摘要

Extrahepatic portal venous obstruction (EHPVO) in developing countries constitutes the most common cause of non-cirrhotic portal hypertension (PHT) in children. Local precipitating factor particularly infection on a preexisting prothrombotic state could probably be the reason for selective portal vein thrombosis. In this era of highly successful endoscopic and pharmacotherapy management of PHT, issues that assume importance are those of endoscopic outcome beyond esophageal variceal eradication, growth retardation, portal biliopathy and hypersplenism. Following esophageal variceal eradication in EHPVO a significant decrease in gastric varices along lesser curvature (GOV1), increase in isolated gastric varices and increased frequency and severity of portal hypertensive gastropathy take place. Small rebleeding risk persists from gastric varices. EHPVO in children leads to growth retardation. Anthropometric and preliminary hormonal evaluation suggests resistance to the action of growth hormone. Evidence to suggest acceleration of growth following shunt surgery is weak. Mechanism of growth failure in children with EHPVO is still not clear. Portal biliopathy is a serious complication that results in cholangitis and biliary obstruction. Management of portal biliopathy is difficult that needs endoscopic and surgical intervention. Progressive increase in hypersplenism occurs on medical therapy. Research should be directed towards knowing the exact etiology, mechanism of growth retardation and best management strategy for dealing with portal biliopathy.