Budd-Chiari syndrome is a condition in which veins that drain the liver are narrowed or blocked, preventing blood from flowing out of the liver.
Blockage may be partial or complete, and may occur in small or large veins.
Only about 1 in a million people are affected.
There are many possible causes of Budd-Chiari syndrome, mainly related to underlying conditions that increase the tendency of the blood to clot. In more than 80% of cases the cause can be identified. Causes include:
Myeloproliferative disorders (conditions that cause abnormal production of blood cells in the bone marrow)
Not all Budd-Chiari syndrome patients experience symptoms, which usually develop gradually over weeks or months. Blockages occur suddenly in about 20% of cases. Symptoms include:
A patient presenting with abdominal pain, ascites, and liver enlargement will often be suspected of having Budd-Chiari syndrome. Blood tests will often be abnormal, indicating elevated liver enzymes and liver disease. Doctors may initially mistake Budd-Chiari syndrome for cirrhosis.
Physical examination may find that the liver is enlarged; further testing may include:
Findings may include liver cell death and severe lactic acidosis.
In some cases, treatment for Budd-Chiari syndrome is as simple as breaking up blood clots using anti-clotting medication, as well as diuretics and restricting sodium intake. Most patients, however, require surgery to unblock the veins inside the liver. A shunt may be inserted, to bypass a clotted vein. Angioplasty may be used to clear or widen a vein. For those with liver disease, a liver transplant is usually the best option.
Prior to any surgery, a venography is required. This involves X-rays of the veins, taken after a dye that is visible in X-rays is injected.
A low-salt diet, along with diuretics, helps to prevent fluid from accumulating in the abdomen.
After treatment, most patients continue taking anticoagulants indefinitely in order to prevent recurrence.
Prognosis depends on patient age, the severity of the blockage, the underlying cause of the blockage, time to diagnosis, and the treatment. Untreated, most patients die of liver failure within 3 years. If treated, two-thirds of patients survive for more than 10 years after diagnosis.
Budd-Chiari syndrome, if left untreated, will lead liver damage (cirrhosis) ranging from mild to severe, as well as possibly encephalopathy.
Vomiting blood is a medical emergency.
Pain in the upper right abdomen, along with ascites and liver enlargement, is one of the main signs of Budd-Chiari syndrome.
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