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Budd-Chiari (pronounced bud kee-ah-ree) syndrome is a rare liver disease - even more rare in children. It involves clotting of blood in the hepatic veins, which carry blood out of the liver. When the veins of the liver are blocked, blood is prevented from flowing out of the liver and returning to the heart. Budd-Chiari syndrome produces mild to severe liver damage; this varies from patient to patient.
There are multiple causes of Budd-Chiari syndrome, mainly due to an increased tendency of the body to form blood clots. Most patients with Budd-Chiari syndrome have an underlying condition that predisposes them to blood clotting. However, in many cases, the exact Budd-Chiari syndrome causes are unknown.
Blood disorders are the most common cause of Budd-Chiari syndrome in the United States. These mainly include myeloproliferative disorders (abnormal spread of cells from the bone marrow) such as polycythemia (an increased number of red blood cells), and sickle cell disease, a genetic blood disorder. Careful monitoring and controlling of these conditions is the most effective known method of preventing Budd-Chiari syndrome.
In adult women, Budd-Chiari syndrome has also been linked to the use of oral contraceptives (birth control pills) and pregnancy. In other cases, it may be inherited. Other known causes include:
The most common Budd-Chiari syndrome symptom is ascites, or fluid build-up in the abdomen. This is caused by the pressure created by blockages in the veins. The pressure also sometimes causes hepatomegaly, or enlargement of the liver. Patients may also have abnormal blood tests indicative of liver disease. Some individuals with Budd-Chiari syndrome may be jaundiced (have yellow skin). Pain in the upper right part of the abdomen is another common symptom.
Other symptoms include:
An internist (a physician who specializes in diseases of the internal organs), a gastroenterologist (a physician who specializes in digestive system disorders), or a general surgeon can diagnose Budd-Chiari syndrome. Often, the examining doctors first suspect cirrhosis as a cause of the symptoms in patients with Budd-Chiari syndrome. When doing a physical examination, the doctor may find that the liver is enlarged. Further testing may include:
Once abnormalities are found, additional testing may be done:
In some cases, anti-clotting medications such as urokinase may be prescribed to break up blockages as pediatric Budd-Chiari syndrome treatment. This is usually done when the clotting is sudden, and the clots have not been in place for long.
Most pediatric patients with Budd-Chiari syndrome need surgery to unblock their veins. A balloon catheter may be used to open up the blocked hepatic veins. Many patients have surgery to insert a shunt. The shunt acts as a bypass system, rerouting blood flow around the clotted vein and into a large vein called the vena cava.
For those who have deteriorating liver function and complications (such as cirrhosis), liver transplantation is usually the best option.
The prognosis for people diagnosed with Budd-Chiari syndrome often depends on where the blockage is, and the number of veins that are blocked. The underlying cause of the disease, time of diagnosis, duration of symptoms, and the form of treatment are also critically important factors. Long-term survival is possible when treatment is begun before permanent liver damage occurs. Liver cells can fix themselves if there is no permanent damage.
Learn about other Liver Disease States.
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One Children’s Hospital Way
4401 Penn Ave.
Pittsburgh, PA 15224
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