Triple A syndrome-related achalasia treated by per-oral endoscopic myotomy: Three case reports.

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.

• Authors: Feng-Chen Liu, Yun-Lu Feng, Ai-Ming Yang, Tao Guo
• Journal: World journal of clinical cases (World J Clin Cases) Vol. 10 Issue 19 Pg. 6529-6535 (Jul 06 2022) ISSN: 2307-8960 [Print] United States
• PMID: 35979302 (Publication Type: Case Reports)
• Copyright: ©The Author(s) 2022. Published by Baishideng Publishing Group Inc. All rights reserved.