Budd-Chiari Syndrome

What is Budd-Chiari Syndrome?

Budd-Chiari syndrome is a condition in which the hepatic veins (veins that drain the liver) are blocked or narrowed by a clot (mass of blood cells). This blockage causes blood to back up into the liver, and as a result, the liver grows larger. The spleen (an organ located on the upper left side of the abdomen that helps fight infection by filtering the blood) may also grow larger.

Budd-Chiari Syndrome may also cause other conditions, including:

• Portal hypertension (increased pressure in the portal vein, which carries blood from the intestines to the liver).
• Esophageal varices (twisted veins in the esophagus, or “food tube”).
• Ascites (a buildup of fluid in the abdomen).
• Cirrhosis (scarring of the liver).
• Varicose veins (abnormal, swollen blood vessels) in the abdomen and/or rectum.

What causes Budd-Chiari syndrome?

Budd-Chiari syndrome can be caused by conditions and situations that cause your blood to clot (form a blockage). These include:

• Myeloproliferative diseases (those that affect the blood and bone marrow), including polycythemia (the body makes too many red blood cells), and thrombocythemia (the body produces too many platelets).
• Sickle cell disease (a blood disease in which red blood cells change shape from round to sickle-shaped).
• Inflammatory bowel disease (a group of disorders that cause irritation and swelling of the digestive tract).
• Pregnancy.

What are the 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.
• Edema (swelling) in the legs.
• Liver failure.
• Hepatic encephalopathy (reduced brain functioning caused by liver disease).
• Vomiting.
• Enlarged spleen.
• Fatigue (extreme tiredness).

How is Budd-Chiari syndrome diagnosed?

Budd-Chiari syndrome is diagnosed through a physical examination and with certain tests. Your doctor will ask you about your symptoms and will look for signs of Budd-Chiari, such as ascites (swelling in the abdomen). Your doctor will also arrange for blood tests to check how well your liver is functioning and to learn if you are at greater risk for blood clots.

Imaging tests will determine if your veins are blocked by clots. These tests include:

• Ultrasound, a procedure that transmits high-frequency sound waves through body tissues. The echoes are recorded and transformed into video or photographs of the internal structures of the body.
• Computed tomography (CT) scan, which uses X-rays and computers to produce images of a cross-section of the body.
• Magnetic resonance imaging (MRI), a test that uses a large magnet, radio waves, and a computer to produce very clear pictures of the body.

To see if you've had cirrhosis (scarring of the liver), your doctor may order a liver biopsy (removal of cells or tissue for examination under a microscope).

How is Budd-Chiari syndrome treated?

Treatments for Budd-Chiari syndrome are designed to dissolve blood clots and to help improve blood flow in the liver. Treatments are usually drug therapy, non-surgical procedures, and surgery:

• Drug therapy: Your doctor will prescribe drugs to dissolve the blood clots. In addition, the blood-thinning drug warfarin (Coumadin®) is often prescribed to prevent future clots.
• Procedures: There are two non-surgical procedures used in treating Budd-Chiari syndrome: transjugular intrahepatic portosystemic shunt and percutaneous transluminal angioplasty:
  • The transjugular intrahepatic portosystemic shunt (TIPS) is a radiologic procedure in which a stent (a tubular device) is placed in the middle of the liver to reroute the blood flow. During the procedure, a radiologist makes a tunnel through the liver with a needle, connecting the portal vein (the vein that carries blood from the digestive organs to the liver) to one of the hepatic veins (the three veins that carry blood from the liver). A metal stent (a small, hollow tube) is placed in this tunnel to keep the track open. The TIPS procedure reroutes blood flow in the liver and reduces pressure in all abnormal veins, including the bowel and the liver.
  • In the percutaneous transluminal angioplasty procedure, the doctor inserts a catheter (a thin, hollow tube with a balloon at the tip) through the skin and into a blood vessel. The catheter 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: If you have liver failure (the liver no longer functions adequately), a liver transplant is the usual treatment.

What is the prognosis (outlook) for patients who have Budd-Chiari syndrome?

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.