Budd-Chiari Syndrome
Budd-Chiari Syndrome Causes
Often, the patient is known to have a tendency towards thrombosis, although Budd-Chiari syndrome can also be the initial symptom of such a tendency. Some examples of genetic tendencies include Protein S deficiency, Protein C deficiency, and the Factor V Leiden mutation. An important non-genetic risk factor is the utilization of estrogen-containing (combined) forms of hormonal contraception. Other risk factors include the antiphospholipid syndrome, dacarbazine, aspergillosis, Behcet's disease, pregnancy, and trauma.
Budd-Chiari Syndrome Definition
In medicine (gastroenterology and hepatology), Budd-Chiari syndrome is the clinical picture resulted from occlusion of the hepatic vein or inferior vena cava. It presents with the classical triad of abdominal pain, ascites and hepatomegaly. Some examples of occlusion include thrombosis of hepatic veins and membranous webs in the inferior vena cava. The syndrome can be acute, fulminant, chronic, or asymptomatic. It takes place in 1 out of 100,000 individuals and is more common in females. Some 10-20% also have a sort of obstruction of the portal vein.
Budd-Chiari Syndrome Symptoms and Signs
The acute syndrome presents with rapidly progressive severe upper abdominal pain, hepatomegaly (enlarged liver), jaundice, ascites, elevated liver enzymes, and eventual encephalopathy. The fulminant syndrome presents early on with encephalopathy and ascites. Severe hepatic necrosis and lactic acidosis may be evident as well. Caudate lobe hypertrophy is often found. Most of the patients have a slower-onset form of Budd-Chiari syndrome which can be painless. A system of venous collaterals may form around the occlusion which may resemble a spider's web. Patients may develop to cirrhosis and show the signs of liver failure. An asymptomatic form may be wholly silent and discovered only incidentally and is generally not concerning.
Budd-Chiari Syndrome Treatment
A few of the patients can be treated medically with sodium restriction, diuretics to control ascites, anticoagulants such as heparin and warfarin, and general symptomatic management. Most of the patients require further intervention. Milder forms of Budd-Chiari may be treated with surgical shunts to help divert blood flow around the obstruction or the liver itself. Shunts must be placed early on after diagnosis for best results. The transjugular intrahepatic portosystemic shunt (TIPS) is same as a surgical shunt. It accomplishes the same goal but has a lower procedure-related mortality, which has resulted to a growth in its popularity. Patients with stenosis or vena caval obstruction can benefit from angioplasty. Limited studies on thrombolysis with direct infusion of urokinase and tissue plasminogen activator (tPA) into the obstructed vein have been proven to show moderate success in treating Budd-Chiari syndrome; however, it is not routinely attempted.