Extrahepatic portal hypertension in children. Long term evaluation.

Extrahepatic portal hypertension in children continues to be a poorly understood entity in many respects. The etiology in most patients is undetermined. The natural history of the condition is not clearly appreciated, particularly with respect to the eventual fate of esophageal varices and the risk of variceal bleeding. Considerable difference of opinion exists in regard to the results of surgical intervention to control or prevent bleeding as well as in regard to the long term affects of such operations. Forty-one children have been followed at this hospital over the past 30 years. A recent and thorough follow-up evaluation has been completed. The purpose of this paper is to analyze this group of patients in regard to these poorly understood areas. Particular attention is paid to the natural history of the condition, the results of therapy, the mortality, and the overall current status of these patients.