[Rational therapy of prehepatic portal hypertension]

Prehepatic portal hypertension is mostly caused by an inflammatory closure (thrombosis) of the portal vein in the perinatal period. This form of portal hypertension is characterized by the following considerable facts: patients' liver is not damaged and a divergent collateral circulation (hepatofugal and hepatopetal) is formed. Due to the latter, portal circulation may improve spontaneously and no clinical signs of the disease are manifested. Most patients, however bleed rependedly from the cardioesophageal varices with various frequency and various severity. A direct surgical intervention had been successful in about 50% of cases. At present, it is almost replaced by endoscopic sclerotization giving compatible results. The pathogenetically based surgical method is the portosystemic shut that is necessary in 20% of patients, mostly as a secondary intervention. Pathophysiological sequelae of the shunt are less severe than in cirrhotic patients due to intact liver of patients with PPH and to favorable possibility of recirculation of portal blood via the hepatic artery. An appropriate therapeutical strategy accounts for favorable long-term prognosis of patients with prehepatic portal hypertension.