What is Budd-Chiari Syndrome?

Budd-Chiari Syndrome

is a condition caused by the sudden blockage of the hepatic (liver) veins.The blockage could be caused by a blood clot, cancer or any other physical obstruction.

It is a very rare disease and occurs in 1 out of a million individuals.

It is more common in females than males. 10-20% of patients of the discussed condition also report the obstruction of the hepatic portal vein. The hepatic portal vein is the main blood vessel which drains blood from the gastrointestinal cavity and spleen to the liver.

Budd-Chiari syndrome

Causes of Budd-Chiari Syndrome


The primary (75%) and the most detected cause of this syndrome is thrombosis or formation of blood clot in the hepatic vein. Some of the conditions which causes thrombosis are given below:

  • Inflammatory or auto immune disorder
  • A blood clotting disorder
  • Polycythemia vera (Excess red blood cells)
  • Use or oral contraceptives
  • Pregnancy
  • Post pregnancy
  • Bacterial infections such as TB


The secondary (25%) cause of this syndrome is obstruction of blood flow due to a tumor.


Cancerous masses also cause obstruction.


Rarely, patients of bone marrow transplants may develop a blockage as a reaction to medication.


  • Intense abdominal pain
  • Hepatomegaly (Enlarged Liver)
  • Jaundice
  • Vomiting Blood
  • Ascites (Abnormal fluid retention in the abdomen)
  • Elevated Liver enzymes
  • Encephalopathy ( brain damage and dysfunction).
  • Destruction of liver tissues as in Cirrhosis of liver

Treatment of Budd-Chiari Syndrome

Treatment depends a lot on the underlying cause of the syndrome.

  • Clot dissolving medication like urokinase directly injected in the vein is used to remove blood clots.
  • Blood thinners or anti coagulants like Heparin or Warfarin are used in individuals with blood clotting disorders to prevent further complications.
  • Patients of ascites are put on a sodium restricted diet and diuretics (medicines which expel accumulated water from body).
  • Surgical intervention may be required. Shunts could be placed to make an alternate route for the blood to flow bypassing the obstruction. The transjugular intrahepatic portosystemic shunt (TIPS) is a shunting procedure with very low mortality rates.
  • Angioplasty can be done in patients with venal cava obstruction whereby narrowing blood vessels due to obstruction is mechanically widened by the use of a balloon catheter.

When every other procedure fails and the disease progresses towards cirrhosis with a life expectancy of less than a year then liver transplantation is the only viable option. The survival chances from

Budd-Chiari syndrome

after transplantation improves to 69-87%.

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