Perioperative Management of an Adolescent With Friedreichs Ataxia and Hypertrophic Cardiomyopathy

Mineto Kamata, David Martin, Allan Beebe, Walter Samora, Aymen Naguib, Joseph D. Tobias


Friedreichs ataxia (FA) is an autosomal recessive neurodegenerative disorder with an incidence of 1:50,000 which usually presents during adolescents. Given the progressive neurologic involvement, surgical intervention is frequently required for correction of orthopedic sequelae including scoliosis. Associated co-morbid conditions including hypertrophic cardiomyopathy, restrictive lung disease, and diabetes mellitus may impact the perioperative care of such patients. We present a 14-year-old girl with FA presenting for posterior spinal fusion for the treatment of scoliosis. The perioperative management of such patients is discussed.

J Med Cases. 2014;5(9):473-477


Friedreich’s ataxia; Hypertrophic cardiomyopathy; Posterior spinal fusion; Scoliosis

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