Anesthetic challenges of Allgrove’s triple A syndrome

Abstract

Allgrove’s Triple A syndrome is an autosomal recessive disorder characterized by the triad of primary adrenal insufficiency, achalasia cardia and alacrima. The perioperative period poses significant anesthetic challenges due to the risk of adrenal crisis and aspiration related to achalasia. The possible presence of autonomic dysfunction (4A syndrome) further complicates the anesthetic management. Here we describe the successful perioperative management of an adult with Allgrove syndrome, highlighting the importance of stress-dose corticosteroids and meticulous perioperative care.