A disease of the liver, where thrombosis has occurred in the hepatic veins [Described 1845. After George Budd (1808-82), Professor of Medicine at King’s College Hospital, London; Hans von Chiari (1851-1916), Viennese pathologist who was Professor of Pathological Anatomy at Strasbourg and later at Prague.]
A rare disorder in which the veins that drain blood from the liver become blocked or narrowed. Budd-Chiari syndrome leads to a swollen liver, portal hypertension (increased blood pressure in the portal vein, which carries blood from the intestines to the liver), and liver failure. Blockage of the veins may be due to a blood clot, pressure from a tumor, or a congenital abnormality. If left untreated, this syndrome is often fatal.
Budd-Chiari syndrome is an uncommon condition characterized by the blockage or narrowing of the veins responsible for draining blood from the liver. As a consequence, blood accumulates in the liver, leading to its enlargement. This condition can result in liver failure and portal hypertension, which is an elevated pressure in the vein responsible for carrying blood to the liver.
The treatment of Budd-Chiari syndrome focuses on addressing the underlying cause of the vein obstruction, which can stem from factors such as blood clot formation, pressure exerted on the veins by a liver tumor, or a congenital abnormality of the veins present from birth. Unfortunately, in most cases, treatment options yield limited effectiveness. Unless a liver transplant is feasible, the disease typically progresses and proves fatal within a span of two years.