Budd-Chiari syndrome overview: Difference between revisions
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{{Budd-Chiari syndrome}} | {{Budd-Chiari syndrome}} | ||
{{CMG}}; {{AE}} {{ADI}} | {{CMG}}; {{AE}} {{ADI}} | ||
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== Overview == | == Overview == | ||
The Budd-Chiari syndrome is the clinical picture caused by [[occlusion]] of the [[hepatic vein]] or [[inferior vena cava]]. Its presents with the classical triad of [[abdominal pain]], [[ascites]] and [[hepatomegaly]]. Examples of occlusion include thrombosis of [[hepatic veins]] and membranous webs in the [[inferior vena cava]]. The syndrome can be [[fulminant]], acute, chronic, or asymptomatic. | |||
==Historical Perspective== | ==Historical Perspective== | ||
It is named for George Budd<ref>{{WhoNamedIt|synd|1335}}</ref><ref>G. Budd. On diseases of the liver. London, J. Churchill, 1845. Page 135.</ref> and Hans Chiari.<ref>H. Chiari. Erfahrungen über Infarktbildungen in der Leber des Menschen. Zeitschrift für Heilkunde, Prague, 1898, 19: 475-512.</ref> | It is named for George Budd<ref>{{WhoNamedIt|synd|1335}}</ref><ref>G. Budd. On diseases of the liver. London, J. Churchill, 1845. Page 135.</ref> and Hans Chiari.<ref>H. Chiari. Erfahrungen über Infarktbildungen in der Leber des Menschen. Zeitschrift für Heilkunde, Prague, 1898, 19: 475-512.</ref> | ||
==Classification== | |||
==Pathophysiology== | ==Pathophysiology== | ||
Any obstruction of the [[vein|venous]] vasculature of the liver is referred to as Budd-Chiari syndrome, from the [[venule]]s to the [[right atrium]]. This leads to increased portal vein and hepatic sinusoid pressures as the blood flow stagnates. The increased portal pressure causes: 1) increased filtration of vascular fluid with the formation of protein-rich ascites in the abdomen; and 2) collateral venous flow through alternative veins leading to gastric varices and hemorrhoids. Obstruction also causes hepatic necrosis and eventual centrilobular fibrosis due to ischemia. Renal failure may occur, perhaps due to the body sensing an "underfill" state and subsequent activation of the renin-angiotensin pathways and excess sodium retention. | Any obstruction of the [[vein|venous]] vasculature of the liver is referred to as Budd-Chiari syndrome, from the [[venule]]s to the [[right atrium]]. This leads to increased portal vein and hepatic sinusoid pressures as the blood flow stagnates. The increased portal pressure causes: 1) increased filtration of vascular fluid with the formation of protein-rich ascites in the abdomen; and 2) collateral venous flow through alternative veins leading to gastric varices and hemorrhoids. Obstruction also causes hepatic necrosis and eventual centrilobular fibrosis due to ischemia. Renal failure may occur, perhaps due to the body sensing an "underfill" state and subsequent activation of the renin-angiotensin pathways and excess sodium retention. | ||
==Causes== | |||
==Differentiating {{PAGENAME}} from Other Diseases== | |||
==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
It occurs in 1 out of 100,000 individuals and is more common in females. Some 10-20% also have obstruction of the portal vein. | It occurs in 1 out of 100,000 individuals and is more common in females. Some 10-20% also have obstruction of the portal vein. | ||
==Risk Factors== | |||
==Screening== | |||
==Natural History, Complications, and Prognosis== | |||
===Natural History=== | |||
===Complications=== | |||
===Prognosis=== | |||
==Diagnosis== | ==Diagnosis== | ||
===CT=== | ===CT=== | ||
[[Computed tomography]] (CT) or [[magnetic resonance imaging]] (MRI) is sometimes employed although these methods are generally not as sensitive. | [[Computed tomography]] (CT) or [[magnetic resonance imaging]] (MRI) is sometimes employed although these methods are generally not as sensitive. | ||
===Diagnostic Criteria=== | |||
===History and Symptoms=== | |||
===Physical Examination=== | |||
===Laboratory Findings=== | |||
===Imaging Findings=== | |||
===Other Diagnostic Studies=== | |||
==Treatment== | ==Treatment== | ||
===Medical Therapy=== | ===Medical Therapy=== | ||
A minority of patients can be treated medically with [[sodium]] restriction, [[diuretics]] to control ascites, anticoagulants such as [[heparin]] and [[warfarin]], and general symptomatic management. The majority of patients require further intervention. Milder forms of Budd-Chiari may be treated with surgical shunts to divert blood flow around the obstruction or the liver itself. Shunts must be placed early after diagnosis for best results. The [[transjugular intrahepatic portosystemic shunt]] (TIPS) is similar to a surgical shunt. It accomplishes the same goal but has a lower procedure-related mortality, which has led to a growth in its popularity. Patients with stenosis or vena caval obstruction may benefit from [[angioplasty]]. <ref>Fisher NC, McCafferty I, Dolapci M, Wali M, Buckels JA, Olliff SP, Elias E. Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. Gut. 1999 Apr;44(4):568-74.</ref> Limited studies on [[thrombolysis]] with direct infusion of [[urokinase]] and [[tissue plasminogen activator]] (tPA) into the obstructed vein have shown moderate success in treating Budd-Chiari syndrome; however, it is not routinely attempted. | A minority of patients can be treated medically with [[sodium]] restriction, [[diuretics]] to control ascites, anticoagulants such as [[heparin]] and [[warfarin]], and general symptomatic management. The majority of patients require further intervention. Milder forms of Budd-Chiari may be treated with surgical shunts to divert blood flow around the obstruction or the liver itself. Shunts must be placed early after diagnosis for best results. The [[transjugular intrahepatic portosystemic shunt]] (TIPS) is similar to a surgical shunt. It accomplishes the same goal but has a lower procedure-related mortality, which has led to a growth in its popularity. Patients with stenosis or vena caval obstruction may benefit from [[angioplasty]]. <ref>Fisher NC, McCafferty I, Dolapci M, Wali M, Buckels JA, Olliff SP, Elias E. Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. Gut. 1999 Apr;44(4):568-74.</ref> Limited studies on [[thrombolysis]] with direct infusion of [[urokinase]] and [[tissue plasminogen activator]] (tPA) into the obstructed vein have shown moderate success in treating Budd-Chiari syndrome; however, it is not routinely attempted. | ||
===Surgery=== | |||
===Prevention=== | |||
==References== | ==References== | ||
{{Reflist|2}} | {{Reflist|2}} | ||
[[Category:Hepatology]] | [[Category:Hepatology]] | ||
[[Category:Gastroenterology]] | [[Category:Gastroenterology]] | ||
{{WS}} | |||
{{WH}} | |||
Revision as of 17:49, 18 July 2016
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Govindavarjhulla, M.B.B.S. [2]
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Overview
The Budd-Chiari syndrome is the clinical picture caused by occlusion of the hepatic vein or inferior vena cava. Its presents with the classical triad of abdominal pain, ascites and hepatomegaly. Examples of occlusion include thrombosis of hepatic veins and membranous webs in the inferior vena cava. The syndrome can be fulminant, acute, chronic, or asymptomatic.
Historical Perspective
It is named for George Budd[1][2] and Hans Chiari.[3]
Classification
Pathophysiology
Any obstruction of the venous vasculature of the liver is referred to as Budd-Chiari syndrome, from the venules to the right atrium. This leads to increased portal vein and hepatic sinusoid pressures as the blood flow stagnates. The increased portal pressure causes: 1) increased filtration of vascular fluid with the formation of protein-rich ascites in the abdomen; and 2) collateral venous flow through alternative veins leading to gastric varices and hemorrhoids. Obstruction also causes hepatic necrosis and eventual centrilobular fibrosis due to ischemia. Renal failure may occur, perhaps due to the body sensing an "underfill" state and subsequent activation of the renin-angiotensin pathways and excess sodium retention.
Causes
Differentiating Budd-Chiari syndrome overview from Other Diseases
Epidemiology and Demographics
It occurs in 1 out of 100,000 individuals and is more common in females. Some 10-20% also have obstruction of the portal vein.
Risk Factors
Screening
Natural History, Complications, and Prognosis
Natural History
Complications
Prognosis
Diagnosis
CT
Computed tomography (CT) or magnetic resonance imaging (MRI) is sometimes employed although these methods are generally not as sensitive.
Diagnostic Criteria
History and Symptoms
Physical Examination
Laboratory Findings
Imaging Findings
Other Diagnostic Studies
Treatment
Medical Therapy
A minority of patients can be treated medically with sodium restriction, diuretics to control ascites, anticoagulants such as heparin and warfarin, and general symptomatic management. The majority of patients require further intervention. Milder forms of Budd-Chiari may be treated with surgical shunts to divert blood flow around the obstruction or the liver itself. Shunts must be placed early after diagnosis for best results. The transjugular intrahepatic portosystemic shunt (TIPS) is similar to a surgical shunt. It accomplishes the same goal but has a lower procedure-related mortality, which has led to a growth in its popularity. Patients with stenosis or vena caval obstruction may benefit from angioplasty. [4] Limited studies on thrombolysis with direct infusion of urokinase and tissue plasminogen activator (tPA) into the obstructed vein have shown moderate success in treating Budd-Chiari syndrome; however, it is not routinely attempted.
Surgery
Prevention
References
- ↑ Template:WhoNamedIt
- ↑ G. Budd. On diseases of the liver. London, J. Churchill, 1845. Page 135.
- ↑ H. Chiari. Erfahrungen über Infarktbildungen in der Leber des Menschen. Zeitschrift für Heilkunde, Prague, 1898, 19: 475-512.
- ↑ Fisher NC, McCafferty I, Dolapci M, Wali M, Buckels JA, Olliff SP, Elias E. Managing Budd-Chiari syndrome: a retrospective review of percutaneous hepatic vein angioplasty and surgical shunting. Gut. 1999 Apr;44(4):568-74.