An Overview of Budd-Chiari Syndrome

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Budd-Chiari syndrome is a condition caused by obstruction of blood flow out of the liver, most often by a blood clot. Primary Budd-Chiari syndrome is present when there is obstruction due to a primarily venous process (thrombosis or phlebitis). Secondary Budd-Chiari is present when there is compression or invasion of veins in the liver by a lesion that originates outside of the vein (such as a tumor).

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 Behçet disease, inflammatory bowel disease, sarcoidosis, Sjögren's 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. Epidemiological studies suggest that in non-Asian countries, Budd-Chiari syndrome is more common in women and usually presents in the third or fourth decade of life (although it may occur in children or older adults). However, in Asia, there is a slight predominance of men, with a median age of 45 years at presentation.

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 to 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. Management is tailored to patient's clinical and anatomical presentation. 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.

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  • Roy, P., Nwakakwa, Shojmanesh, H., & Khurana, V. (2003). Budd-Chiari syndrome. eMedicine.