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Budd-Chiari Syndrome

(Budd-Chiari's Syndrome)

By

Whitney Jackson

, MD, University of Colorado School of Medicine

Last full review/revision May 2020| Content last modified May 2020
Click here for the Professional Version
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Budd-Chiari syndrome is caused by blood clots that completely or partially block blood flow from the liver. The blockage may occur anywhere from the small and large veins that carry blood from the liver (hepatic veins) to the inferior vena cava.

  • Some people have no symptoms, but others experience fatigue, abdominal pain, nausea, and jaundice.

  • Fluid may accumulate in the abdomen, the spleen may enlarge, and sometimes severe bleeding occurs in the esophagus.

  • Doppler ultrasonography can detect narrowed or blocked veins.

  • Drugs may be used to dissolve or decrease the size of the clot, or a connection may be made between veins to allow blood to bypass the liver.

Budd-Chiari syndrome usually occurs when a clot narrows or blocks the hepatic veins, which carry blood out of the liver.

Because blood flow out of the liver is impeded, blood backs up in the liver, causing it to enlarge. The spleen may also enlarge. This backup of blood (congestion) causes blood pressure in the portal vein (which carries blood to the liver from the intestines) to increase. This increased pressure, called portal hypertension Portal Hypertension 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 , can result in dilated, twisted (varicose) veins in the esophagus (esophageal varices Gastrointestinal Bleeding Bleeding may occur anywhere along the digestive (gastrointestinal [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 Gastrointestinal Bleeding ). Portal hypertension, plus the engorged and damaged liver, leads to fluid accumulating in the abdomen (called 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 ). The kidneys contribute to ascites by causing salt and water to be retained.

The clot may extend to also block the inferior vena cava (the large vein that carries blood from the lower parts of the body, including the liver, to the heart). Varicose veins in the abdomen near the skin’s surface may develop and become visible.

Causes of 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.

Symptoms of Budd-Chiari Syndrome

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 Swelling (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 [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 Gastrointestinal Bleeding ), 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 Cirrhosis of the Liver 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 Jaundice in Adults ). Severe liver failure is rare.

Diagnosis of Budd-Chiari Syndrome

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

  • An enlarged liver, ascites, liver failure, or cirrhosis when there is no obvious cause, even after testing

  • Abnormal results of blood tests done to evaluate the liver plus conditions that increase the risk of blood clots

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 During imaging tests, contrast agents may be used to distinguish one tissue or structure from its surroundings or to provide greater detail. Contrast agents include Radiopaque contrast agents... read more (which is visible on x-rays) is injected into a vein in the groin.

Prognosis of Budd-Chiari Syndrome

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 of Budd-Chiari Syndrome

  • Drugs (to dissolve or prevent blood clots)

  • Procedures to improve blood flow

  • Liver transplantation (in people with liver failure)

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.

Problems resulting from the disorder are also treated:

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

Drugs Mentioned In This Article

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COUMADIN
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Jaundice in Adluts
Jaundice is a condition in which the skin and white parts of the eyes appear yellow. It occurs when there is too much bilirubin, a yellow waste product, in the blood. Which of the following is NOT a common cause of jaundice?
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