Acromegaly, Endocrine Dysfunction and Polyostotic Fibrous Dysplasia Associated With Mccune-Albright Syndrome: Anesthetic Considerations
Journal Title: International Journal of Anesthesiology & Research (IJAR) - Year 2014, Vol 2, Issue 4
Abstract
McCune-Albright Syndrome is a rare disorder affecting the skin, bones and endocrine tissues. It is characterized by the presence of café au lait spots, polyostotic fibrous dysplasia, and autonomous endocrine hyperfunction. Perioperative management of patients with this syndrome is frequently complex, as they are prone to a large array of comorbidities including pathologic fractures and spinal instability, acromegaly, hyperthyroidism, hypophosphatemia, Cushing’s syndrome and chronic systemic hypertension, liver disease, and arrythmias. Anesthesiologists are faced with a spectrum of challenges in the perioperative management of these patients. Macroglossia and macrognathia can result in airways which are difficult to visualise and secure. Frail bones and spinal instability often complicate positioning, and endocrinopathies may result in an increased propensity for cardiac arrhythmias perioperatively. The objective of this paper is to report the case of a 36 year-old male with McCune-Albright Syndrome presenting for a T10-11 laminectomy due to spinal cord compression and to discuss our management of this complicated patient with a history of hypophosphatemia, scoliosis, multiple long bone fractures, non-ischemic cardiomyopathy, atrial fibrillation, and severe acromegaly from a growth hormone-secreting pituitary adenoma.
Authors and Affiliations
Kenneth Moran
Keywords
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- DOI 10.19070/2332-2780-1400012
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