Budd-Chiari Syndrome Health Article

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.