Hamza Kassimi, Larbi Ed-dafali, Alae Alae El koraichi, Salma Ech Cherif El Kettani, Aziza Bentalha
Journal: Medpeer Publisher
ISSN: 3066-2737
Volume: 2
Issue: 10
Date of Publication: 2025/10/16
Allgrove syndrome, also known as triple A syndrome, is a rare genetic disorder characterized by adrenal insufficiency, achalasia, and alacrima, with potential neurological and autonomic complications. This complex clinical triad poses a significant anesthetic challenge, requiring meticulous preparation that integrates the pathophysiological specificities of the disease to ensure perioperative patient safety.
We report the case of a 9-year-old patient with Allgrove syndrome who underwent Heller's myotomy for refractory achalasia. Perioperative management included hydrocortisone replacement therapy, rapid sequence induction to reduce the risk of pulmonary aspiration, strict glycemic monitoring, and ocular protection to prevent complications related to alacrima.
adrenal insufficiency, achalasia, perioperative management, triple a syndrome, allgrove syndrome
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