Presinusoidal extrahepatic portal hypertension: a review of thirty-five cases variously treated.

Thirty-five cases of proven preinusoidal extrahepatic portal hypertension in children have been reviewed with particular reference to etiology, clinical course, therapy, and long-term follow up results. Thirty-three patients has significant gastrointestinal bleeding and 31 were operated upon. There were no operative deaths. Portosystemic venous shunts were successful in 12 of 21 patients. A variety of procedures were used for 15 of the 17 "unshuntable" individuals. Some form of esophagectomy and proximal partial gastrectomy with colon substitution appears to be the most effective operation for control of hemorrhage in the "unshuntable" patients. Expectant treatment is preferred in the infant and younger child, but the older child should have a definitive shunt or direct attack on the varices since recurrent bleeding is usually the rule.