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What is Budd Chiari Syndrome?

Budd Chiari Syndrome (BCS) is a rare liver condition where the hepatic veins are narrowed or blocked due to blood clots. Due to the blockages, blood flowing into the liver has difficulty in flowing out leading to build-up of blood in the liver. This congestion can cause hepatomegaly, ascites (fluid build-up in the peritoneal cavity) and decompensation of the liver. Other complications due to BCS are portal hypertension, esophageal varices (twisted veins in the food pipe), varicose veins in the abdomen and even cirrhosis of the liver.

The exact cause of BCS is unknown in most of the cases. BCS affects both males and females equally.

BCS can be usually treated with drugs and surgical procedures. Only in severe cases, liver transplant may be necessary. 

The causes of BCS still remain unknown. Almost 90% of the cases are idiopathic. Around 10% of patients diagnosed with BCS have another condition known as polycythemia vera (a type of blood cancer). Other causes may be due to exposure to radiation, harmful chemicals, sepsis, chemotherapy drugs or birth control pills. People with sickle cell disease are also prone to BCS due to the veno-occlusive nature of the disease.

  • Pain the upper right quadrant of the abdomen
  • Ascites (fluid in the peritoneal cavity)
  • Hepatomegaly
  • Splenomegaly (enlarged spleen)
  • Jaundice
  • Vomiting
  • Tiredness
  • Bleeding in the esophagus
  • Edema or swelling in the legs and around the ankles
  • Acute liver failure
  • Hepatic encephalopathy
BCS is diagnosed through a physical examination and a range of other tests. The doctor will usually palpate the liver to check for enlargement and check for ascites of the abdomen. You may be asked to do a complete liver function test to check the functioning of the liver. Other tests include:
  • Abdominal ultrasound
  • CT scan
  • MRI scan
  • Endoscopy
  • Biopsy to check for liver cirrhosis
There is no cure as such for BCS. Treatment involves improving blood flow in the liver by addressing any existing blood clots and blockages. Treatment may involve a combination of drugs, non-surgical procedures and surgery.
  • Drugs – like blood thinners are used to dissolve any existing clots. Warfarin is the standard drug used which can also prevent future clots
  • Non-surgical procedures – like shunting and angioplasty may be used to improve the blood flow
  • Liver transplant surgery – only in rare cases with an extremely decompensated liver, liver transplant surgery is an option