Budd-Chiari Syndrome

Published on 27/02/2015 by admin

Filed under Anesthesiology

Last modified 27/02/2015

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15. Budd-Chiari Syndrome

Definition

Budd-Chiari syndrome is an uncommon condition of obstruction of intrahepatic veins. It is induced by thrombotic or nonthrombotic obstruction of hepatic venous outflow, leading to congestive hepatopathy. The hepatopathy results from large and small vein obstruction of venous outflow, producing hepatocellular injury from microvascular ischemia. It eventually results in portal hypertension and then liver insufficiency and failure.

Incidence

Exact incidence of Budd-Chiari syndrome is not known; it is classified as a rare entity both in the United States and internationally.

Etiology

Budd-Chiari syndrome frequently occurs in patients who already have some manner of thrombotic diathesis, including myeloproliferative disorders, pregnancy, tumors, chronic inflammatory diseases, clotting disorders, and infections.
Causes of Budd-Chiari Syndrome

Chronic Infections

• Amoebic abscess
• Aspergillosis
• Hydatid cysts
• Syphilis
• Tuberculosis

Chronic Inflammatory Diseases

• Behçet’s disease (see p. 49)
• Inflammatory bowel disease
• Sarcoidosis
• Sjögren syndrome (see p. 316)
• Systemic lupus erythematosus (see p. 335)

Hematologic Disorders

• Antiphospholipid antibody syndrome
• Essential thrombocytosis
• Paroxysmal nocturnal hemoglobinuria
• Polycythemia rubra vera
• Unspecified myeloproliferative disorder

Inherited Thrombotic Diathesis

• Antithrombin III deficiency
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