Budd-Chiari syndrome is a rare liver disorder, affecting one in a million adults. The syndrome is characterized by narrowing and obstruction of the veins of the liver (hepatic veins).
Symptoms associated with Budd Chiari syndrome include pain in the upper right quadrant of the abdomen, or an abnormally large liver.
Often, the patient presents with accumulation of fluid in the space between the two layers of the membrane that lines the stomach (ascites). Additional symptoms may include: nausea, yellowing of white part of the eye, vomiting, and/or an abnormally large spleen.
Who is typically affected
Budd Chiari syndrome affects males and females equally. Most cases tend to affect individuals between the ages of twenty and forty. Often patients who suffer from Budd Chiari syndrome also suffer from Polycythemia vera, another rare, chronic disorder characterized by overproduction of red blood cells and elements of the bone marrow involved in the formation of red blood cells.
Sometimes Budd-Chiari syndrome begins suddenly and rather severely, typically during pregnancy. During pregnancy, the blood normally clots more readily. In some women, a blood clotting disorder may first become apparent during pregnancy.
Disorders that develop near the hepatic veins, such as parasitic infections and liver tumors or kidney tumors that press on or invade the veins are also causes of Budd Chiari syndrome. In Asia and South Africa, the cause is commonly a membrane (web) that blocks the inferior vena cava. These webs are often present at birth.
Approximately 70% of individuals who suffer from Budd Chiari syndrome will not know the exact cause.
Diagnosis and Treatment
Typically, the syndrome is diagnosed by ultrasound or an MRI. To verify the diagnosis, the patient would need an angiography to confirm the diagnosis.