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 of occlusion

• 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:
  • 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 the 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 the anatomical location of obstruction into 3 subtypes:
  • Type I - truncal type: inferior vena cava occlusion with or without the involvement of hepatic veins.
  • Type II - radicular type: major hepatic veins occlusion.
  • Type III - venooclusive type: small centrilobular veins occlusion.