VASCULAR DISEASE OF THE LIVER



Portal vein thrombosis, hepatic vein thrombosis (Budd-Chiari syndrome), and veno-occlusive dis­ease are uncommon disorders of hepatic vascu­lature that usually present with portal hyperten­sion with or without associated liver dysfunction.

Portal vein thrombosis may develop after ab­dominal trauma, umbilical vein infection, sepsis, or pancreatitis or in association with cirrhosis or hypercoagulable states; in most cases, however, and particularly in children, the cause is un­known. The disease presents with the manifes­tations of portal hypertension (see Chapter 46); however, liver histology is usually normal. The diagnosis is established by angiography. Surgical management is difficult owing to the absence of suitable patent vessels for portal-systemic shunt­ing.

The BuddChiari syndrome is associated with abdominal trauma, use of oral contraceptives, po­lycythemia vera, paroxysmal nocturnal hemoglo­binuria, other hypercoagulable states, and con­genital webs of the vena cava. Illness may be acute or chronic with abdominal pain, hepatomegaly, ascites, and portal hypertension as prominent fea­tures. The diagnosis is usually suspected when centrilobular necrosis is seen on liver biopsy and is established angiographically by inability to catheterize the hepatic veins. Although elevation of serum bilirubin and transaminases is often mild, liver function is often poor and mortality rates of 40 to 90 per cent are reported. Anticoa­gulants have not proven useful; however, recent studies have suggested that the side-to-side por­tacaval shunts, performed to relieve hepatic congestion, may improve survival.

Veno-occlusive disease or nonthrombotic oc­clusion of hepatic venules is a smallvessel var­iant of the Budd-Chiari syndrome. Veno-occlu­sive disease develops in man and animals exposed to native medicinal teas containing pyr-rolozidine alkaloids from Senecio and Crotalia genera of plants. Cases have also been reported in association with the use of certain chemothera-peutic agents and with bone marrow transplan­tation. The occluded venules can be seen on liver biopsy, and a distinctive abnormal vascular pat­tern may be seen when contrast is injected into the hepatic vein. Patients with alcoholic liver dis­ease also frequently exhibit some degree of he­patic venule sclerosis or occlusion. No specific treatment is available. Some patients appear to re­cover spontaneously, whereas others require ther­apy for the complications of portal hypertension.





[1] 2 3 4 5 6 » ... Son Sayfa »