Surgical Treatment of Scoliosis in Patients with Charcot-Marie-Tooth Disease

Objective: Apparently, scoliosis occurs in approximately one-third of patients with Charcot-Marie-Tooth disease. Little is known about the response of these curves to treatment. The purpose of this study was to evaluate the results of spinal surgery in these peculiar patients. Methods: We retrospectively evaluated the results of spinal surgery in eight patients who had scoliosis due to clinically and electrophysiologically proven Charcot-Marie-Tooth disease. Radiographs were reviewed. The location and direction of the curve pattern, the age at the time of surgery, type of surgery, number of levels fused, instrumentations used, intra or postoperative complications, and results and need for reoperation were recorded. Results: Eight patients associated with Charcot-Marie-Tooth disease who underwent scoliotic surgery were identified. The average age and curve at the time of surgery were 21.1 years and 56.4° respectively. 62.5% of the curves had left thoracic component and more than one third was associated with thoracic hyperkyphosis. Long posterior spinal fusion was performed most often, with an average of 11.5 spinal segments fused. Instrumentation was used in all posterior fusions. At an average of 39 months (range, 24 to 72 months) postoperatively, the fusion appeared to be solid in all patients. Conclusion: Scoliosis in patients with Charcot-Marie-Tooth disease differs from that in patients with idiopathic scoliosis in regarding to the etiology and the prevalence of thoracic hyperkyphosis, but the surgical management appears to be similar. Spondylodesis does not appear to be associated with a high rate of complications.