Spinal deformities in patients with Charcot-Marie-tooth disease. A review of 12 patients.

Twelve patients with Charcot-Marie-Tooth disease and scoliosis, seven girls and five boys (average age at presentation, ten years), were investigated for an average of 49 months. All but one of the patients were ambulatory. The curve patterns were thoracic in two, thoracolumbar in four, and double thoracic and lumbar curves in six. There was an associated kyphosis in six. Three patients had brace treatment with curve control in two. Four patients underwent posterior spinal fusion with Harrington instrumentation, one with segmental sublaminar wires. Post-operative management consisted of a cast for one patient and a brace for the remaining three. Follow-up period in the surgical patients ranged from 19 to 45 months (average, 32 months). One patient had a superior mesenteric artery syndrome and two had a pseudarthrosis.