Budd-Chiari syndrome


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.


The acute syndrome presents with rapidly progressive: severe upper abdominal pain, jaundice, hepatomegaly (enlarged liver), ascites, elevated liver enzymes, and eventual encephalopathy. The fulminant syndrome presents early with encephalopathy and ascites. Severe hepatic necrosis and lactic acidosis may be present as well. Caudate lobe hypertrophy is often present. The majority of patients have a slower-onset form of Budd-Chiari syndrome. This can be painless. A system of venous collaterals may form around the occlusion which may be seen on imaging as a "spider's web." Patients may progress to cirrhosis and show the signs of liver failure


* The cause cannot be found in about half of the patients * Primary (75%): thrombosis of the hepatic vein * Secondary (25%): compression of the hepatic vein by an outside structure (e.g. a tumor) * Pregnancy and oral contraceptive use * Congenital venous webs * Occasionally inferior vena caval stenosis


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.


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.