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Meng MM, Liu KL, Xue XY, Hao K, Dong J, Yu CK, Liu H, Wang CH, Su H, Lin W, Jiang GJ, Wei N, Wang RG, Shen WB, Wu J. Endoscopic classification and pathological features of primary intestinal lymphangiectasia. World J Gastroenterol 2022; 28:2482-2493. [PMID: 35979262 PMCID: PMC9258282 DOI: 10.3748/wjg.v28.i22.2482] [Citation(s) in RCA: 4] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 11/02/2021] [Revised: 12/09/2021] [Accepted: 04/28/2022] [Indexed: 02/06/2023] Open
Abstract
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.
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Affiliation(s)
- Ming-Ming Meng
- Department of Gastroenterology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
| | - Kui-Liang Liu
- 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
- Departments of Lymphatic Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
| | - Jian Dong
- 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
| | - Hong Liu
- Department of Gastroenterology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
| | - Cang-Hai Wang
- Department of Gastroenterology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
| | - Hui Su
- Department of Gastroenterology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
| | - Wu Lin
- Department of Gastroenterology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
| | - Guo-Jun Jiang
- Department of Gastroenterology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
| | - Nan Wei
- Department of Gastroenterology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
| | - Ren-Gui Wang
- Departments of Radiology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
| | - Wen-Bin Shen
- Departments of Lymphatic Surgery, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
| | - Jing Wu
- Department of Gastroenterology, Beijing Shijitan Hospital, Capital Medical University, Beijing 100038, China
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Oh TG, Chung JW, Kim HM, Han SJ, Lee JS, Park JY, Song SY. Primary intestinal lymphangiectasia diagnosed by capsule endoscopy and double balloon enteroscopy. World J Gastrointest Endosc 2011; 3:235-40. [PMID: 22110841 PMCID: PMC3221958 DOI: 10.4253/wjge.v3.i11.235] [Citation(s) in RCA: 19] [Impact Index Per Article: 1.4] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 05/06/2011] [Revised: 10/19/2011] [Accepted: 10/28/2011] [Indexed: 02/05/2023] Open
Abstract
Primary intestinal lymphangiectasia (PIL) is a rare disorder characterized by dilated intestinal lymphatics and the development of protein-losing enteropathy. Patients with PIL develop hypoalbuminemia, hypocalcemia, lymphopenia and hypogammaglobulinemia, and present with bilateral lower limb edema, fatigue, abdominal pain and diarrhea. Endoscopy reveals diffusely elongated, circumferential and polypoid mucosae covered with whitish enlarged villi, all of which indicate intestinal lymphangiectasia. Diagnosis is confirmed by characteristic tissue pathology, which includes dilated intestinal lymphatics with diffusely swollen mucosa and enlarged villi. The prevalence of PIL has increased since the introduction of capsule endoscopy. The etiology and prevalence of PIL remain unknown. Some studies have reported that several genes and regulatory molecules for lymphangiogenesis are related to PIL. We report the case of a patient with PIL involving the entire small bowel that was confirmed by capsule endoscopy and double-balloon enteroscopy-guided tissue pathology who carried a deletion on chromosome 4q25. The relationship between this deletion on chromosome 4 and PIL remains to be investigated.
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Affiliation(s)
- Tak Geun Oh
- Tak Geun Oh, Joo Won Chung, Hee Man Kim, Jung Yeob Park, Si Young Song, Division of Gastroenterology, Department of Internal Medicine, Yonsei Institute of Gastroenterology, Yonsei University College of Medicine, Seoul 120-752, South Korea
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