Budd-Chiari syndrome

Alternative names
Hepatic vein obstruction (Budd-Chiari); Hepatic veno-occlusive disease

Definition

Hepatic vein obstruction refers to a blockage of the hepatic vein, which carries blood away from the liver.

Causes, incidence, and risk factors

Hepatic vein obstruction prevents blood from flowing out of the liver and back to the heart. This blockage can cause liver damage. Obstruction of this vein can be caused by masses pressing on the vessel (tumor) or by thrombus (clot) formation within the vessel.

Most often, it is caused by conditions that increase the body’s propensity to form blood clots. These include:

     
  • Any of a number of hereditary or acquired clotting abnormalities  
  • Myeloproliferative disorders - abnormal proliferation of cells from the bone marrow  
  • Chronic inflammatory or autoimmune diseases  
  • Cancers  
  • Oral contraceptives and pregnancy  
  • Infections

Symptoms

     
  • Right-sided abdominal pain and a large liver  
  • Jaundice (yellowing of the skin)  
  • Ascites (swelling of the abdomen due to the development of fluid in the abdomen)  
  • Vomiting blood

Signs and tests

     
  • Elevated results of liver function tests  
  • Ultrasound of the liver  
  • CT scan or MRI of the abdomen  
  • Liver biopsy

Treatment

Treatment varies, depending on the cause of the obstruction, and may include the following:

     
  • Medical therapy for symptoms  
  • Anticoagulation medications  
  • Surgical interventions  
  • Radiological procedures

Complications
Hepatic vein obstruction can progress to liver failure, which can be fatal.

Calling your health care provider
Call your health care provider if you have symptoms of hepatic vein obstruction or if you are undergoing treatment and any new symptoms develop.

Johns Hopkins patient information

Last revised: December 5, 2012
by Potos A. Aagen, M.D.

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