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.[1][2]
  • 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

References

  1. Martens P, Nevens F (2015). "Budd-Chiari syndrome". United European Gastroenterol J. 3 (6): 489–500. doi:10.1177/2050640615582293. PMC 4669515. PMID 26668741.
  2. Shin N, Kim YH, Xu H, Shi HB, Zhang QQ, Colon Pons JP, Kim D, Xu Y, Wu FY, Han S, Lee BB, Li LS (2016). "Redefining Budd-Chiari syndrome: A systematic review". World J Hepatol. 8 (16): 691–702. doi:10.4254/wjh.v8.i16.691. PMC 4909431. PMID 27326316.

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