Extra-hepatic portal hypertension (EHPH) defined as non cirrhotic, presinusoidal and prehepatic portal hypertension, with obstruction and cavernomatous transformation of the main portal vein, entails a high, early and prolonged risk of gastro-intestinal bleeding (GIB) mainly from esophageal and/or gastric varices, and less often a risk of cholangiopathy or protein-losing enteropathy. Diagnosis of EHPH may be done with non invasive imaging techniques. Assessment of bleeding risk is based on results of endoscopic examination. Occurence of a bleeding episode or onset during follow-up of endoscopic signs of high risk of GIB require radical eradication of varices. Radical cure of EHPH is achieved at best by bypass surgery restoring a physiological portal flow, and as a second choice by shunt surgery. Endoscopic therapy has a place as first line treatment of GIB episodes, and also in a few cases with poor extrahepatic portal network contra-indicating efficient vascular surgery.
Service de Chirurgie Pédiatrique, Centre Hospitalier Universitaire Bicêtre, Assistance Publique Hôpitaux de Paris, Le Kremlin Bicêtre, France
Address reprint requests and correspondence: Frédéric Gauthier, MD, Service de Chirurgie Pédiatrique, Centre Hospitalier Universitaire Bicêtre, Assistance Publique Hôpitaux de Paris, Faculté de Médecine Paris Sud, 78 rue du Général Leclerc, 94270 Le Kremlin Bicétre, France
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