SPINAL DEFORMITY IN CHARCOT-MARIE-TOOTH DISEASE

Study Design. This retrospective study reviewed 100 children with clin ically and electrodiagnostically proven Charcot-Marie-Tooth disease (C MTD). Objective. To determine the incidence of spinal deformity in chi ldren with CMTD. Summary of Background Data. A 10% incidence of kyphos coliosis has been reported. Methods. All charts and electrodiagnostic studies were reviewed to confirm that CMTD diagnostic criteria were me t. Existing radiographs on 89 children were available. Cobb angles wer e measured and deformity was defined as scoliosis greater-than-or-equa l-to 10-degrees and kyphosis >40-degrees. Results. Thirty-seven of 89 CMTD children had spinal deformity. There was scoliosis in 20, kyphosc oliosis in 14, and kyphosis in 3. In children with radiographs taken a t maturity, 50% had deformity. The most common scoliotic pattern was a thoracic curve with convexity in either direction. Spinal deformity i s more likely in female and Type I patients. Conclusions. This study f ound an incidence of 37%-50% spinal deformity in children with CMTD, w ith female and Type I patients at greatest risk. However, the deformit y rarely required treatment.