Review
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Hepatol. Feb 27, 2015; 7(2): 127-138
Published online Feb 27, 2015. doi: 10.4254/wjh.v7.i2.127
Portal hypertensive enteropathy
Parit Mekaroonkamol, Robert Cohen, Saurabh Chawla
Parit Mekaroonkamol, Robert Cohen, Saurabh Chawla, Grady Memorial Hospital, Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Atlanta, GA 30322, United States
Author contributions: Mekaroonkamol P and Chawla S contributed significantly to conception, manuscript preparation and revision; Cohen R participated with data contribution, interpretation and revision.
Open-Access: This article is an open-access article which was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
Correspondence to: Saurabh Chawla, MD, Assistant Professor of Medicine, Director of Interventional Endoscopy, Grady Memorial Hospital, Division of Digestive Diseases, Department of Medicine, Emory University School of Medicine, Faculty Office Building, 49 Jesse Hill Jr. Drive,Suite 431, Atlanta, GA 30322, United States. saurabh.chawla@emory.edu
Telephone: +1-140-47781684 Fax: +1-140-47781681
Received: August 27, 2014
Peer-review started: September 2, 2014
First decision: October 14, 2014
Revised: October 28, 2014
Accepted: November 17, 2014
Article in press: November 19, 2014
Published online: February 27, 2015
Abstract

Portal hypertensive enteropathy (PHE) is a condition that describes the pathologic changes and mucosal abnormalities observed in the small intestine of patients with portal hypertension. This entity is being increasingly recognized and better understood over the past decade due to increased accessibility of the small intestine made possible by the introduction of video capsule endoscopy and deep enteroscopy. Though challenged by its diverse endoscopic appearance, multiple scoring systems have been proposed to classify the endoscopic presentation and grade its severity. Endoscopic findings can be broadly categorized into vascular and non-vascular lesions with many subtypes of both categories. Clinical manifestations of PHE can range from asymptomatic incidental findings to fatal gastrointestinal hemorrhage. Classic endoscopic findings in the setting of portal hypertension may lead to a prompt diagnosis. Occasionally histopathology and cross sectional imaging like computed tomography or magnetic resonance imaging may be helpful in establishing a diagnosis. Management of overt bleeding requires multidisciplinary approach involving hepatologists, endoscopists, surgeons, and interventional radiologists. Adequate resuscitation, reduction of portal pressure, and endoscopic therapeutic intervention remain the main principles of the initial treatment. This article reviews the existing evidence on PHE with emphasis on its classification, diagnosis, clinical manifestations, endoscopic appearance, pathological findings, and clinical management. A new schematic management of ectopic variceal bleed is also proposed.

Keywords: Portal hypertension, Enteropathy, Intestinal vasculopathy, Ectopic varices, Gastrointestinal bleeding

Core tip: Portal hypertensive enteropathy (PHE) is an under recognized complication of portal hypertension. It can present with a broad spectrum of clinical manifestations and endoscopic findings, making its diagnosis challenging. Video capsule endoscopy and deep enteroscopy are diagnostic tools of choice. PHE should be considered in patients with portal hypertension who present with occult or overt gastrointestinal bleeding, especially when portal hypertensive gastropathy and advanced cirrhosis are also present. Adequate resuscitation, reduction of portal pressure, and endoscopic therapeutic intervention remain the mainstay of initial treatment though definitive management may require a multidisciplinary approach involving hepatologists, endoscopists, surgeons, and interventional radiologists.