blood vessels. Computer tomography (CT), magnetic resonance imaging (MRI), or ultrasound can be used to see if there is a blockage or narrowing of the veins. The best diagnostic procedure, however, is venography, in which a dye is injected into the hepatic (liver) veins. The dye allows the veins to be seen on an x-ray. Other tests may need to be done, both to determine the underlying cause, if possible, or to exclude other causes of liver failure.

Treatment

There are several approaches to treating Budd-Chiari syndrome, including drug therapy, radiologic treatment, and surgical intervention. Drugs are used to decrease coagulation (blood clot formation) and to increase urine output to rid excess fluid buildup in the abdomen. Radiologic treatment includes angioplasty and stenting. Angioplasty is a procedure in which a balloon is inflated within the blocked vein to increase its diameter for better blood flow. Stenting is normally done after an angioplasty and involves the insertion of a stiff material within the vein to help maintain the opening from further blockage. Surgical intervention helps relieve liver congestion by changing venous blood flow. In some cases, if liver damage is very severe, liver transplantation may be needed. The underlying cause of Budd-Chiari syndrome must also be treated along with relieving the current symptoms of the disease and preventing further blockage of the hepatic veins.

Prognosis

The prognosis of Budd-Chiari syndrome is better when it occurs at a younger age and if the abdominal fluid (ascites) is controllable. Prognosis also is highly dependent on the reversibility of the underlying cause, and the success of the procedures to reestablish normal blood flow.

Connect with other parents

In the spirit of community and support, Madisons Foundation offers the unique service of connecting parents of children with rare diseases. If you would like to be connected to other parents of children with this disease, please fill out this brief form.

Weblinks

Children s Liver Association for Support Services
This organization helps families of children with liver disease through support services, such as a helpline, contact with other families, and financial support.

Children s Liver Disease Foundation
This website has educational materials, information about research projects, links to other websites, and group support information for families and children. This organization is based in the United Kingdom.

The Liver Foundation for Kids
This website has a glossary of commonly used medical terms. This foundation aims to raise funding for research projects and to provide support for families.

Google Search for Budd-Chiari Syndrome

References and Sources

Hauser SC (2004). Etiology of the Budd-Chiari syndrome. UpToDate, v. 12.2. Hauser SC (2004). Symptoms, diagnosis, and treatment of the Budd-Chiari syndrome. UpToDate, v. 12.2. Roy P, Nwakakwa V, Shojamanesh H, and Khurana V (2003). Budd-Chiari Syndrome. www.emedicine.com/med/topic2694.htm