Noncirrhotic extrahepatic and intrahepatic portal hypertension

A variety of conditions may lead to the development of portal hypertension in the absence of cirrhosis. In virtually all cases, increased resistance to flow at the level of the portal vein, sinusoids, or hepatic vein is the major contributing factor to the elevated portal pressure, although increased flow may be a contributor in certain instances. Variceal hemorrhage is the major complication of these disorders, but is better tolerated than in cirrhosis because of better hepatocellular function. Therapy should in general be conservative and directed toward any underlying pathology, where such can be identified.