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Etiology of the Budd-Chiari syndrome

Michelle Lai, MD, MPH
Section Editor
Sanjiv Chopra, MD, MACP
Deputy Editor
Anne C Travis, MD, MSc, FACG, AGAF


The Budd-Chiari syndrome can be defined as any pathophysiologic process that results in an interruption or diminution of the normal flow of blood out of the liver [1,2]. However, as commonly used, the Budd-Chiari syndrome implies thrombosis of the hepatic veins and/or the intrahepatic or suprahepatic inferior vena cava. An alternative nomenclature that recognizes an obliterative process involving principally the inferior vena cava ("obliterative hepatocavopathy") has also been proposed [3]. The term is usually distinguished from two other conditions that interfere with hepatic venous flow: sinusoidal obstruction syndrome (hepatic veno-occlusive disease) and right heart failure.

Disorders that involve the microscopic centrilobular veins and small sublobular veins within the liver are usually due to nonthrombotic fibrous occlusion and obliteration of these small veins. The distinct anatomic location, pathology, and conditions (eg, ingestion of toxic pyrrolizidine alkaloids or exposure to high doses of radiation or chemotherapeutic agents) with which sinusoidal obstruction syndrome is associated allow it to be placed in its own category [4,5]. (See "Diagnosis of hepatic sinusoidal obstruction syndrome (veno-occlusive disease) following hematopoietic cell transplantation".)

Although disorders of the heart may present clinically as the Budd-Chiari syndrome (eg, constrictive pericarditis, chronic right-sided heart failure with severe tricuspid insufficiency), the pathogenesis and treatment of these disorders are quite different from those usually considered typical of the Budd-Chiari syndrome. (See "Congestive hepatopathy".)

Thus, the Budd-Chiari syndrome is due to occlusion or partial occlusion of one, two, or all three of the major hepatic veins (right, middle, and left) and/or occlusion or partial occlusion of the inferior vein cava (figure 1) [6]. In addition, several small veins that enter the inferior vena cava directly from the posterior surface of the caudate lobe and occasionally from the right and left hepatic lobes may or may not be involved by the occlusive process.

This topic review will discuss the major causes of the Budd-Chiari syndrome. The symptoms, diagnosis, and treatment of this disorder are discussed separately. (See "Budd-Chiari syndrome: Epidemiology, clinical manifestations, and diagnosis".)


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Literature review current through: Sep 2016. | This topic last updated: Jan 30, 2015.
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