Triple A to triple S: From diagnosis, to anesthetic management of Allgrove syndrome.

INTRODUCTION

Allgrove syndrome (AS) is a rare autosomal recessive disorder characterized by achalasia cardia, alacrimia, and adrenocorticotropic hormone-resistant adrenal insufficiency which is sometimes associated with autonomic dysfunction. It has also been referred to as the triple A syndrome in view of the cardinal symptoms described above. First described by Allgrove et al in 1978, the disorder usually presents mostly during the first decade of life. These patients have the threat of adrenal crisis, shock, and hypoglycemia and are usually on steroid supplementation.

CASE REPORT: The anesthesiologist's encounter with such patients, although rare, is mostly for repair of the achalasia cardia. We thus report a similar case of AS in a 2-year-old girl who was scheduled to undergo Heller myotomy along with the preoperative evaluation and intraoperative management of the same.

CONCLUSION

Being aware of the pathophysiology of AS gives useful insight about the disease and successful perioperative management in the form of the triple S strategy, that is, stress dose of steroids, slow induction and positioning, and finally maintenance of stable hemodynamics and euglycemia.