Section 10. Liver and Gallbladder Disorders

Chapter 138. Blood Vessel Disorders of the Liver

Topics: Introduction | Abnormalities of the Hepatic Artery | Veno-occlusive Disease | Budd-Chiari Syndrome | Portal Vein Thrombosis | Blood Vessel Disorders Resulting From Other Diseases

Budd-Chiari Syndrome

Budd-Chiari syndrome is a rare disorder caused by blood clots that completely or partially block the large veins that carry blood from the liver (hepatic veins).

Usually, the cause of Budd-Chiari syndrome is not known. Some affected people have a blood clotting disorder or sickle cell disease or are pregnant. Direct pressure on the veins, which may result from injury, liver abscess (a pus-filled pocket of infection), liver cancer, or kidney cancer (which can press on the hepatic veins), also increases the likelihood of developing blood clots.

Symptoms and Diagnosis

The symptoms of Budd-Chiari syndrome may begin suddenly and severely, but usually they begin gradually. The liver swells with blood and becomes tender. The blood accumulation in the liver raises the pressure in the portal vein, although the consequences may not develop for months. One such consequence of this raised pressure is the formation of dilated, twisted (varicose) veins in the esophagus (esophageal varices), which may rupture and bleed, sometimes massively, often with vomiting of blood. In addition, fluid leaks from the surface of the swollen liver into the abdominal cavity (ascites), and abdominal pain and mild jaundice (a yellowish discoloration of the skin and the whites of the eyes) occur.

Within a few days to several months, other symptoms of liver failure may occur (see Section 10, Chapter 135). The blood clots occasionally extend to block the inferior vena cava--the largest vein entering the heart. This blockage causes considerable swelling in the legs and abdomen.

The characteristic symptoms are the main clues used by the doctor to make the diagnosis. X-rays of the veins taken after injection of a radiopaque dye (a dye visible on x-rays) may reveal the precise location of the blockage. Ultrasound or magnetic resonance imaging (MRI) scanning or a liver biopsy (in which a sample of liver tissue is removed by needle for examination under a microscope) may be used to confirm the diagnosis (see Section 10, Chapter 134).

Prognosis and Treatment

Fewer than one third of people with Budd-Chiari syndrome survive for 1 year without prompt and effective treatment.

If the vein is narrowed rather than blocked, anticoagulants (drugs that prevent clots) or thrombolytics (drugs that dissolve clots) may be used. If esophageal varices develop and bleed, surgery may be performed to reduce the pressure in the portal vein. During surgery, the portal vein is connected to the inferior vena cava, causing blood flow to bypass the liver. However, this newly created connection (shunt) can increase the risk of liver encephalopathy (brain damage from liver disease). Liver transplantation (see Section 16, Chapter 187) can be an effective treatment, particularly for people with severe liver failure.