Spinal surgery in spinal muscular atrophy

Fifteen patients with surgical treatment of spinal muscular atrophy were reviewed. The curve pattern was thoracic in 3, thoracolumbar in 11, and double thoracic and thoracolumbar in 1. Follow-up averaged 31 months. Eleven patients underwent posterior spinal fusion with Harrington instrumentation, with segmental wiring in four, and two had Luque instrumentation. The average age at time of surgery was 14.4 years. The average curve correction was 48%; that with the pelvic obliquity corrected, 63%. Surgery is best done when the curve is approximately 50-60 degrees, and Luque sublaminar wiring of Harrington or Luque rods with no external support appears to be the procedure of choice.