Budd-Chiari syndrome classification

Budd-Chiari syndrome (BCS) may be classified into several subtypes based on etiology, disease duration, severity and anatomical location of the 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 primary and secondary sub-types. Budd-Chiari syndrome may be classified according to disease duration and severity into acute, subacute, chronic, fulminant liver failure. Budd-Chiari syndrome may be classified according to the anatomical location of obstruction into type I (truncal), type II -(radicular) and type III (venooclusive) disease.

Budd-Chiari syndrome may be classified into several subtypes based on:^[1][2][3]

• 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): inferior vena cava occlusion with or without the involvement of hepatic veins.
• Type II (radicular): major hepatic veins occlusion.
• Type III (veno-occlusive): small centrilobular veins occlusion.

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