Na JE, Kim JE, Park S, Kim ER, Hong SN, Kim YH, Chang DK. Experience of primary intestinal lymphangiectasia in adults: Twelve case series from a tertiary referral hospital. World J Clin Cases 2024; 12(4): 746-757 [PMID: 38322684 DOI: 10.12998/wjcc.v12.i4.746]
Corresponding Author of This Article
Ji Eun Kim, MD, PhD, Department of Medicine, Samsung Medical Center, 81 Irwon-ro, Gangnam-gu, Seoul 06351, South Korea.happyjinny0706@gmail.com
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Retrospective Study
Open-Access Policy of This Article
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/
World J Clin Cases. Feb 6, 2024; 12(4): 746-757 Published online Feb 6, 2024. doi: 10.12998/wjcc.v12.i4.746
Experience of primary intestinal lymphangiectasia in adults: Twelve case series from a tertiary referral hospital
Ji Eun Na, Ji Eun Kim, Sujin Park, Eun Ran Kim, Sung Noh Hong, Young-Ho Kim, Dong Kyung Chang
Ji Eun Na, Department of Internal Medicine, Inje University Haeundae Paik Hospital, Busan 48108, South Korea
Ji Eun Kim, Eun Ran Kim, Sung Noh Hong, Young-Ho Kim, Dong Kyung Chang, Department of Medicine, Samsung Medical Center, Seoul 06351, South Korea
Sujin Park, Department of Pathology, Samsung Medical Center, Seoul 06351, South Korea
Author contributions: Na JE and Kim JE contributed study concept and design; Na JE and Kim JE contributed acquisition, analysis, or interpretation of the data; Na JE and Kim JE contributed writing and drafting of the manuscript; Kim JE, Hong SN, Kim ER, Kim YH, Chang DK and Park S contributed critical revision of the manuscript for important intellectual content; Na JE contributed statistical analysis; all authors approved the final submission.
Institutional review board statement: The study was reviewed and approved by the Institutional Review Board of Samsung Medical Center (SMC IRB No. 2023-02-093-001).
Informed consent statement: The requirement for informed consent was waived due to the retrospective nature of the study and its observational design.
Conflict-of-interest statement: The authors have no conflicts of interest relevant to this study to disclose.
Data sharing statement: Data underlying this article are available from the corresponding author upon reasonable request.
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: Ji Eun Kim, MD, PhD, Department of Medicine, Samsung Medical Center, 81 Irwon-ro, Gangnam-gu, Seoul 06351, South Korea.happyjinny0706@gmail.com
Received: October 11, 2023 Peer-review started: October 11, 2023 First decision: December 8, 2023 Revised: December 17, 2023 Accepted: January 8, 2024 Article in press: January 8, 2024 Published online: February 6, 2024 Processing time: 106 Days and 1.3 Hours
Abstract
BACKGROUND
While primary intestinal lymphangiectasia (PIL) is considered a rare condition, there have been several reported cases in adults. Nevertheless, the absence of clear guidance from diagnosis to treatment and prognosis poses challenges for both physicians and patients.
AIM
To enhance understanding by investigating clinical presentation, diagnosis, treatment, complications, and prognoses in adult PIL cases.
METHODS
We enrolled adult patients diagnosed with PIL between March 2016 and September 2021. The primary outcome involved examining the diagnosis and treatment process of these patients. The secondary outcomes included identifying complications (infections, thromboembolism) and assessing prognoses (frequency of hospitalization and mortality) during the follow-up period.
RESULTS
Among the 12 included patients, peripheral edema (100%) and diarrhea (75%) were the main presenting complaints. Laboratory tests showed that all the patients exhibited symptoms of hypoalbuminemia and hypogammaglobulinemia. Radiologically, the predominant findings were edema of the small intestine (67%) and ascites (58%). The typical endoscopic finding with a snowflake appearance was observed in 75% of patients. Among the 12 patients, two responded positively to octreotide and sirolimus, and eight who could undergo maintenance therapy discontinued subsequently. Complications due to PIL led to infection in half of the patients, thromboembolism in three patients, and one death.
CONCLUSION
PIL can be diagnosed in adults across various age groups, with different severity and treatment responses among patients, leading to diverse complications and prognoses. Consequently, tailored treatments will be necessary. We anticipate that our findings will contribute to the management of PIL, an etiology of protein-losing enteropathy.
Core Tip: We reviewed the diagnosis, treatment, and prognosis of adult patients diagnosed with primary intestinal lymphangiectasia and found varying severities. Hence, tailored treatments are essential for adult patients with primary intestinal lymphangiectasia given the diverse presentations and responses.
