Budd-Chiari syndrome natural history, complications and prognosis

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

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Overview

If left untreated, patients with Budd-Chiari syndrome have a high mortality rate.About 90% die within 3 years as the disease may progresss to develop intractable ascites with emaciation, gastrointestinal bleeding, and liver failure.Prognosis is generally good with treatment, and the 5 year survival rate of patients with treatment of Budd-Chiari syndrome is approximately 74 %.Poor prognostic factors include older age at diagnosis, chronic disease, severe liver failure and associated refractory ascites.

Natural History

• The symptoms of Budd-Chiari syndrome usually develop in the third or fourth decade of life and start with symptoms such as hepatomegaly, ascites, and abdominal pain.
• Without treatment, the patient will develop complications like hepatic encephalopathy, variceal hemorrhage, hepatorenal syndrome, portal hypertension, Complications secondary to hepatic decompensation, which will/ may eventually lead to intractable ascites with emaciation, gastrointestinal bleeding, and liver failure.

Complications

• Complications that can develop as a result of Budd-Chiari syndrome are:
  • Portal Hypertension
  • Hepatic encephalopathy
  • Variceal hemorrhage
  • Hepatorenal syndrome
  • Hepatic decompensation
  • Bacterial peritonitis especially following paracentesis