Introduction and Aims: Traditional treatment for adolescent hyperkyphosis, including Scheuermann’s disease, has included apical anterior spine release/fusion (ASF) prior to posterior instrumented fusion. We wished to reassess the need for ASF when using a posterior column shortening technique with a threaded rod compression instrumentation system.

Method: PSF-only group: 17 patients, mean age 15.7 years (range 12.8–18.5), underwent posterior column shortening by chevron-shaped lamina resection and fusion using hybrid hook/screw, dual 4.8mm threaded compression rod instrumentation (TRI). A/PSF group: seven patients, mean age 15.4 years (14–16.8), underwent open or endoscopic ASF followed by posterior TRI. Fourteen of 24 patients had strict Sorensen criteria of Scheuermann’s. At mean follow-up of 30 months (range 24–56), patients were assessed for amount and maintenance of correction, sagittal balance, and evidence of pseudoarthrosis.

Results: Mean pre-operative measured kyphosis in PSF-only was 79.6 degrees (range 67–90), and was corrected to 38.2 degrees (22–55) post-operative, and 37.1 degrees (22–50, 53%) at final follow-up. In the A/PSF group, pre-operative kyphosis was 79.0 degrees (62–93), corrected to 41.6 degrees (34–48) post-operative, and was 42.6 degrees (25–48, 46%) at final follow-up. There was no difference in the amount of correction (p=.28) or its maintenance between the two groups. Similarly, there were no differences between groups in assessing pre-operative and final T2-12 kyphosis (p=.13), T12-S1 lordosis (p=.98), or C7 sagittal balance (p=.10). The mean T10-L2 sagittal alignment was improved in the PSF-only patients (final kyphosis 7.8°) vs. 18.9° in the A/PSF patients (p=.04). There was a greater correction of Voutsinas’ index (PSF-only=.08, A/PSF=.15, p=.01) for the posterior-only group. No patient lost > eight degrees correction (range 12–8) between immediate post-operative and final follow-up, and no instrumentation complications occurred.

Conclusion: Using posterior column shortening and larger diameter (4.8mm) threaded rods with hooks cephalad to the apex and screws caudal, we have found no advantage in correction from preliminary anterior apical release, nor a difference in maintenance of correction. ASF is unnecessary when adolescent hyperkyphosis/Scheuermann’s is treated by this technique.

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.