Anesthesia Management of a Patient Having Allgrove Syndrome

Background: Allgrove syndrome is an autosomal recessive disorder characterized by achalasia cardia, alacrimia and adrenocorticotropic hormone resistant adrenal insufficiency with progressive neurological impairment with or without mental retardation. Materials and Methods: Patient had undergone Heller’s myomectomy under GA. PAC was done, all routine investigations, oesophageal motility tests, ACTH level, Schirmer test was done. She was pre-medicated with glycopyrrolate, midazolam and induced with fentanyl, propofol, vecuronium, intubated with cuffed ET tube 6.5 and reversed with Neostigmine and glycopyrrolate. Result and Conclusion: Careful pre-operative evaluation, choice of sedatives, induction agents, muscle relaxant to avoid hyperventilation, hypokalemia, close monitoring of ABG and electrolytes, careful glycaemic control, eye protection, care to avoid aspiration and regurgitation are imperative for optimal management with Allgrove syndrome. The co-ordination of care for these patients require a multidisciplinary team of endocrinologist, neurologist, gasteroenterologist, ophthalmologist, surgeons and anaesthesiologists. We successfully conducted this case of 22 yrs old female with ALLGROVE SYNDROME.