Budd-Chiari syndrome classification: Difference between revisions
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==Classification== | ==Classification== | ||
*Budd-Chiari syndrome may be classified into several subtypes based on: | *Budd-Chiari syndrome may be classified into several subtypes based on:<ref name="pmid2940846">{{cite journal |vauthors=Murphy FB, Steinberg HV, Shires GT, Martin LG, Bernardino ME |title=The Budd-Chiari syndrome: a review |journal=AJR Am J Roentgenol |volume=147 |issue=1 |pages=9–15 |year=1986 |pmid=2940846 |doi=10.2214/ajr.147.1.9 |url=}}</ref><ref name="pmid12971957">{{cite journal |vauthors=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 |title=Clinicopathological forms and prognostic index in Budd-Chiari syndrome |journal=J. Hepatol. |volume=39 |issue=4 |pages=496–501 |year=2003 |pmid=12971957 |doi= |url=}}</ref><ref name="pmid17569137">{{cite journal |vauthors=Aydinli M, Bayraktar Y |title=Budd-Chiari syndrome: etiology, pathogenesis and diagnosis |journal=World J. Gastroenterol. |volume=13 |issue=19 |pages=2693–6 |year=2007 |pmid=17569137 |pmc=4147117 |doi= |url=}}</ref> | ||
**Etiology | **Etiology | ||
**Disease duration and severity | **Disease duration and severity |
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Overview
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.
- 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.
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.