Budd-Chiari syndrome classification: Difference between revisions
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*Budd-Chiari syndrome may be classified according to etiology into two subtypes/groups | *Budd-Chiari syndrome may be classified according to etiology into two subtypes/groups | ||
**Primary:Hepatic venous outflow obstruction is a result of thrombosis. | **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. | **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 | *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. | **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. | **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. | **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. | **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. | *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 of obstruction into 3 subtypes: Type I - truncal type | *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. | |||
==References== | ==References== |
Revision as of 17:05, 2 November 2017
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:
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Overview
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.