Case Report
Copyright ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jul 6, 2022; 10(19): 6529-6535
Published online Jul 6, 2022. doi: 10.12998/wjcc.v10.i19.6529
Triple A syndrome-related achalasia treated by per-oral endoscopic myotomy: Three case reports
Feng-Chen Liu, Yun-Lu Feng, Ai-Ming Yang, Tao Guo
Feng-Chen Liu, Yun-Lu Feng, Ai-Ming Yang, Tao Guo, Department of Gastroenterology, Peking Union Medical College Hospital, Beijing 100730, China
Author contributions: Liu FC wrote this article; Feng YL designed and revised this article; Yang AM and Gou T performed per-oral endoscopic myotomy procedures.
Informed consent statement: All study participants or their legal guardian provided informed written consent prior to study enrollment.
Conflict-of-interest statement: The authors declare that they have no conflict of interest.
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: Yun-Lu Feng, MD, Doctor, Department of Gastroenterology, Peking Union Medical College Hospital, No. 1 Shuai Fu Yuan, Dongcheng District, Beijing 100730, China. f13161662700@163.com
Received: October 6, 2021
Peer-review started: October 6, 2021
First decision: December 4, 2021
Revised: December 19, 2021
Accepted: May 8, 2022
Article in press: May 8, 2022
Published online: July 6, 2022
Processing time: 261 Days and 1.1 Hours
Abstract
BACKGROUND

Triple A syndrome is a rare autosomal recessive disease characterized by adrenocorticotropic hormone-resistant adrenal insufficiency, alacrima and achalasia. In the last 5 years, per-oral endoscopic myotomy (POEM) has proved highly successful in treating primary achalasia over the long term, but its long-term performance has not been certified by achalasia related to Triple A syndrome.

CASE SUMMARY

Triple A syndrome is a rare autosomal recessive disease characterized by adrenocorticotropic hormone-resistant adrenal insufficiency, alacrima and achalasia. In the past 5 years, POEM has proved highly successful in treating primary achalasia over the long term, but its long-term performance has not been certified by achalasia related to Triple A syndrome. Eckardt scores and esophageal manometry improved significantly during the 2 years following POEM; however, grade-A reflux esophagitis recurred in 66.7% of patients in 12 mo post-procedure.

CONCLUSION

Based on these case studies, POEM is efficacious and safe for a treatment of achalasia associated with Triple A syndrome.

Keywords: Per-oral endoscopic myotomy; Triple A syndrome; Achalasia; Case report

Core Tip: Per-oral endoscopic myotomy (POEM) has proved an ideal way to treat primary achalasia in both short- and long-term but there is little evidence for achalasia related to Triple A syndrome, a rare disease. Herein, we enrolled three patients and performed POEM on them. During the following 2 years, we evaluated them by Eckardt score, barium swallowing test and Esophagogastroduodenoscopy. As a result, they all improved in all aspects.