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Budd-Chiari Syndrome

Budd-Chiari Syndrome is a topic covered in the 5-Minute Clinical Consult.

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Basics

Description

A rare disorder caused by obstruction of the hepatic venous outflow tract resulting in right upper quadrant (RUQ) abdominal pain, portal hypertension (HTN), hepatomegaly, and ascites

Epidemiology

  • ~80% of all BCS cases occur in women.
  • Average age of BCS patients is 30 years.
  • ~85% of BCS patients have at least one prothrombotic risk factor; ~25% have two or more.
  • ~40% of BCS patients also have a primary myeloproliferative disorder.

Incidence
~1/100,000 worldwide per year (1)

Prevalence
~1.4/100,000 worldwide per year (1)

Etiology and Pathophysiology

  • Reduced or obstructed hepatic venous outflow causes increased sinusoidal pressure, resulting in hepatic congestion, RUQ abdominal pain, and ascites.
  • Outflow obstruction can occur anywhere from small hepatic veins to the atriocaval junction.
  • Often classified as (i) primary: obstruction arising from within the venous lumen or (ii) secondary: obstruction from outside the venous lumen
  • Thrombosis of hepatic vein due to inherited thrombophilia or hypercoagulable states
    • Myeloproliferative disease
    • Polycythemia vera
    • Pregnancy or postpartum state
    • Use of oral contraceptives
    • Paroxysmal nocturnal hemoglobinuria
    • Hepatocellular carcinoma (HCC)
    • Antiphospholipid syndrome; prothrombin mutation
    • Factor V Leiden mutation
    • Hyperhomocysteinemia
    • Protein C and S deficiencies
    • Antithrombin III deficiency
    • Inflammatory bowel disease
    • Behçet syndrome
  • Vena caval webs: more common in Asians
  • Idiopathic membranous stenosis of inferior vena cava (IVC)
  • Compression of the hepatic veins:
    • HCC
    • Abscess or cysts; sepsis
    • Status postomphalocele or gastroschisis repair
  • Bush teas (pyrrolizidine alkaloids)
  • Blunt abdominal trauma

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Citation

Stephens, Mark B., et al., editors. "Budd-Chiari Syndrome." 5-Minute Clinical Consult, 27th ed., Wolters Kluwer, 2019. Medicine Central, im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816185/all/Budd_Chiari_Syndrome.
Budd-Chiari Syndrome. In: Stephens MB, Golding J, Baldor RA, et al, eds. 5-Minute Clinical Consult. 27th ed. Wolters Kluwer; 2019. https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816185/all/Budd_Chiari_Syndrome. Accessed April 19, 2019.
Budd-Chiari Syndrome. (2019). In Stephens, M. B., Golding, J., Baldor, R. A., & Domino, F. J. (Eds.), 5-Minute Clinical Consult. Available from https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816185/all/Budd_Chiari_Syndrome
Budd-Chiari Syndrome [Internet]. In: Stephens MB, Golding J, Baldor RA, Domino FJ, editors. 5-Minute Clinical Consult. Wolters Kluwer; 2019. [cited 2019 April 19]. Available from: https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816185/all/Budd_Chiari_Syndrome.
* Article titles in AMA citation format should be in sentence-case
TY - ELEC T1 - Budd-Chiari Syndrome ID - 816185 ED - Stephens,Mark B, ED - Golding,Jeremy, ED - Baldor,Robert A, ED - Domino,Frank J, BT - 5-Minute Clinical Consult, Updating UR - https://im.unboundmedicine.com/medicine/view/5-Minute-Clinical-Consult/816185/all/Budd_Chiari_Syndrome PB - Wolters Kluwer ET - 27 DB - Medicine Central DP - Unbound Medicine ER -