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: Mazia Fatima, MBBS [2]

Overview

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 such as hepatic encephalopathy, variceal hemorrhage, hepatorenal syndrome, portal hypertension, complications secondary to hepatic decompensation, which may eventually lead to intractable ascites with emaciation, gastrointestinal bleeding, and liver failure. 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. If left untreated, patients with Budd-Chiari syndrome have a high mortality rate. About 90% die within 3 years as the disease may progress 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

Complications

Prognosis

Factors associated with a good prognosis

Good prognostic factors include:

Factors associated with a poor prognosis

Poor prognostic factors include:

References

  1. 1.0 1.1 Grus T, Lambert L, Grusová G, Banerjee R, Burgetová A (2017). "Budd-Chiari Syndrome". Prague Med Rep. 118 (2–3): 69–80. doi:10.14712/23362936.2017.6. PMID 28922103.
  2. Lin M, Zhang F, Wang Y, Zhang B, Zhang W, Zou X, Zhang M, Zhuge Y (2017). "Liver cirrhosis caused by chronic Budd-Chiari syndrome". Medicine (Baltimore). 96 (34): e7425. doi:10.1097/MD.0000000000007425. PMC 5571988. PMID 28834866.
  3. Khuroo MS, Al-Suhabani H, Al-Sebayel M, Al Ashgar H, Dahab S, Khan MQ, Khalaf HA (2005). "Budd-Chiari syndrome: long-term effect on outcome with transjugular intrahepatic portosystemic shunt". J. Gastroenterol. Hepatol. 20 (10): 1494–502. doi:10.1111/j.1440-1746.2005.03878.x. PMID 16174064.
  4. Qi X, Ren W, Wang Y, Guo X, Fan D (2015). "Survival and prognostic indicators of Budd-Chiari syndrome: a systematic review of 79 studies". Expert Rev Gastroenterol Hepatol. 9 (6): 865–75. doi:10.1586/17474124.2015.1024224. PMID 25754880.
  5. Montano-Loza AJ, Tandon P, Kneteman N, Bailey R, Bain VG (2009). "Rotterdam score predicts early mortality in Budd-Chiari syndrome, and surgical shunting prolongs transplant-free survival". Aliment. Pharmacol. Ther. 30 (10): 1060–9. doi:10.1111/j.1365-2036.2009.04134.x. PMID 19723029.

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