Budd-Chiari Syndrome

(Budd-Chiari syndrome)

Under a Budd-Chiari syndrome is defined as the closure of the venous effluent from the liver, the cause of which can be anywhere cava inferior between the hepatic veins of the small branches to V. and the right atrium. The clinical manifestations range from asymptomatic to fulminant liver failure. Diagnosis is based on the color Doppler-based ultrasound. The treatment consists of supportive medical procedures and methods for maintaining or restoring the venous flow, such as thrombolysis, pressure relief by shunts and long-term anticoagulation.

(See also Overview of vascular diseases of the liver.)

Under a Budd-Chiari syndrome is defined as the closure of the venous effluent from the liver, the cause of which can be anywhere cava inferior between the hepatic veins of the small branches to V. and the right atrium. The clinical manifestations range from asymptomatic to fulminant liver failure. Diagnosis is based on the color Doppler-based ultrasound. The treatment consists of supportive medical procedures and methods for maintaining or restoring the venous flow, such as thrombolysis, pressure relief by shunts and long-term anticoagulation. (See also Overview of vascular diseases of the liver.) Etiology In the Western world, the most common cause of a blood clot in the hepatic veins and the adjacent inferior vena cava. Blood clots usually result from one of the following: Thrombophilic states (for example, protein C or S deficiency, antiphospholipid syndrome, anti-thrombin III deficiency, Factor V Leiden, pregnancy, use of oral contraceptives.) Hematologic diseases (e.g. . B. myeloproliferative disorders such as polycythemia vera and paroxysmal nocturnal hemoglobinopathy) Inflammatory bowel disease connective tissue trauma infection (z. B. hydatid disease, amebiasis) tumor invasion of the hepatic vein (z. B. hepatocellular or renal cell carcinoma) Sometimes the Budd-Chiari syndrome begins during pregnancy and unmasked a previously asymptomatic thrombophilia. The causes of the closure often remain unknown. In Asia and South Africa, the underlying disorder is often a membranous closure (power) of the inferior vena cava above the liver, probably as an expression of recanalization a recent thrombosis in adults or growth disorder (z. B. Venenstenose) in children. This type of flow restriction is called obliterative Hepatocavopathie. A Budd-Chiari syndrome usually develops over weeks or months. If it develops over a period of time often develop cirrhosis and portal hypertension. Symptoms and signs Clinical manifestations range from asymptomatic to fulminant liver failure or cirrhosis. Symptoms vary depending on whether the closure occurs acutely or over a longer period. Acute occlusion (about 20% of cases) causes fatigue, pain in the right upper abdomen, nausea, vomiting, mild jaundice, painful pressure hepatomegaly and ascites. It is typically present during pregnancy. Fulminant hepatic failure with encephalopathy is rare. Aminotransferase levels are quite high. Chronic obstructions that develop over weeks to months, may have few or no symptoms until a manifest portal hypertension occurs, or manifest itself with fatigue, abdominal pain and hepatomegaly. Lower extremity edema and ascites may be the result of obstruction, even if no cirrhosis is present. Cirrhosis of the liver may develop, which can lead to variceal bleeding, massive ascites, splenomegaly, hepatopulmonalem syndrome or a combination of several. The complete closure of the inferior vena cava caused edema in the abdominal wall and the legs and visible, tortuous superficial abdominal veins of the pelvis to the rib cage. Diagnosis Clinical examination and liver function tests Vascular imaging DAs Budd-Chiari syndrome is suspected in patients with hepatomegaly, ascites, liver failure or cirrhosis, when there is no obvious other cause (eg., Alcohol abuse, hepatitis) or the cause is unknown Liver function tests usually increases, but there is no clear and specific pattern. The presence of risk factors for thrombosis, increase the taking into this diagnosis. The first imaging method is the Doppler ultrasound showing the direction of blood flow and the location of the closure. MRI and CT angiography may help verifiable if the ultrasound does not lead to diagnosis. Conventional angiography (venography with pressure measurements or arteriography) are necessary if therapeutic or surgical intervention is planned. A liver biopsy is sometimes performed to determine whether cirrhosis has developed. Prognosis Without treatment dies the majority of patients with a complete venous occlusion within three years of liver failure. In patients with incomplete obstruction of the natural course is variable. Therapy Supportive treatment to restore and maintain an adequate venous drainage The treatment depends on the time course (acute versus chronic) and severity (fulminant hepatic failure versus decompensated cirrhosis versus unchanged / asymptomatic) of the disease. The cornerstone of care are Supportive therapy for complications (eg. As ascites, hepatic failure, esophageal varices) decompression of congested liver (ie maintenance of venous outflow) preventing the spread of thrombosis Aggressive interventions (eg. As thrombolysis, stents) are applied, when the disease is acute (eg., within 4 weeks, and the absence of cirrhosis). Thrombolysis can resolve frischeThromben that allow recanalization and thus relieve the liver congestion. Radiological methods such as transjugular portosystemic shunt can play an important role. In the presence of membranes ( “webs”) in the vena cava or stenosis of the hepatic veins may restore a percutaneous transluminal balloon angioplasty with stent intraluminal blood flow from the liver. If the dilation of a narrowed liver drainage is not technically possible, transjugular intrahepatic portosystemic shunt (TIPS) and various surgical shunts may allow decompression of portal flow path. Portosystemic shunts should not be used typically when hepatic encephalopathy is present; Moreover, such shunts impair liver function. In addition, stent clots are relatively common, v. a. in patients with hematologic or thrombotic underlying disease. A long-term anticoagulation is often necessary for the prevention of Budd-Chiari recurrence. Liver transplantation is in the case of fulminant hepatic failure or of congestive cirrhosis, potentially life-saving measure. Points to the most common cause of Budd-Chiari syndrome (obstruction of the venous liver effluent) is a blood clot that blocks the hepatic veins, and the inferior vena cava. The diagnosis should with typical findings (z. B. hepatomegaly, ascites, hepatic failure, cirrhosis) should be considered in patients who are untreated or in those with abnormal liver and risk factors for thrombosis. The diagnosis is confirmed by Doppler ultrasound or indeterminate result with MRI or CT. The venous outflow is restored (for. Example, by thrombolysis, angioplasty, stents), and complications are treated.

Health Life Media Team

Leave a Reply