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
Hepatic vein obstruction
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).
vein obstruction prevents blood from flowing out of the liver
and back to the heart. This blockage can cause liver damage.
Obstruction of this vein can be caused by masses pressing on the
vessel (tumor) or by thrombus (clot) formation within the
Most often, it is caused by conditions that increase the body's
propensity to form blood clots. These include:
Any of a number of hereditary or acquired
Myeloproliferative disorders abnormal
proliferation of cells from the bone marrow
Chronic inflammatory or autoimmune diseases
Oral contraceptives and pregnancy
Right-sided abdominal pain and a large liver
Jaundice (yellowing of the skin)
Ascites (swelling of the abdomen due to the
development of fluid in the abdomen)
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.
The best approach to prevention is to carefully control the
blood disorders that can lead to Budd-Chiari syndrome.
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