Budd-Chiari syndrome classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2]
Overview
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.
Classification
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
Classification based on etiology
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.
Classification based on disease duration and severity
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.
Classification based on anatomical location
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.
References
- ↑ Murphy FB, Steinberg HV, Shires GT, Martin LG, Bernardino ME (1986). "The Budd-Chiari syndrome: a review". AJR Am J Roentgenol. 147 (1): 9–15. doi:10.2214/ajr.147.1.9. PMID 2940846.
- ↑ Langlet P, Escolano S, Valla D, Coste-Zeitoun D, Denie C, Mallet A, Levy VG, Franco D, Vinel JP, Belghiti J, Lebrec D, Hay JM, Zeitoun G (2003). "Clinicopathological forms and prognostic index in Budd-Chiari syndrome". J. Hepatol. 39 (4): 496–501. PMID 12971957.
- ↑ Aydinli M, Bayraktar Y (2007). "Budd-Chiari syndrome: etiology, pathogenesis and diagnosis". World J. Gastroenterol. 13 (19): 2693–6. PMC 4147117. PMID 17569137.