MedMaven

by Dr. C.H. Weaver M.D. updated 10/2020

Budd-Chiari syndrome is a condition in which the hepatic veins in the liver are blocked or narrowed by a blood clot or clots. The blockage of blood flow causes blood to back up into the liver, and as a result, the liver grows larger. Without treatment, people who have a completely blocked hepatic vein can die of liver failure within three years. Survival in this case is improved with liver transplantation. People who have partially blocked veins can live longer, but the life expectancy is different for each person.

What causes Budd-Chiari syndrome?

Budd-Chiari syndrome is caused by conditions and situations that cause an individual’s blood to clot inappropriately. Several types of cancer and blood disorders may cause Budd-Chiari an these include:

  • Myeloproliferative disorders including polycythemia vera and essential thrombocythemia.
  • Sickle cell disease
  • Inflammatory bowel disease

What are the signs and symptoms of Budd-Chiari syndrome?

The symptoms of Budd-Chiari syndrome include:

  • Pain in the upper abdomen.
  • Ascites (swelling in the abdomen caused by excess fluid).
  • Jaundice (skin, whites of the eyes and mucous membranes turn yellow).
  • Enlarged and tender liver.
  • Bleeding in the esophagus from esophageal varices
  • Edema (swelling) in the legs.
  • Vomiting.
  • Enlarged spleen.
  • Fatigue
  • Liver failure and hepatic encephalopathy (reduced brain functioning caused by liver disease).

How is Budd-Chiari syndrome diagnosed?

Budd-Chiari syndrome is diagnosed with certain tests that can determine if the hepatic veins are blocked by clots. These tests include ultrasound, CT and MRI scans.

How is Budd-Chiari syndrome treated?

Treatments for Budd-Chiari syndrome are designed to prevent additional blood clots from forming, dissolve existing blood clots and to help improve blood flow in the liver. Treatment may consist of drug therapy, non-surgical procedures, and/or surgery:

  • Drug therapy:
    • Drugs to dissolve the blood clots.
    • Warfarin (Coumadin®) is often prescribed to prevent future clots.
  • Transjugular intrahepatic portosystemic shunt (TIPS) is a procedure in which a stent (a tubular device) is placed in the liver to reroute the blood flow. Under radiographic guidance a radiologist makes a tunnel through the liver with a needle that connect the portal vein to one of the hepatic veins and places the stent in this tunnel. The procedure reroutes blood flow in the liver and reduces pressure in all abnormal veins.
  • Percutaneous transluminal angioplasty. A catheter (a thin, hollow tube with a balloon at the tip) is inserted through the skin and into a blood vessel then is guided to the area where the clot is located. When the catheter reaches the clot, the balloon is inflated to widen the vein. A stent may be placed at the site to keep the vein open.
  • Surgery: For individuals with liver failure a liver transplant is the usual treatment.
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Myeloproliferative Neoplasms MPN

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