Budd-Chiari syndrome classification: Difference between revisions
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==Overview== | ==Overview== | ||
Budd-Chiari syndrome 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 two subtypes: primary and secondary.Budd-Chiari syndrome may be classified according to disease duration and severity into four subtypes: acute, subacute, chronic, fulminant liver failure.Budd-Chiari syndrome may be classified according to the anatomical location of obstruction into 3 subtypes: type I - truncal type, type II - radicular type, type III - venooclusive type. | Budd-Chiari syndrome 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 two subtypes: primary and [[secondary]]. Budd-Chiari syndrome may be classified according to [[disease]] duration and severity into four subtypes: [[acute]], [[subacute]], [[chronic]], [[fulminant liver failure]]. Budd-Chiari syndrome may be classified according to the [[Anatomical|anatomical location]] of [[obstruction]] into 3 subtypes: type I - truncal type, type II - radicular type, type III - venooclusive type. | ||
==Classification== | ==Classification== | ||
*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> | *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 | ||
**Anatomical location of occlusion | **[[Anatomical]] location of [[occlusion]] | ||
*An obstruction below 300µm in diameter is not considered as BCS by some authors. | *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 | *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 obstruction|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 obstruction|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: | *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|clinical manifestations]] within weeks with intractable [[ascites]] and [[Necrosis|hepatic necrosis]]. | ||
**Subacute: Insidious onset symptoms develop over 3 months.Clinical manifestations of | **[[Subacute]]: Insidious onset [[symptoms]] develop over 3 months. [[Clinical|Clinical manifestations]] of [[ascites]] and [[Necrosis|hepatic necrosis]] may be minimal as the [[portal]] and [[Collaterals|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 failure|acute liver injury]] with [[elevated transaminases]], [[jaundice]], [[hepatic encephalopathy]], and an elevated [[Prothrombin time (PT)|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. | *[[Collaterals|Venous collaterals]] are not developed in [[patients]] with [[acute liver failure]] or [[Acute liver failure|acute liver]] disease whereas [[Collaterals|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: | *Budd-Chiari syndrome may be classified according to the [[Anatomical|anatomical location]] of [[obstruction]] into 3 subtypes: | ||
**Type I - truncal type: inferior vena cava occlusion with or without the involvement of hepatic veins. | **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 II - radicular type: major [[Hepatic vein obstruction|hepatic veins occlusion]]. | ||
**Type III - venooclusive type: small centrilobular veins occlusion. | **Type III - venooclusive type: small centrilobular [[Veins|veins occlusion]]. | ||
==References== | ==References== | ||
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Overview
Budd-Chiari syndrome 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 two subtypes: primary and secondary. Budd-Chiari syndrome may be classified according to disease duration and severity into four subtypes: acute, subacute, chronic, fulminant liver failure. Budd-Chiari syndrome may be classified according to the anatomical location of obstruction into 3 subtypes: type I - truncal type, type II - radicular type, type III - venooclusive type.
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.