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Budd-Chiari Syndrome

Reviewed 06/2018
 


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...

DIAGNOSIS

HISTORY

  • Presentation is typically insidious. Can be acute, fulminant, subacute, or chronic. Classic triad includes abdominal pain, ascites, and hepatomegaly.

  • Most patients present with RUQ abd...

TREATMENT

  • Treatment generally follows a stepwise therapeutic strategy; acuity dictates need for surgery (2).

  • Interventions are medical, endovascular, and surgical.

  • The overall goals of therapy are:

    • Relie...

ONGOING CARE

PROGNOSIS

  • Without intervention, 5-year survival is <10%.

  • With intervention, the 5-year survival rate is ~70%.

  • Unfavorable prognostic factors include:

    • Advanced age

    • Elevated creatinine serum ...

REFERENCES

1
Valla DC. Hepatic venous outflow tract obstruction etiopathogenesis: Asia versus the West. J Gastroenterol Hepatol.  2004;19:S204–S211.
2
Seijo S, Plessier A, Hoekstra J, et al; for Eur...

ADDITIONAL READING

  • Jayanthi V, Udayakumar N. Budd-Chiari syndrome. Changing epidemiology and clinical presentation. Minerva Gastroenterol Dietol.  2010;56(1):71–80.

  • Martens P, Nevens F. Budd-Chiar...

SEE ALSO

  • Ascites; Cholestasis of Pregnancy, Intrahepatic

  • Algorithms: AST Elevation; Hepatomegaly

CODES

ICD10

I82.0 Budd-Chiari syndrome 

ICD9

453.0 Budd-chiari syndrome 

SNOMED

82385007 Budd-Chiari syndrome (disorder) 

CLINICAL PEARLS

  • Consider BCS in young female patients with sudden onset of RUQ pain, hepatomegaly, and ascites.

  • BCS is commonly mistaken for right heart failure.

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