title: Triple A to triple S: From diagnosis, to anesthetic management of Allgrove syndrome.
abstract: BACKGROUND
  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.
  
  
  METHODS
  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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department of Anaesthesiology and Critical Care, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India. Electronic address: mriduldhar@hotmail.com.
authors.forename: Mridul
authors.initials: M
authors.lastname: Dhar
authors.affiliation: Department of Anaesthesiology and Critical Care, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India.
authors.forename: Nimisha
authors.initials: N
authors.lastname: Verma
authors.affiliation: Department of Anaesthesiology and Critical Care, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India.
authors.forename: Ram Badan
authors.initials: RB
authors.lastname: Singh
authors.affiliation: Department of Anaesthesiology and Critical Care, Institute of Medical Sciences, Banaras Hindu University, Varanasi, Uttar Pradesh, India.
authors.forename: Vishal Krishna
authors.initials: VK
authors.lastname: Pai
mesh_terms: D000309:Adrenal Insufficiency / Q000175:diagnosis* / Q000601:surgery*; D000758:Anesthesia; D006348:Cardiac Surgical Procedures; D003680:Deglutition Disorders / Q000150:complications; D004931:Esophageal Achalasia / Q000175:diagnosis* / Q000601:surgery*; D005260:Female; D006330:Heart Defects, Congenital / Q000601:surgery; D006801:Humans; D007223:Infant; D019990:Perioperative Care; D010859:Pigmentation Disorders / Q000150:complications
keywords: Achalasia; Adrenal crisis; Alacrimia; Allgrove syndrome; Steroids