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
ARTICLE HIGHLIGHTS
Case characteristics

A 68-year-old female patient presented three times to the Emergency Room with confusion, lethargy, dysarthria, nausea and vomiting.

Clinical diagnosis

Disabling portosystemic encephalopathy due to a giant inferior mesenteric-caval shunt via the left internal iliac vein.

Differential diagnosis

Elevated concentrations of circulating ammonia in patients with normal liver function were reported in a wide spectra of conditions which include high protein diet, severe constipation, gastrointestinal bleeding, several drugs, gastric Helicobacter pylori infection and urinary tract infection with high urease-producing bacteria (Pseudomonas, Proteus), haemodialysis and enzyme deficit of urea cycle.

Laboratory diagnosis

Serum ammonia level was found far above the normal value > 400 mg/dL (nv < 75 mg/dL). Euthyroidism, euglycemia, normal levels of ACTH and cortisol, normal GH with low IGF-1 for age INR in range, normal renal function and sodium/potassium levels were observed. Slightly elevated transaminases and direct/indirect bilirubin were detected. Viral Hepatitis markers were negative as well as HIV1-2 serology, tumor markers and humoral autoimmunity markers.

Imaging diagnosis

Abdomen computed tomography angiography revealed the patency of portal vein trunk with an enlarged superior mesenteric vein and a giant portosystemic shunt. The shunt presented a maximum caliber of 20 mm and showed a large retroperitoneal loop emerging from the spleno-mesenteric confluence with discharge in the left hypogastric vein.

Pathological diagnosis

Giant inferior mesenteric-caval shunt via the left internal iliac vein.

Treatment

Percutaneous transcatheter embolization using Amplatzer plug and coils.

Related reports

Mechanical embolization of an inferior mesenteric-caval shunt via the left internal iliac vein was described in a similar case by Otake et al[2], Intern Med 2001.

Term explanation

Mechanical embolization was preferred to sclerosis with chemical agents to avoid the risk of reflux in the portal system with potential catastrophic complications. A control CT confirmed the complete shunt exclusion. As a consequence, mesenteric venous blood restarted to fully hepatopetally flow into the liver, metabolic detoxification of ammonia increased and encephalopathy subsided.

Experiences and lessons

It is crucial that physicians initially recognize the presence of hyperammonemic encephalopaty and then, even if rare, consider those case not related to cirrhosis that can therefore be fully healed. Accurate diagnosis and subsequent appropriate treatments are able to fully revert the symptoms in most patients.