Case Report
Copyright ©The Author(s) 2017. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Dec 21, 2017; 23(47): 8426-8431
Published online Dec 21, 2017. doi: 10.3748/wjg.v23.i47.8426
Disabling portosystemic encephalopathy in a non-cirrhotic patient: Successful endovascular treatment of a giant inferior mesenteric-caval shunt via the left internal iliac vein
Luca de Martinis, Gloria Groppelli, Riccardo Corti, Lorenzo Paolo Moramarco, Pietro Quaretti, Pasquale De Cata, Mario Rotondi, Luca Chiovato
Luca de Martinis, Gloria Groppelli, Pasquale De Cata, Mario Rotondi, Luca Chiovato, Unit of Endocrinology and Internal Medicine, University of Pavia, ICS Maugeri SPA Società Benefit, Pavia 27100, Italy
Riccardo Corti, Lorenzo Paolo Moramarco, Pietro Quaretti, Unit of Interventional Radiology, Radiology Department, IRCCS Fondazione Policlinico San Matteo, Pavia 27100, Italy
Author contributions: de Martinis L and Groppelli G performed the data collection; Moramarco LP and Quaretti P performed the vascular intervention; Corti R provided radiological support; de Martinis L, De Cata P, Quaretti P, Rotondi M and Chiovato L designed and wrote the report; all authors contributed to revision of this manuscript.
Informed consent statement: The patient gave her written informed consent authorizing use and disclosure of her protected health information.
Conflict-of-interest statement: All authors have no conflicts of interests to declare.
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: Luca de Martinis, MD, Doctor, Unit of Endocrinology and Internal Medicine, University of Pavia, ICS Maugeri SPA Società Benefit, Via Maugeri 10, Pavia 27100, Italy. luca.demartinis01@universitadipavia.it
Telephone: +39-339-6940039 Fax: +39-382-592692
Received: September 22, 2017
Peer-review started: September 23, 2017
First decision: October 11, 2017
Revised: October 20, 2017
Accepted: November 1, 2017
Article in press: November 1, 2017
Published online: December 21, 2017
Processing time: 88 Days and 10.6 Hours
Abstract

Hepatic encephalopathy is suspected in non-cirrhotic cases of encephalopathy because the symptoms are accompanied by hyperammonaemia. Some cases have been misdiagnosed as psychiatric diseases and consequently patients hospitalized in psychiatric institutions or geriatric facilities. Therefore, the importance of accurate diagnosis of this disease should be strongly emphasized. A 68-year-old female patient presented to the Emergency Room with confusion, lethargy, nausea and vomiting. Examination disclosed normal vital signs. Neurological examination revealed a minimally responsive woman without apparent focal deficits and normal reflexes. She had no history of hematologic disorders or alcohol abuse. Her brain TC did not demonstrate any intracranial abnormalities and electroencephalography did not reveal any subclinical epileptiform discharges. Her ammonia level was > 400 mg/dL (reference range < 75 mg/dL) while hepatitis viral markers were negative. The patient was started on lactulose, rifaximin and low-protein diet. On the basis of the doppler ultrasound and abdomen computed tomography angiography findings, the decision was made to attempt portal venography which confirmed the presence of a giant portal-systemic venous shunt. Therefore, mechanic obliteration of shunt by interventional radiology was performed. As a consequence, mesenteric venous blood returned to hepatopetally flow into the liver, metabolic detoxification of ammonia increased and hepatic encephalopathy subsided. It is crucial that physicians immediately recognize the presence of non-cirrhotic encephalopathy, in view of the potential therapeutic resolution after accurate diagnosis and appropriate treatments.

Keywords: Non-cirrhotic patient; Portosystemic shunt; Hyperammonaemia; Interventional radiology; Mechanical embolization; Encephalopathy

Core tip: We present the case of a non-cirrhotic female patient who first presented to the Emergency Room with acute hyperammonemic encephalopathy causing massive relapsing neurological symptoms due to a huge inferior mesenteric-caval shunt via the left internal iliac vein which was successfully cured by interventional radiology procedure. Therefore, the importance of accurate diagnosis and appropriate treatment of this disease should be strongly emphasized.