Budd-Chiari syndrome is hepatic veins occlusion. The consequences are portal hypertension because of blood congestion in liver and portal venous system and finally liver cirrhosis and failure. This syndrome is quite infrequent.
Note: hepatic veins bring the blood from liver to inferior vena cava (see picture).
Signs and Symptoms
The acute signs of the syndrome may include: severe abdominal pain, jaundice, hepatomegaly, ascites and other symptoms associated with liver problems. Most patients have a slower onset and it was discovered accidentally they may be symptom-free and or may already have progressive cirrhosis and liver failure.
Pathophysiology and etiology
The most common cause is hepatic veins thrombosis (75%) in patients with primary predisposition (coagulopathies like Protein C or Protein S deficiency, Leiden mutation...) or other factors like:
- polycythemia vera;
- use of oral contraceptives;
- trauma (cause of a new abnormality of hepatic vein);
Other causes of hepatic veins occlusion (25%) can be:
- hepatocellular carcinoma;
- metastasis in liver;
- infections in liver (TB).
There are a few diagnostic methods for hepatic veins examination:
- USG of liver with Doppler;
- CT, CT angiography;
- lab: elevated liver markers (AST, ALT), possible coagulopathy;
- hematology: RBC abnormalities, thrombocytopathy...
But there is usually portal hypertension symptoms domination (ascites, esophageal varices). That is the reason, why Budd-Chari syndrome should be always excluded in patients with portal hypertension and liver cirrhosis!
- portal hypertension (with all consequences);
- nonalcoholic liver cirrhosis and failure;
- pulmonary embolism.
- therapy of the cause:
- thrombophilia (heparin, warfarin);
- therapy of consequences:
- therapy of portal hypertension;
- liver transplantation.
- KASPER, Dennis L – FAUCI, Anthony S – LONGO, Dan L, et al. Harrison's principles of Internal Medicine. 16th edition. New York : McGraw-Hill Companies, Inc, 2005. 2607 pp. pp. 1892-1896. ISBN 0-07-139140-1.
- Wikipedia.org. Budd–Chiari syndrome [online]. Wikipedia.org, ©2011. The last revision 2011-08-13, [cit. 2011-09-19]. <http://en.wikipedia.org/wiki/Budd-Chiari_syndrome>.