Budd-Chiari Syndrome

Blood flow out of the liver is blocked

Budd-Chiari syndrome is a condition caused by obstruction of the blood flow out of the liver, most often by a blood clot. Budd-Chiari syndrome most often occurs in individuals with underlying disorders that cause blood clotting, including antiphospholipid syndrome and myeloproliferative disorders such as polycythemia vera and paroxysmal nocturnal hemoglobinuria. Chronic inflammatory disorders such as Behcet disease, inflammatory bowel disease, sarcoidosis, Sjogren syndrome, or lupus may also cause Budd-Chiari syndrome.

Budd-Chiari syndrome affects people of all ethnic backgrounds and affects men and women equally. It is known that Budd-Chiari syndrome is a rare disorder, but exactly how often it occurs is not known.

Symptoms
Most people who develop Budd-Chiari syndrome have three main symptoms:

• Ascites, in which fluid collects in the abdominal cavity, often making the abdomen distended
• Abdominal pain
• An enlarged liver, known as hepatomegaly, because blood can flow into the liver but not out of it.

The build-up of blood in the liver can cause damage of the liver cells. If the liver is not working well, the person can develop jaundice (yellowing of the eyes and skin) and kidney problems.

Diagnosis
The usual symptoms of Budd-Chiari syndrome are not necessarily clues to its diagnosis, because those symptoms could be caused by a number of disorders. If an individual has a disorder that might cause Budd-Chiari syndrome, though, that can help with the diagnosis. The fluid that collects in the abdomen can be tested to help confirm the diagnosis. Ultrasound and magnetic resonance imaging (MRI) can help assess liver function and its blood flow. A sample (biopsy) of the liver can be taken to examine the cells under the microscope.

Treatment
If left untreated, Budd-Chiari syndrome can fatally damage the liver. Medications can be given to dissolve any existing blood clots in the liver and reduce the formation of new clots. A low-salt diet can help control ascites. Special surgical procedures can relieve the blood congestion in the liver. If the liver is badly damaged, a liver transplant may be needed.

Information for this article was taken from:
Roy, P., Nwakakwa, Shojmanesh, H., & Khurana, V. (2003). Budd-Chiari syndrome. eMedicine, accessed at http://www.emedicine.com/med/topic2694.htm