Hepatic veno-occlusive disease is caused by endothelial injury, leading to nonthrombotic occlusion of the terminal hepatic venules and hepatic sinusoids, rather than of the hepatic veins or inferior vena cava (as in Budd-Chiari syndrome).
Venous congestion causes portal hypertension and ischemic necrosis (which leads to cirrhosis).
Common causes include
Symptoms and Signs
Initial manifestations include sudden jaundice, ascites, and tender, smooth hepatomegaly. Onset is within the first 3 wk of transplantation in bone marrow or hematopoietic cell recipients, who either recover spontaneously within a few weeks (or sometimes, with mild cases, after an increase in immunosuppressant therapy) or die of fulminant liver failure. Other patients have recurrent ascites, portal hypertension, splenomegaly, and, eventually, cirrhosis.
The diagnosis is suspected in patients with unexplained clinical or laboratory evidence of liver disease, particularly in those with known risk factors, such as bone marrow or hematopoietic cell transplantation.
Laboratory results are nonspecific: elevated aminotransferase and conjugated bilirubin levels. PT/INR becomes abnormal when disease is severe. Ultrasonography shows retrograde flow in the portal vein.
If the diagnosis is unclear, invasive tests become necessary—eg, liver biopsy or measurement of the portal-hepatic venous pressure gradient (a pressure gradient > 10 mm Hg suggests veno-occlusive disease). Measuring the pressure across the liver entails inserting a catheter percutaneously into a hepatic vein and then wedging it into the liver. This wedged pressure reflects portal vein pressure. (An exception is portal vein thrombosis; in this case, the pressure is normal despite portal hypertension.)
Ursodeoxycholic acid helps prevent graft-vs-host disease in bone marrow or hematopoietic cell transplant recipients. Management includes withdrawing the causative agent (such as herbal teas) and providing supportive therapy.
Most patients have mild to moderate disease and do quite well. Transjugular intrahepatic portosystemic shunting (TIPS) can be tried for relief of portal hypertension, but has not yet been shown to prolong survival, particularly when veno-occlusive disease is severe. In 25%, veno-occlusive disease is severe, accompanied by fulminant liver failure. Liver transplantation is a last resort.
Last full review/revision September 2013 by Nicholas T. Orfanidis, MD
Content last modified October 2013