Budd-Chiari Syndrome Clinical Presentation

Updated: Oct 10, 2018
  • Author: Praveen K Roy, MD, AGAF; Chief Editor: BS Anand, MD  more...
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Presentation

History

The classic triad of abdominal pain, ascites, and hepatomegaly is observed in the vast majority of patients with Budd-Chiari syndrome, [18] but it is nonspecific. A high index of suspicion is needed to make the diagnosis.

If the liver has had time to develop collaterals and decompress, patients can be asymptomatic (≤20% [18] ) or present with few symptoms. As the syndrome progresses, however, it can lead to liver failure and portal hypertension with corresponding symptoms (eg, encephalopathy, hematemesis). The American Association for the Study of Liver Diseases has released guidelines for the management of acute liver failure. [19]

The clinical variants of Budd-Chiari syndrome have been described as follows [5, 6, 7] :

  • Acute and subacute forms: Characterized by rapid development of abdominal pain, ascites, hepatomegaly, jaundice, and renal failure.

  • Chronic form: Most common presentation; patients present with progressive ascites; jaundice is absent; approximately 50% of patients also have renal impairment

  • Fulminant form: Uncommon presentation; fulminant or subfulminant hepatic failure is present, along with ascites, tender hepatomegaly, jaundice, and renal failure.

Patients with acute onset of obstruction typically present with acute right upper quadrant pain. Abdominal distention can also be a significant symptom, because of ascites. Jaundice is rarely observed.

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Physical Examination

Physical examination may reveal the following findings:

  • Jaundice

  • Ascites

  • Hepatomegaly

  • Splenomegaly

  • Ankle edema

  • Stasis ulcerations

  • Prominence of collateral veins

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