Budd-Chiari Syndrome

Budd-Chiari syndrome is a condition of obstruction of the hepatic vein, which is the major vein that leaves the liver. This disorder usually is a result of an underlying condition that predisposes to blood clotting. It is a relatively common disease among patients with a predisposition to thrombosis, although this syndrome can also be the first symptom of such a predisposition.

Causes Of Budd-Chiari Syndrome

Most cases of Budd-Chiari syndrome are caused by a condition of thrombosis of the hepatic vein. Oral contraceptives, deficiencies in proteins C and S are considered risk factors. Other risk factors for thrombosis include genetic anomalies like the Factor V Leiden mutation.

The second cause of Budd-Chiari syndrome is a compression of the hepatic vein by another structure. Factors that may cause compression of the hepatic vein and thus lead to Budd-Chiari syndrome include liver tumors, pregnancy and trauma.

Other possible cause of Budd-Chiari syndrome is polycythemia vera, a relatively rare myeloproliferative (excessive production) disease of the red blood cells.

Symptoms Of Budd-Chiari Syndrome

The most common symptom in Budd-Chiari syndrome is ascites, or fluid accumulation in the abdomen. Patients also show rapidly progressive symptoms, including severe upper abdominal pain, hepatomegaly (abnormal enlargement of all or part of the liver), jaundice (yellow skin), ascites and eventual encephalopathy. In its fulminant form, Budd-Chiari syndrome presents early with ascites and encephalopathy. Severe hepatic necrosis (death of the tissues) and lactic acidosis may be also present, as well as caudate lobe hypertrophy.

Most patients have a non-fulminant form of Budd-Chiari syndrome that in some cases may be painless. Eventually, patients may progress to cirrhosis and liver failure.

Treatment Of Budd-Chiari Syndrome

A small number of patients with Budd-Chiari syndrome have responded well to certain medications. Sodium restriction and diuretics have been useful in controlling ascites.

Most patients, however, will need liver surgery. The procedure for moderate cases of Budd-Chiari syndrome involves finding a way to eliminate the occlusion that prevents blood from flowing. Depending on each specific case, this can be achieved with surgical shunts, angioplasty or other surgical techniques.

Patients who show symptoms of rapidly deteriorating liver function will need liver transplantation. Usually, this solves the problem, but there have been very rare cases in which Budd-Chiari syndrome returns after transplant.

Prevention Of Budd-Chiari Syndrome

The most effective way to prevent Budd-Chiari syndrome is to early detect and treat any possible cause. Early detection and adequate treatment of thrombosis, benign or malign cysts and blood coagulation anomalies will prevent the appearance of Budd-Chiari syndrome.

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