Surgical treatment of scoliosis in a spinal muscular atrophy population.

Seventy-eight patients were diagnosed with spinal muscular atrophy between 1969 and 1988. Scoliosis developed in 34 of these patients, an incidence of 60%. Thirty-one patients could be retrospectively reviewed by chart review or interview. The average follow-up was 11.5 years. Onset of scoliosis averaged 8.8 years. Twenty-two patients were treated nonsurgically and nine surgically. Patients had improved sitting balance and endurance after surgery. Complications of surgery included loss of correction in one patient, one pseudarthrosis, and one patient who required prolonged ventilatory support. The prolonged survival of patients with spinal muscular atrophy justifies aggressive orthopaedic management of scoliosis to prevent progression of deformity and improve sitting comfort.