Budd-Chiari syndrome (BCS) is an extremely rare condition, affecting just one in every million people. Named after 19th-century British physician, George Budd, and his contemporary, Austrian pathologist, Hans Chiari, the disorder is characterized by an obstruction in the hepatic veins that can also affect the inferior vena cava and the right atrium. This tendency towards thrombosis affects men and women between 20 and 40 years old and makes BCS a potentially life-threatening disorder that can spur cascading complications.
The smaller veins leading from the liver to the right atrium become occluded, slowing blood flow. The impeded flow increases blood pressure in the system of veins, which causes increased filtration of vascular fluid, leading to the accumulation of ascitic fluid in the abdomen. Portal hypertension, in conjunction with the fluid's need to find alternative routes, causes esophogeal, rectal, and gastric blood vessels to swell. If this continues, congestive hepatopathy, also known as nutmeg liver, develops, along with renal failure.
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