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

Prognosis

• The prognosis of Budd-Chiari syndrome is good with treatment.
• Without treatment, Budd-Chiari syndrome will result in death as a result of progressive liver failure within 3 years from diagnosis.
• Budd-Chiari syndrome is associated with a 10-year survival rate of 55% with treatment.Among patient with liver transplantation, the 5-year survival rate is 70%.
• Calculation of prognostic index for Budd-Chiari can be done using the following formula:
• Prognostic index = (ascites score x 0.75) + (Pugh score x 0.28) + (age x 0.037) + (creatinine level x 0.0036).
• Prognostic index score less than 5.4 is associated with a good prognosis.
• Good prognostic factors include:
  • Younger age at diagnosis
  • Low Child-Pugh score
  • Absence of ascites or easily controlled ascites
  • Low serum creatinine level
• Poor prognostic factors include:
  • Older age at diagnosis
  • Chronic disease
  • Severe liver failure
  • Refractory ascites.