Perioperative Management of an Adolescent With Friedreich’s Ataxia and Hypertrophic Cardiomyopathy

Abstract

Friedreich's 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.