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Liver and intrahepatic bile ducts-nontumor

Vascular disorders

Budd-Chiari syndrome


Reviewers: Komal Arora, M.D. (see Reviewers page)
Revised: 15 May 2012, last major update May 2012
Copyright: (c) 2004-2012, PathologyOutlines.com, Inc.

General
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● Also called hepatic vein thrombosis
● Common form of venous outflow obstruction
● Either acute thrombotic occlusion (usually fatal) or subacute and chronic occlusive syndromes with hepatomegaly, weight gain, ascites, abdominal pain
● Associated with contraceptive steroids, myeloproliferative disorders
● Also paroxysmal nocturnal hemoglobinuria, pregnancy, postpartum state, intra-abdominal cancer (hepatocellular carcinoma) with inferior vena cava occlusion, idiopathic (30%)
● High mortality for acute disease
Survival: high mortality for acute thrombotic occlusion, 5-year survival of 50% for chronic form

Treatment
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● Portosystemic venous shunt (causes reverse flow through portal vein), angiography (to dilate obstruction)

Gross description
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● Swollen liver with red-purple, tense capsule

Gross images
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Normal (left) versus occluded (right) +hepatic veins

Micro description
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● Severe centrilobular congestion/necrosis, progressing to centrilobular fibrosis
● Large regenerative nodules, focal nodular hyperplasia and hepatocellular adenomas can be seen

Additional references
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Histopathology 2004;44:172, Hepatology 1998;27:488, Semin Liver Dis 2008;28:259, Hepatology 2003;37:510

End of Liver and intrahepatic bile ducts-nontumor > Vascular disorders > Budd-Chiari syndrome


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