Lv YQ, Wang ML, Tang TY, Li YQ. Comprehensive treatment and a rare presentation of Cronkhite–Canada syndrome: Two case reports and review of literature. World J Gastrointest Surg 2023; 15(11): 2646-2656 [PMID: 38111781 DOI: 10.4240/wjgs.v15.i11.2646]
Corresponding Author of This Article
Yu-Qin Li, MD, Chief Physician, Professor, Department of Gastroenterology, The First Hospital of Jilin University, No. 71 Xinmin Street, Changchun 130021, Jilin Province, China. liyuq@jlu.edu.cn
Research Domain of This Article
Gastroenterology & Hepatology
Article-Type of This Article
Case Report
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 Gastrointest Surg. Nov 27, 2023; 15(11): 2646-2656 Published online Nov 27, 2023. doi: 10.4240/wjgs.v15.i11.2646
Comprehensive treatment and a rare presentation of Cronkhite–Canada syndrome: Two case reports and review of literature
Yan-Qing Lv, Mei-Lan Wang, Tong-Yu Tang, Yu-Qin Li
Yan-Qing Lv, Department of Hepatobiliary and Pancreatic Medicine, The First Hospital of Jilin University, Changchun 130021, Jilin Province, China
Mei-Lan Wang, Department of Gastroenterology, Jilin Provincial People's Hospital, Changchun 130021, Jilin Province, China
Tong-Yu Tang, Yu-Qin Li, Department of Gastroenterology, The First Hospital of Jilin University, Changchun 130021, Jilin Province, China
Author contributions: Lv YQ collected and sorted out the cases, reviewed the literature, and wrote the manuscript; Wang ML reviewed the literature; Tang TY contributed to the content and editing of the manuscript; Li YQ reviewed and revised the manuscript; All authors have read and approved the final manuscript.
Supported byJilin Provincial Science and Technology Department Project, No. 20200201343JC; and Science and Technology Development Program of Jilin Province, No. 20210402013GH.
Informed consent statement: This study was approved by the Ethics Committee of the First Bethune Hospital of Jilin University to waive informed written consent about personal and medical data collection.
Conflict-of-interest statement: The authors declare that they have no conflict of interest to disclose.
CARE Checklist (2016) statement: The authors have read the CARE Checklist (2016), and the manuscript was prepared and revised according to the CARE Checklist (2016).
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: Yu-Qin Li, MD, Chief Physician, Professor, Department of Gastroenterology, The First Hospital of Jilin University, No. 71 Xinmin Street, Changchun 130021, Jilin Province, China. liyuq@jlu.edu.cn
Received: August 15, 2023 Peer-review started: August 15, 2023 First decision: September 1, 2023 Revised: September 15, 2023 Accepted: October 17, 2023 Article in press: October 17, 2023 Published online: November 27, 2023 Processing time: 103 Days and 20.8 Hours
Abstract
BACKGROUND
Cronkhite–Canada syndrome (CCS) is a rare sporadic polyposis syndrome that presents with gastrointestinal and ectodermal symptoms in addition to nutritional deficiencies. CCS combined with hypothyroidism is an even rarer condition, with no standard treatment guidelines.
CASE SUMMARY
The present study described 2 patients with CCS: A 67-year-old woman with concomitant hypothyroidism and 68-year-old man treated with endoscopic mucosal resection (EMR). Both patients had multiple gastrointestinal symptoms and ectodermal changes, along with multiple gastrointestinal polyps. Microscopic examination showed that the mucosa in both patients was hyperemic and edematous, with pathologic examination showing distorted, atrophic, and dilated glands. Patient 1 had concomitant hypothyroidism and was treated with levothyroxine. Due to her self-reduction of hormone dose, her disease relapsed. Patient 2 underwent EMR, but refused further hormonal or biological treatments. Subsequently, he was treated with an oral Chinese medical preparation.
CONCLUSION
Pharmacotherapy can induce and maintain remission in CCS patients, with adjuvant EMR, long-term follow-up, and endoscopic surveillance being necessary.
Core Tip: Cronkhite–Canada syndrome (CCS) is a rare, non-genetic syndrome characterized by ectodermal abnormalities and diffuse gastrointestinal polyps with protein loss. To date, 7 patients with CCS combined with hypothyroidism have been identified. We report two cases, one of which is combined with hypothyroidism. Through indexing and analyzing PubMed, Web of Science, and Embase databases, we summarized the clinical characteristics of CCS combined with hypothyroidism. Additionally, we concluded that pharmacotherapy can induce and maintain remission in CCS patients, with adjuvant endoscopic mucosal resection, long-term follow-up, and endoscopic surveillance being necessary.
