Budd-Chiari syndrome is a very rare condition that occurs only in about one person in a million. It affects the hepatic veins, causing serious problems. To fully understand, we should know some facts about the venous blood system in the liver. Venous blood is brought into the liver tissue by the portal vein, which carries blood from the intestines and spleen. The blood is cleaned from toxic substances and then it is led out of the liver by hepatic veins that open into the inferior vena cava. Budd-Chiari syndrome leads to closure of those hepatic veins and the blood can not properly flow out of the liver.
In some cases, there is no clear cause of the condition. Other times, the closure of hepatic veins is caused by blood clots, whose occurrence may be related to usage of hormonal contraception, by local tumors (such as the liver cancer) or by congenital defects of hepatic veins.
The symptoms are usually serious. The blood outflow from liver is disrupted and blood accumulates in the liver tissue. This overpressure is then transferred into the portal vein and this leads to gradual development of portal hypertension with all its consequences including ascites, esophageal varices, etc. (see relevant article). Blood accumulating in the liver may damage it, resulting in non-alcoholic liver cirrhosis with increased risk of gradual liver failure.
Medical history and physical examination help us to find the above-mentioned symptoms. In blood tests we find elevated liver tests expressing liver damage. Abdominal ultrasound and computed tomography are useful to find signs of liver cirrhosis and diagnose any organic disorders of the hepatic veins (blood clots, tumors).
The therapy may be conservative – therapy of portal hypertension (see related article) and administration of anticoagulants in thrombosis of hepatic veins. TIPS may be also used. It is an artificial connection of portal system and hepatic veins. It is an invasive way of treatment of complicated portal hypertension. Advanced forms of Budd-Chiari syndrome may be sometimes solved by liver transplant.