Budd-Chiari syndrome risk factors

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

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Risk Factors

• Approximately 80 percent of patients with the Budd-Chiari syndrome have an underlying disorder.Many patients may have more than one risk factor. Same patient may have multiple causes that predispose to the development of Budd-Chiari Syndrome.
• Associated risk factors include:
• Hematologic disorders including:
  • Polycythemia rubra vera
  • Paroxysmal nocturnal hemoglobinuria
  • myeloproliferative disorder
  • Antiphospholipid antibody syndrome
  • Essential thrombocytosis
  • Inherited thrombotic diathesis

• Coagulopathies include the following:
  • Protein C deficiency
  • Protein S deficiency
  • Antithrombin III deficiency
  • Factor V Leiden deficiency
• Chronic infections like:
  • Hydatid cysts
  • Aspergillosis
  • Amebic abscess
  • Syphilis
  • Tuberculosis
• Chronic inflammatory diseases such as:
  • Behçet disease
  • Inflammatory bowel disease
  • Sarcoidosis
  • Systemic lupus erythematosus
  • Sjögren syndrome
  • Mixed connective-tissue disease
• Tumors such as
  • Hepatocellular carcinoma (HCC)
  • Renal cell carcinoma
  • Leiomyosarcoma
  • Adrenal carcinoma
  • Wilms tumor
  • Right atrial myxoma
• Congenital membranous obstructions that includes the following:
  • Type I: Thin membrane is present in the vena cava or the atrium
  • Type II: A part of the vena cava is absent
  • Type III: The inferior vena cava (IVC) cannot be filled, and collaterals have developed
• Miscellaneous risk factors of Budd-Chiari syndrome include the following:
  • Alpha1-antitrypsin deficiency
  • Dacarbazine
  • Urethane
  • Hypoplasia of the suprahepatic veins
  • Postsurgical obstruction
  • Posttraumatic obstruction
  • Total parenteral nutrition (TPN): Budd-Chiari syndrome can be a complication of TPN via an IVC catheter in a neonate