Chapter 19 Budd–Chiari syndrome and other vascular disorders
1 Hepatic vein occlusion, or Budd–Chiari syndrome, is an uncommon disorder characterized by hepatomegaly, ascites, and abdominal pain. The disorder most often occurs in patients with an underlying thrombotic diathesis including polycythemia vera, factor V Leiden mutation, protein C deficiency, paroxysmal nocturnal hemoglobinuria, tumors, and chronic inflammatory diseases.
2 The diagnosis is confirmed by visualization of thrombus or absent flow in hepatic veins on Doppler ultrasonography, three-phase computed tomography, or magnetic resonance imaging.
3 Budd–Chiari syndrome can be fatal without treatment. The approach to treatment should be stepwise, beginning with anticoagulation and followed by angioplasty or transjugular intrahepatic portosystemic shunt for portal decompression. Liver transplantation should be reserved for patients with advanced disease in whom other treatments fail. Improvements in treatment now offer 5-year survival rates of 85% to 90%.
4 Portal vein thrombosis (PVT) occurs in patients with an underlying thrombotic disorder or in patients with cirrhosis. Extension of hepatocellular carcinoma into the portal vein can also result in thrombosis. In the acute phase, anticoagulation is recommended. Band ligation of varices and beta blockers are used to prevent variceal bleeding in patients with chronic PVT.
5 Sinusoidal obstruction syndrome (veno-occlusive disease) of the liver is an occlusive disease of the small hepatic venules that mimics Budd–Chiari syndrome. It develops primarily in patients following allogeneic or autologous bone marrow transplantation, probably as a result of toxic injury to the endothelial cells caused by cytoreductive therapy. Treatment is largely supportive.
1. BCS is classified according to the following:
b. The site of obstruction
2. Most patients with BCS present within 3 months of the onset of symptoms. Most have subacute or chronic disease at the time of presentation, a finding suggesting that thrombosis of intrahepatic veins leads subsequently to occlusion of large collecting veins.
3. Membranous occlusion of the hepatic veins (MOHV) is a common cause of BCS in Asia but is rarely seen in the United States. The pathogenesis is the subject of controversy; many investigators have assumed that webs are congenital, but the onset of symptoms in the fourth decade of life and the pathologic features are more suggestive of a post-thrombotic event.
4. Most patients with BCS have an underlying thrombotic diathesis. In less than 20% of cases is the disorder idiopathic. Disorders associated with BCS include the following:
a. Hematologic disorders
b. Inherited thrombotic diathesis
1. The classic triad of hepatomegaly, ascites, and abdominal pain is seen in most patients but is nonspecific.
2. The natural history of untreated BCS is progression of symptoms often resulting in death caused by complications of portal hypertension. Untreated, the mortality rate is greater than 50%, except in some patients with membranous webs, in whom symptoms develop slowly.
3. Routine biochemical and hematologic parameters are as follows:
4. Ascitic fluid characteristics are useful clues to the diagnosis:
1. A high index of suspicion is necessary for diagnosis because clinical manifestations and laboratory results are nonspecific.
2. Imaging techniques for visualizing hepatic veins are as follows:
Color-flow Doppler ultrasonography is better than duplex ultrasound, which is superior to real-time ultrasonography.
b. Magnetic resonance imaging (MRI) with gadolinium contrast or pulsed sequencing
3. Hepatic venography
4. Pathologic findings on liver biopsy specimens
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