Retrospective Study
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Gastroenterol. Jun 14, 2022; 28(22): 2482-2493
Published online Jun 14, 2022. doi: 10.3748/wjg.v28.i22.2482
Endoscopic classification and pathological features of primary intestinal lymphangiectasia
Ming-Ming Meng, Kui-Liang Liu, Xin-Ying Xue, Kun Hao, Jian Dong, Chun-Kai Yu, Hong Liu, Cang-Hai Wang, Hui Su, Wu Lin, Guo-Jun Jiang, Nan Wei, Ren-Gui Wang, Wen-Bin Shen, Jing Wu
Ming-Ming Meng, Kui-Liang Liu, Hong Liu, Cang-Hai Wang, Hui Su, Wu Lin, Guo-Jun Jiang, Nan Wei, Jing Wu, Department of Gastroenterology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
Xin-Ying Xue, Department of Respiratory and Critical Care Medicine, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
Kun Hao, Wen-Bin Shen, Departments of Lymphatic Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
Jian Dong, Ren-Gui Wang, Departments of Radiology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
Chun-Kai Yu, Departments of Pathology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
Author contributions: Meng MM, Liu KL, Xue XY, Hao K, Dong J and Yu CK contributed equally to this work; Wu J, Meng MM and Liu KL were the gastroenterologists; Shen WB and Hao K were the lymphologist; Wang RG and Dong J performed the radiological diagnosis; Yu CK performed the pathological diagnosis; Meng MM and Xue XY designed the research study; Liu KL, Liu H, Wang CH, Su H, Lin W, Jiang GJ and Wei N performed the primary literature and data extraction; Meng MM, Xue XY analyzed the data and wrote the manuscript; Wu J, Shen WB and Wang RG were responsible for revising the manuscript for important intellectual content; and all authors read and approved the final version.
Supported by National Natural Science Foundation of China, No. 61876216; and Beijing Shijitan Hospital Foundation of Capital Medical University, No. 2019-LB12.
Institutional review board statement: The study was reviewed and approved by the Beijing Shijitan Hospital, Capital Medical University Institutional Review Board [Approval No. Sjtkyll-lx-2021(90)].
Informed consent statement: All study participants or their legal guardian provided informed written consent about personal and medical data collection prior to study enrolment.
Conflict-of-interest statement: The authors declare no conflicts of interest for this article.
Data sharing statement: Technical appendix, statistical code, and dataset available from the corresponding author at wujing36@163.com. Participants gave informed consent for data sharing.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (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: https://creativecommons.org/Licenses/by-nc/4.0/
Corresponding author: Jing Wu, MD, PhD, Chief Doctor, Professor, Department of Gastroenterology, Beijing Shijitan Hospital, Capital Medical University, No. 10 Tieyi Street, Haidian District, Beijing 100038, China. wujing36@163.com
Received: November 2, 2021
Peer-review started: November 2, 2021
First decision: December 3, 2021
Revised: December 9, 2021
Accepted: April 28, 2022
Article in press: April 28, 2022
Published online: June 14, 2022
ARTICLE HIGHLIGHTS
Research background

Primary intestinal lymphangiectasia (PIL) is a rare disorder that typically presents as PLE, diarrhea, and limb edema.

Research motivation

Unspecific symptoms and a wide range of clinical manifestations can significantly hamper the establishment of a definitive diagnosis.

Research objectives

This study aimed to classify the endoscopic features of the intestinal mucosa in PIL under endoscopy, combine post-lymphographic computed tomography findings and the patients’ pathological characteristics of the patients, and explain their causes.

Research methods

This retrospective analysis of endoscopic images of patients diagnosed with PIL during a 12-year period at our research center summarized and classified types into four distinct endoscopic features and further explored the differences in radiologic and pathologic features of the different endoscopic features.

Research results

The endoscopic symptoms of 123 patients with PIL can be divided into four types: Nodular (13.8%), granular (60.2%), vesicular (4.1%), and edematous (21.9%).

Research conclusions

We proposed the theory of nodular and vesicle types domestically through observation, and made breakthroughs in overcoming the issue of inaccurate diagnosis based on a few independent early-stage case reports.

Research perspectives

According to the different partings under endoscopy, the clinical symptoms, therapeutic efficacy, and prognosis of patients with PIL were observed.