What is Budd-Chiari syndrome?

Updated: Oct 10, 2018
  • Author: Praveen K Roy, MD, AGAF; Chief Editor: BS Anand, MD  more...
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Answer

Budd-Chiari syndrome is an uncommon condition induced by thrombotic or nonthrombotic obstruction of the hepatic venous outflow and is characterized by hepatomegaly, ascites, and abdominal pain. [49] It most often occurs in patients with an underlying thrombotic diathesis, including in those who are pregnant or who have a tumor, a chronic inflammatory disease, a clotting disorder, an infection, or a myeloproliferative disorder, such as polycythemia vera or paroxysmal nocturnal hemoglobinuria. (See Etiology and Presentation.)

Obstruction of large- or small-caliber veins leads to hepatic congestion as blood flows into, but not out of, the liver. Microvascular ischemia due to congestion causes hepatocellular injury. Portal hypertension and liver insufficiency result. [5, 6, 49] (See Pathophysiology.)

Budd-Chiari syndrome should be considered separate from veno-occlusive disease (VOD), also known as sinusoidal obstruction syndrome, which is characterized by toxin-induced, nonthrombotic obstruction of prehepatic veins (see the images below). (See Presentation and Workup.)


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