Recent concepts regarding extra-hepatic portal hypertension

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.