Challenge of the Week / Pediatrics / 11.19.2021

A 14-year-old male patient recently diagnosed with a defect in chromosome 9q13 related to the trinucleotide repeat GAA is referred for scoliosis evaluation. He walks with a slightly wide-based gait and has absent deep tendon reflexes. On examination, he has bilateral rigid cavovarus feet. Posteroanterior scoliosis radiographs reveal a major curve with a Cobb angle of 30°. Which of the following is the most appropriate treatment for his scoliosis?
    • Posterior spinal fusion with instrumentation
    • The description is that is that of Friedreich ataxia. Scoliosis is associated, but at 30° can be observed.
    • Growing rod spinal instrumentation with delayed spinal fusion
    • Growing rods are utilized for a younger age and a more severe curve.
    • Observation with possible bracing
    • The description is that is that of Friedreich ataxia. Scoliosis is associated, but at 30° can be observed and possibly braced.
    • Combined anterior/posterior spinal fusion with instrumentation
    • The description is that is that of Friedreich ataxia. Scoliosis is associated, but at 30° can be observed and possibly braced.

     

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