Abstract
Introduction and Aims: While scoliosis is known to be associated with Charcot-Marie-Tooth disease, little is known about the response to treatment of spinal deformity in this population. The purpose of this study was to characterise scoliosis in CMT, and to assess the effect of bracing and the efficacy and safety of surgery.
Method: A retrospective review of medical records and radiographs of patients with CMT from a major neuro-muscular clinic was performed to calculate the prevalence of scoliosis and to characterise the deformity in affected patients. Orthotic and operative records were reviewed in patients who were braced and/or had spinal fusions.
Results: Forty-three of 271 patients with CMT had scoliosis, for a prevalence of 15.9%. There were 18 females and 25 males, and the age at diagnosis of scoliosis averaged 12.7 years (range 7.8–17.8 years). Thirty-one of 43 curves were in the thoracic spine, with 15 curves being left thoracic. Curve magnitude at diagnosis averaged 27.8 degrees (11–65 degrees), and 18 of 34 curves with available lateral radiographs had hyperkyphosis.
Curve progression of more than five degrees was present in 67.9% of those curves with follow-up. All five non-ambulatory patients progressed and had surgery. Bracing was prescribed in 39.5% of patients, and 11 of 15 braced patients progressed and had surgery.
Surgery was scheduled in 32.6% of patients. The average age at surgery was 13.8 years (11.5–15.8 years), and curve magnitude averaged 63.1 degrees (50–80 degrees), with 78.6% of surgical curves being kyphotic. Posterior spinal fusion was performed in 11, anterior/posterior fusion in one, and halo traction followed by posterior spinal fusion in one. All curves were instrumented. Curve correction averaged 51.7%. Intra-operative neurologic monitoring (SSEP’s +/− MEP’s) was successful in only three of 11 patients. No neurologic complications occurred. One re-operation for delayed infection was necessary.
Conclusion: Scoliosis occurs in 15.9% of CMT patients. It is associated with thoracic kyphosis and an increased incidence of left thoracic curves. Bracing is usually unsuccessful. Surgery was necessary in 32.6% overall, and 100% of non-ambulators who had scoliosis. Instrumentation was safe and effective, but intra-operative neurologic monitoring is usually impossible.
These abstracts were prepared by Editorial Secretary, George Sikorski. Correspondence should be addressed to Australian Orthopaedic Association, Ground Floor, The William Bland Centre, 229 Macquarie Street, Sydney, NSW 2000, Australia.
One or more of the authors are receiving or have received material benefits or support from a commercial source.
