Background: Scoliosis appears to occur 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 establish the
prevalence of scoliosis in a large population of children and adolescents with
Charcot-Marie-Tooth disease, to evaluate factors linked with curve
progression, and to assess the response to orthotic and surgical
treatment.
Methods: The medical records of 298 patients were retrospectively
reviewed. Radiographs were reviewed for patients identified as having spinal
deformity. The type, size, and progression of the scoliotic curve were
measured, and the effectiveness of bracing and surgical treatment was
assessed.
Results: Forty-five patients with scoliosis associated with
Charcot-Marie-Tooth disease were identified. The average age at the diagnosis
of the spinal deformity was 12.9 years, and the average curve magnitude at the
time of diagnosis was 27.6°. One-third of the curves were left thoracic,
and 49% were associated with increased thoracic kyphosis. Twenty-four of the
thirty-four curves that were followed for more than one year progressed. Brace
treatment was successful in only three of sixteen patients. Surgery was
performed in fourteen of the forty-five patients. Long posterior spinal
fusions were performed most often, with an average of 13.1 spinal segments
fused. Instrumentation was used in all posterior fusions. Intraoperative
neurologic monitoring was possible for only three of the twelve patients for
whom it was attempted during surgery, but there were no intraoperative
neurologic complications.
Conclusions: Scoliosis in patients with Charcot-Marie-Tooth disease
differs from that in patients with idiopathic scoliosis. Thoracic
hyperkyphosis is common, and bracing is usually unsuccessful. Surgical fusion
does not appear to be associated with a high rate of complications, although
it is often impossible to perform intraoperative neurologic monitoring.
Level of Evidence: Therapeutic Level IV. See Instructions
to Authors for a complete description of levels of evidence.