Budd-Chiari Syndrome

Definition

Budd-Chiari syndrome is a rare problem that results from blood clotting in the veins flowing out of the liver (hepatic veins). The high pressure of blood in these veins leads to an enlarged liver, and to an accumulation of fluid in the abdomen, called ascites.

Description

The liver, the largest internal organ in the human body, is responsible for many vital physiologic processes. Blood flow through the liver nourishes the liver, carries in substances that the liver will process, and carries away substances that the liver has produced. When blood cannot flow out freely from the liver, blood pressure rises in the veins of the liver, leading to blood clots within the liver. Also, some of the blood plasma can leak through the walls of the veins and accumulate within the abdomen (ascites).

Causes and symptoms

The major symptoms include pain in the upper right-hand portion of the abdomen and a build-up of fluid in the abdomen. In the United States, blood disorders are the most common causes. Among these disorders are polycythemia rubra vera (an increase in the number of red blood cells), and sickle cell disease. In parts of the world where liver cancer is common, a form of liver cancer is the most frequent cause.

Other causes sometimes include:

• certain infections
• use of oral contraceptives
• body changes in pregnancy and the postpartum period
• phlebitis (inflammation of a vein)
• injury to the abdomen
• membranous webs (especially in Asia)

Diagnosis

Diagnosis of Budd-Chiari syndrome can be made by an internist (a specialist in diseases of the internal organs), a gastroenterologist (a specialist in the diseases of the digestive system), or a general surgeon. On physical examination, the doctor will note that the liver is larger than normal. Often an ultrasound scan of the liver will show abnormalities in the size of the liver, an abnormal pattern of the veins in the liver, and other abnormalities. A CT scan will often show similar abnormalities.

Once these abnormalities are confirmed, the key test is called hepatic vein catheterization. In this test, a narrow tube is snaked through the body until it reaches the hepatic veins. An instrument at the tip of the catheter can measure the pressure within each segment of the hepatic vein.

In some cases, a tiny amount of radioactive material is injected into a patient, and then an abnormal pattern of radioactivity in the liver can be revealed. In other cases, a liver biopsy enables a physician to examine cells from the liver itself. Cells damaged by Budd-Chiari syndrome have a characteristic appearance easily identifiable to a physician.

Surgery

Most patients with Budd-Chiari syndrome must have surgery. A surgeon will re-route blood flow around the clotted hepatic vein into a large vein called the vena cava. The exact technique will depend on the specific location of the clots and other factors. In certain patients, other surgical techniques may be used. For patients who otherwise would have less than six months to live, liver transplantation is sometimes performed.

In a few patients, a "balloon catheter" can open the blocked blood vessels, without the need for major surgery.

Drugs

Sometimes, anti-clotting drugs such as urokinase can be used for patients with a sudden onset of clotting in the veins of the liver. These drugs do not seem to work when the clots have become established.

Prognosis

If surgery is done before permanent liver damage sets in, long-term survival is possible. In these cases, damaged liver cells can actually recover. If patients are already very sick with liver disease, the surgery may not be as helpful.

Prevention

The best approach to prevention is to carefully control the blood disorders that can lead to Budd-Chiari syndrome.

BOOKS

Gadacz, Thomas R., and John L. Cameron. "Budd-Chiari Syndrome and Surgery of the Hepatic Vasculature." In Shack-elford's Surgery of the Alimentary Tract. 3rd ed. Vol. 3. Ed. J.G. Turcotte. Philadelphia: W. B. Saunders Co., 1991.

Richard H. Lampert

KEY TERMS

Ascites—Accumulation of fluid in the abdomen.

Biopsy—Surgical removal of a tiny bit of tissue for examination under the microscope.

Catheter—A tubular surgical instrument.

Phlebitis—Inflammation of a vein.

Polycythemia rubra vera—An excess number of red blood cells in the blood.

Sickle cell disease—An inherited disease in which red blood cells take an unusual shape, leading to circulation problems.