Budd-Chiari Syndrome

Usually, the cause is a disorder that makes blood more likely to clot, such as the following:

Sometimes Budd-Chiari syndrome begins suddenly and rather severely, typically during pregnancy. During pregnancy, the blood normally clots more readily. In some women, a blood clotting disorder may first become apparent during pregnancy.

Other causes include disorders that develop near the hepatic veins, such as parasitic infections and liver tumors Overview of Liver Tumors Liver tumors may be noncancerous (benign) or cancerous (malignant). Cancerous liver tumors are classified as primary (originating in the liver) or metastatic (spreading from elsewhere in the... read more or kidney tumors Kidney Cancer Most solid kidney tumors are cancerous, but purely fluid-filled tumors (cysts) generally are not. Almost all kidney cancer is renal cell carcinoma. Another kind of kidney cancer, Wilms tumor... read more that press on or invade the veins. In Asia and South Africa, the cause is commonly a membrane (web) that blocks the inferior vena cava. These webs are often present at birth. Often, the cause is unknown.

Symptoms vary somewhat depending on whether the blockage develops slowly or occurs suddenly.

Usually, the blockage and symptoms develop gradually over months. Fatigue is common. The enlarged liver becomes tender, and people have abdominal pain. Fluid may accumulate in the legs, causing swelling Swelling Swelling is due to excess fluid in the tissues. The fluid is predominantly water. Swelling may be widespread or confined to a single limb or part of a limb. Swelling is often in the feet and... read more (edema), or in the abdomen, causing ascites Ascites Ascites is the accumulation of protein-containing (ascitic) fluid within the abdomen. Many disorders can cause ascites, but the most common is high blood pressure in the veins that bring blood... read more . Varicose veins may develop in the esophagus. They may rupture and bleed, sometimes profusely (see Gastrointestinal Bleeding Gastrointestinal Bleeding Bleeding may occur anywhere along the digestive (gastrointestinal or GI) tract, from the mouth to the anus. Blood may be easily seen by the naked eye (overt), or blood may be present in amounts... read more ), and people may vomit blood. Such bleeding is a medical emergency. If cirrhosis Cirrhosis of the Liver Cirrhosis is the widespread distortion of the liver's internal structure that occurs when a large amount of normal liver tissue is permanently replaced with nonfunctioning scar tissue. The scar... read more develops, it can lead to liver failure. Liver failure Liver Failure Liver failure is severe deterioration in liver function. Liver failure is caused by a disorder or substance that damages the liver. Most people have jaundice (yellow skin and eyes), feel tired... read more can cause deterioration of brain function (hepatic encephalopathy Hepatic Encephalopathy Hepatic encephalopathy is deterioration of brain function that occurs in people with severe liver disease because toxic substances normally removed by the liver build up in the blood and reach... read more ), resulting in confusion and even coma.

Blockages occur suddenly in about 20% of people. Blockages that occur during pregnancy tend to occur suddenly. Some of the symptoms are the same as those of gradually developing blockages: fatigue, an enlarged and tender liver, and abdominal pain in the upper abdomen. Additional symptoms include vomiting and a yellow discoloration of the skin and whites of the eyes (jaundice Jaundice in Adults In jaundice, the skin and whites of the eyes look yellow. Jaundice occurs when there is too much bilirubin (a yellow pigment) in the blood—a condition called hyperbilirubinemia. (See also Overview... read more ). Severe liver failure is rare.

Doctors suspect the Budd-Chiari syndrome in people with either of the following:

If results of liver tests Liver Blood Tests Liver tests are blood tests that represent a noninvasive way to screen for the presence of liver disease (for example, viral hepatitis in donated blood) and to measure the severity and progress... read more are abnormal, an imaging test, typically Doppler ultrasonography, is done. If results are unclear, magnetic resonance imaging of blood vessels (magnetic resonance angiography) or computed tomography (CT) is done (see Imaging Tests of the Liver and Gallbladder Imaging Tests of the Liver and Gallbladder Imaging tests of the liver, gallbladder, and biliary tract include ultrasonography, computed tomography (CT), magnetic resonance imaging (MRI), endoscopic retrograde cholangiopancreatography... read more ).

If surgery or a procedure to widen a blood vessel or to reroute blood flow is planned, venography is necessary. For venography, x-rays of the veins are taken after a radiopaque contrast agent Radiopaque Contrast Agents Radiographic contrast agents are substances used to distinguish between internal structures in medical imaging, such as various types of x-rays and magnetic resonance imaging (MRI). During imaging... read more (which is visible on x-rays) is injected into a vein in the groin.

A liver biopsy Biopsy of the Liver Doctors can obtain a sample of liver tissue during exploratory surgery, but more often they obtain a sample by inserting a hollow needle through the person's skin and into the liver. This type... read more may be done to confirm the diagnosis and determine whether cirrhosis Cirrhosis of the Liver Cirrhosis is the widespread distortion of the liver's internal structure that occurs when a large amount of normal liver tissue is permanently replaced with nonfunctioning scar tissue. The scar... read more has developed.

When the vein remains completely blocked, most people, if untreated, die of liver failure Liver Failure Liver failure is severe deterioration in liver function. Liver failure is caused by a disorder or substance that damages the liver. Most people have jaundice (yellow skin and eyes), feel tired... read more within 3 years. When the blockage is incomplete, life expectancy is longer but varies.

Treatment depends on how rapidly the disorder developed and how severe it is.

When symptoms begin suddenly and the cause is a clot, fibrinolytic (thrombolytic) drugs, which dissolve clots, help. For long-term treatment, anticoagulant drugs (such as warfarin) are often needed to prevent clots from enlarging or recurring.

If a vein is blocked by a web or is narrowed, angioplasty may be done to clear or widen it. For this procedure (called percutaneous transluminal angioplasty), a catheter with a deflated balloon at its tip is inserted through the skin into a blood vessel (such as a vein in the neck) and threaded to the blocked vein. The balloon is inflated, widening the vein. A wire mesh tube (stent) can also be inserted and left in place to keep the vein open.

Another solution is to create an alternate route for blood flow, bypassing the liver. This procedure, called transjugular intrahepatic portal-systemic shunting Portosystemic shunting Portal hypertension is abnormally high blood pressure in the portal vein (the large vein that brings blood from the intestine to the liver) and its branches. Cirrhosis (scarring that distorts... read more (TIPS), reduces pressure in the portal vein. For the procedure, a local anesthetic is used to numb the neck, and a catheter with a cutting needle is inserted into a vein in the neck (jugular vein). The catheter is threaded through the vena cava to the hepatic vein. The needle is used to create a connection (called a shunt) between a branch of the hepatic vein and the portal vein, so that blood can bypass the liver. Then, a stent is threaded to and placed in the shunt to keep it open. The shunt enables blood to bypass the liver, flowing from the portal vein (which normally brings blood to the liver) directly to the hepatic veins (which drain blood away from the liver). The blood returns to the heart through the inferior vena cava. However, such shunts increase the risk of hepatic encephalopathy Hepatic Encephalopathy Hepatic encephalopathy is deterioration of brain function that occurs in people with severe liver disease because toxic substances normally removed by the liver build up in the blood and reach... read more (deterioration of brain function due to liver dysfunction). Also, shunts occasionally become blocked, especially in people who have a tendency to form blood clots.

Liver transplantation Liver Transplantation Liver transplantation is the surgical removal of a healthy liver or sometimes a part of a liver from a living person and then its transfer into a person whose liver no longer functions. (See... read more can be lifesaving, particularly for people with severe liver failure Liver Failure Liver failure is severe deterioration in liver function. Liver failure is caused by a disorder or substance that damages the liver. Most people have jaundice (yellow skin and eyes), feel tired... read more .

Problems resulting from the disorder are also treated:

Most people need to take anticoagulants indefinitely to prevent new blockages from developing.