Abstract
IntroductionPrevalence of scoliosis in Charcot-Marie-Tooth (CMT) disease has been reported to range from 10 and 38%. Our study has aimed to analyze prevalence of spinal deformities associated with CMT in our population and the epidemiological and clinical data of the patients. Materials and methodsA retrospective epidemiological study of 30 patients with CMT was performed. Patients were evaluated between January 2001 and December 2010. Sex, age at diagnosis and subtype of CMT, age at onset of scoliosis, curve characteristics, evolution and treatment were analyzed. ResultsA total of 46.7% of the children of the study had scoliosis. Average age at diagnosis of scoliosis was 10.21 years and average curve magnitude at the time of diagnosis was 13.85°. Of the curves, 42.85% were thoracolumbar and 50% were associated with increased thoracic kyphosis. Curve progression was analyzed only when the patients had been followed-up for more than three years and the final Risser sign was ≥3. Progression was documented in 50% of the patients. Orthosis was prescribed in five patients and none required surgical treatment. A higher incidence of scoliosis was observed in males, 78.5%, and in patients who had undergone foot surgery, 62.5%. No statistically significant relationship was found in either case. ConclusionsPrevalence of scoliosis in CMT is higher than in the general population. Patient have long radius curves, often with associated kyphosis. We did not find rapid progression and there was good response to the brace treatment. Spinal deformity should be detected early by systematic examination of the spine.
Published Version
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