Budd-Chiari syndrome (hepatic veno-occlusive)

Budd-Chiari syndrome (hepatic veno-occlusive)

    * Introduction
    * Pathogenesis and causes
    * Signs and symptoms
    * Diagnosis
    * Treatment
Budd-Chiari syndrome or veno-occlusive liver disease Rokitansky syndrome is characterized by obstruction of hepatic veins and blood sequestration in the liver.
When blood enters the liver it is the systemic circulation through the hepatic veins and inferior cava veins and then, a large vessel that takes blood back to heart. In veno-occlusive disease of the blood circulation is blocked it partially. The immediate result is congestion of liver, blood entering the liver sequestration. The liver becomes painfully sensitive, increased volume and cause discomfort. Seghestrat congestion causes the fluid in the abdominal cavity, ascites, one of the most common symptoms of Budd-Chiari syndrome.
If obstruction is extreme and affects the rest of the vessels that bring blood to the heart of lower body half-inferior vena cava, peripheral edema will occur.
Another effect less visible but equally important is severe portal hypertension. It is characterized by increased pressure in the portal vein due to blockage in the path you have to drain blood out of the liver. Venel bypass blocked veins-other cause dilated veins. Blood returns to the heart can be seized by gastro-esophageal veins. They are fragile and can not cope with increased blood pressure and digestive break forcing variceal bleeding.
The disease occurs mostly in people with thrombotic diathesis, including myeloproliferative disorders: polycythemia vera, paroxysmal nocturnal hemoglobinuria in pregnancy, tumors, chronic inflammatory diseases, clotting disorders and infections.
Medical therapy can be instituted short-term symptomatic benefits. As the only therapy is associated with a mortality rate of 85%. This includes control of ascites, anticoagulant therapy, antiplatelet and angioplasty.
Surgical therapy in combination with the growing medical patient survival. It consists of surgical decompression or making a transjugular intrahepatic are portosistemic. Liver transplantation is an option when you installed decompensated cirrhosis.
The prognosis is bad for patients not treated. Deaths result from progressive liver failure in a time of 3 months to 3 years after diagnosis. The survival rate at 5 years after shunting portosistemica is 38-87%. After liver transplantation 5-year survival rate is 70%.