Portal hypertensive enteropathy

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.