Copyright
©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
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
BACKGROUND
The appearance of the intestinal mucosa during endoscopy varies among patients with primary intestinal lymphangiectasia (PIL).
AIM
To classify the endoscopic features of the intestinal mucosa in PIL under endoscopy, combine the patients’ imaging and pathological characteristics of the patients, and explain their causes.
METHODS
We retrospectively analyzed the endoscopic images of 123 patients with PIL who were treated at the hospital between January 1, 2007 and December 31, 2018. We compared and analyzed all endoscopic images, classified them into four types according to the endoscopic features of the intestinal mucosa, and analyzed the post-lymphographic computed tomography (PLCT) and pathological characteristics of each type.
RESULTS
According to the endoscopic features of PIL in 123 patients observed during endoscopy, they were classified into four types: nodular-type, granular-type, vesicular-type, and edematous-type. PLCT showed diffuse thickening of the small intestinal wall, and no contrast agent was seen in the small intestinal wall and mesentery in the patients with nodular and granular types. Contrast agent was scattered in the small intestinal wall and mesentery in the patients with vesicular and edematous types. Analysis of the small intestinal mucosal pathology revealed that nodular-type and granular-type lymphangiectasia involved the small intestine mucosa in four layers, whereas ectasia of the vesicular- and edematous-type lymphatic vessels largely involved the lamina propria mucosae, submucosae, and muscular layers.
CONCLUSION
Endoscopic classification, combined with the patients’ clinical manifestations and pathological examination results, is significant and very useful to clinicians when scoping patients with suspected PIL.
Core Tip: Primary intestinal lymphangiectasia (PIL) is a rare disorder that typically presents as protein-losing enteropathy, diarrhea, and limb edema. Here we analyzed the endoscopic presentation, post-lymphographic computed tomography findings, and pathological features of 123 patients with PIL. We classified PIL into four categories - nodular, granular, vesicle, and edema types, 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.
