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Spinal deformity in Charcot-Marie-Tooth disease
J L Walker1, K R Nelson, D B Stevens
1Shriners Hospitals for Crippled Children, Lexington, Kentucky.
Spine
|May 1, 1994
Summary
Spinal deformity affects up to 50% of children with Charcot-Marie-Tooth disease (CMTD), particularly females and Type I patients. However, this deformity rarely necessitates medical intervention.
Area of Science:
- Neurology
- Orthopedics
- Pediatrics
Background:
- Spinal deformity, specifically kyphoscoliosis, is a known complication of Charcot-Marie-Tooth disease (CMTD).
- Previous reports indicated a 10% incidence of kyphoscoliosis in children with CMTD.
Purpose of the Study:
- To ascertain the prevalence of spinal deformities in pediatric patients diagnosed with Charcot-Marie-Tooth disease.
- To identify risk factors associated with spinal deformity in this population.
Main Methods:
- Retrospective analysis of 100 children with clinically and electrodiagnostically confirmed Charcot-Marie-Tooth disease (CMTD).
- Review of medical records and electrodiagnostic studies to verify CMTD diagnosis.
- Radiographic assessment of 89 children, measuring Cobb angles to define scoliosis (≥10 degrees) and kyphosis (>40 degrees).
Main Results:
- A significant incidence of spinal deformity was observed in 37% (33/89) of the studied children.
- Scoliosis was present in 20 children, kyphoscoliosis in 14, and kyphosis in 3.
- Spinal deformity rates reached 50% in patients whose radiographs were taken at skeletal maturity, with a higher likelihood in females and Type I CMTD patients.
Conclusions:
- The incidence of spinal deformity in children with Charcot-Marie-Tooth disease ranges from 37% to 50%.
- Female gender and Type I CMTD are identified as risk factors for developing spinal deformity.
- Despite the high incidence, spinal deformities in this cohort rarely required treatment.