Budd Chiari Syndrome

Budd Chiari Syndrome

Budd-Chiari syndrome is a medical condition brought about by obstruction of the blood flow out of the liver, most commonly due to a blood clot.^[1] It was first described it in 1845 by Budd while  Chiari added the first pathologic explanation of a liver with "obliterating endophlebitis of the hepatic veins" in 1899.  Budd-Chiari syndrome is characterized by hepatomegaly, ascites and abdominal pain.^[2]

Epidemiology

One out of one million people develops Budd-Chiari syndrome. The disease is more common in females.^[3] Age at presentation of Budd-Chiari Syndrome  is usually the third or fourth decade of life, although children or elderly persons may have the disease.^[2]

Causes

The usual cause is a disorder that makes blood more likely to clot such as polycythemia, sickle cell disease, inflammatory bowel disease, connective tissue disorders and injury. Budd-Chiari syndrome may occur suddenly and severely, for instance during pregnancy since blood coagulates more readily. Other diseases that develop near the hepatic veins may cause Budd-Chiari syndrome like parasitic infections and liver or kidney tumors. In certain regions like Asia and South Africa, a membrane (web) that blocks the inferior vena cava often causes the condition.^[4]

Symptoms

Three main symptoms are noted: abdominal pain, ascites and hepatomegaly. In ascites, fluid collects in the abdominal cavity causing distention . Hepatomegaly or liver enlargement results from blood flowing into the liver but cannot make its way out. The affected individual may develop jaundice and kidney problems.^[1]

Diagnosis

As with any other diseases, medical history and physical examination are essential. Ascitic fluid can be examined to help confirm the diagnosis. Liver function can be evaluated using ultrasound and magnetic resonance imaging (MRI) may help.^[1] Liver biopsy may be done to study the cells and to determine if there is cirrhosis.^[4]

Treatment

Budd-Chiari syndrome can seriously damage the liver if not treated. Drugs can be administered to dissolve blood clots and reduce the formation of new ones. To help control ascites, a low-salt diet is often recommended. Certain surgical methods may be done to alleviate blood congestion in the liver. In severe cases,  a liver transplant is indicated.^[1]

References:

1. http://rarediseases.about.com/od/rarediseasesb/a/021905.htm
2. http://emedicine.medscape.com/article/184430-overview
3. http://www.buddchiarisyndrome.info/
4. http://www.merckmanuals.com/home/liver_and_gallbladder_disorders/blood_vessel_disorders_of_the_liver/budd-chiari_syndrome.html

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