Year 2025 / Volume 117 / Number 12
Letter
Peroral endoscopic myotomy in pediatric triple A syndrome: report of three cases

803-804

DOI: 10.17235/reed.2025.11589/2025

Ali Çağatay Bozkına, Abdullah Murat Buyruk,

Abstract
Triple A syndrome (Allgrove syndrome) is a rare inherited disorder characterized by adrenal insufficiency, alacrima, and achalasia. Despite POEM’s established role in achalasia management, its effectiveness in Triple A syndrome remains largely undefined, as fewer than 10 cases have been reported. We present three genetically confirmed pediatric Triple A patients (two males, one female; 11-16 years) with long-standing achalasia, all with Eckardt scores of 12 on admission and successfully treated with POEM after failing conventional therapies.
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References
Allgrove J, Clayden GS, Grant DB, et al. Familial glucocorticoid deficiency with achalasia of the cardia and deficient tear production. Lancet. 1978;1(8077):1284–1286.
Khelif K, De Laet MH, Chaouachi B, et al. Achalasia of the cardia in Allgrove’s (triple A) syndrome: histopathologic study of 10 cases. Am J Surg Pathol. 2003;27(5):667–672.
Liu FC, Feng YL, Yang AM, et al. Triple A syndrome-related achalasia treated by per-oral endoscopic myotomy: Three case reports. World J Clin Cases. 2022;10(19):6529–6535.
Rizzo R, Balassone V, Torroni F, et al. Peroral endoscopic myotomy in a child with Triple A syndrome (Allgrove syndrome). VideoGIE. 2020;5(6):235–237.
Nakamura J, Hikichi T, Inoue H, et al. Per-oral endoscopic myotomy for esophageal achalasia in a case of Allgrove syndrome. Clin J Gastroenterol. 2018;11(4):273–277.
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Bozkına A, Buyruk A. Peroral endoscopic myotomy in pediatric triple A syndrome: report of three cases. 11589/2025


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Publication history

Received: 19/08/2025

Accepted: 12/09/2025

Online First: 29/09/2025

Published: 12/12/2025

Article Online First time: 41 days

Article editing time: 115 days


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