Budd-Chiari syndrome classification

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

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Classification

• Budd-Chiari syndrome may be classified into several subtypes based on:
  • Etiology
  • Disease duration and severity
  • Anatomical location and Extent of the outflow obstruction

• An obstruction below 300µm in diameter is not considered as BCS by some authors.

• Budd-Chiari syndrome may be classified according to etiology into two subtypes/groups
  • Primary:Hepatic venous outflow obstruction is a result of thrombosis.
  • Secondary:Hepatic venous outflow obstruction is a result of invasion or compression by a tumor.
• Budd-Chiari syndrome may be classified according to disease duration and severity into four subtypes/groups:
  • Acute: Rapid development of clinical manifestations within weeks with intractable ascites and hepatic necrosis.
  • Subacute:Insidious onset symptoms develop over 3 months.Clinical manifestations of Ascites and hepatic necrosis may be minimal as portal and hepatic venous collaterals help in decompression of sinusoids.
  • Chronic: Associated with complications of cirrhosis.
  • Fulminant liver failure: Characterized by acute liver injury with elevated transaminases, jaundice, hepatic encephalopathy, and an elevated prothrombin time/international normalized ratio; hepatic encephalopathy develops within eight weeks after the development of jaundice.

venous collaterals are not developed in patients with acute liver failure or acute liver disease whereas venous collaterals are seen in patients with subacute and chronic liver disease.

• Budd-Chiari syndrome may be classified according to anatomical location into [number] subtypes/groups: [group1], [group2], [group3], and [group4]