Case Report
Copyright ©The Author(s) 2015. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jun 21, 2015; 21(23): 7320-7325
Published online Jun 21, 2015. doi: 10.3748/wjg.v21.i23.7320
How to treat an extensive form of primary intestinal lymphangiectasia?
Rosana Troskot, Dragan Jurčić, Ante Bilić, Marija Gomerčić Palčić, Stanko Težak, Ivana Brajković
Rosana Troskot, Dragan Jurčić, Ante Bilić, Faculty of Medicine, J. J. Strossmayer University of Osijek, Division of Gastroenterology and Hepatology, Department of Internal Medicine, Clinical Hospital “Sveti Duh”, 10000 Zagreb, Croatia
Marija Gomerčić Palčić, Division of Gastroenterology and Hepatology, Department of Internal Medicine, “Sestre milosrdnice” University Hospital Center, University of Zagreb School of Medicine, Vinogradska 29, 10000 Zagreb, Croatia
Stanko Težak, Clinical Department of Nuclear Medicine and Radiation Protection, University Hospital Centre Zagreb, 10000 Zagreb, Croatia
Ivana Brajković, Dialysis clinic, Polyclinic “Sveti Duh” II, 10000 Zagreb, Croatia
Author contributions: Troskot R, Jurčić D, Bilić A, Gomerčić Palčić M and Brajković I collected the data, performed the literature search and wrote the paper; Težak S participated in drafting the outline and revised the paper.
Ethics approval: This case report was reviewed and approved by the Clinical Hospital “Sveti Duh” Ethics Committee.
Informed consent: The patient signed the informed consent form approved by the local Ethics Committee.
Conflict-of-interest: No potential conflicts of interest relevant to this article were reported.
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: Rosana Troskot, MD, PhD, Faculty of Medicine, J. J. Strossmayer University of Osijek, Division of Gastroenterology and Hepatology, Department of Internal Medicine, Clinical Hospital “Sveti Duh”, Sveti Duh 64, 10000 Zagreb, Croatia. rtroskot@gmail.com
Telephone: +385-1-3712226 Fax: +385-1-3712203
Received: January 10, 2015
Peer-review started: January 10, 2015
First decision: February 10, 2015
Revised: February 27, 2015
Accepted: March 31, 2015
Article in press: March 31, 2015
Published online: June 21, 2015
Abstract

We report a case of a 42-year-old man with a rare disorder known as primary intestinal lymphangiectasia, which is characterized by dilated intestinal lymphatics that lead to the development of protein-losing enteropathy. The patient presented with a grand mal seizure caused by malabsorption-derived electrolytes and a protein disorder. Signs of the disease, including chronic diarrhea and peripheral edema, manifested 10 years ago, but a diagnosis was never made. The diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Hyperemic and edematous mucosa of the small intestine corresponded to scattered white spots with dilated intestinal lymphatics and whitish villi in the histological specimen of the biopsied jejunal mucosa. Although numerous therapeutic strategies are available, only octreotide therapy proved to be an effective means of therapeutic resolution in this patient. Although the patient had a partial remission following the use of a slow release formula of octreotide, his prognosis, clinical course, and future treatment challenges are yet to be determined.

Keywords: Primary intestinal lymphangiectasia, Malabsorption, Seizure, Treatment, Diet, Octreotide

Core tip: This is a case of a 42-year-old man who presented with a grand mal seizure, malabsorption-derived electrolytes and a protein disorder caused by primary intestinal lymphangiectasia. After an extensive management and exclusion of secondary intestinal lymphangiectasia, a diagnosis was suspected because of the clinical manifestations, laboratory tests, imaging and endoscopic findings. Although numerous therapeutic strategies are available, only octreotide therapy proved to be a potentially effective means of therapeutic resolution; however, a longer follow-up period is needed. More case reports and/or studies on similar cases are needed to determine the best possible treatment strategies for this rare disorder.