Early-Onset Spinal Deformity in Skeletal Dysplasias: A Multicenter Study of Growth-Friendly Systems

Spine Deform. Jul-Aug 2018;6(4):478-482. doi: 10.1016/j.jspd.2018.01.002.


Purpose: Severe, early-onset spinal deformity is common in patients with skeletal dysplasia. These deformities often present at young ages and are associated with significant pulmonary dysfunction. The objective of this study is to verify the effectiveness of growth-friendly spinal instrumentation systems in promoting growth in patients with skeletal dysplasia and early-onset kyphoscoliosis.

Methods: A retrospective, multicenter comparative cohort study was performed. Twenty-three patients identified to have a skeletal dysplasia (SKD) were evaluated for diagnosis, age at treatment, gender, and type of growing rod construct (spine vs. rib constructs). Patients were matched by age and construct type with similarly treated patients with early-onset scoliosis (CON) without skeletal dysplasia. Radiographic parameters including maximum coronal and sagittal Cobb angle with levels, T1-S1 height, and T1-T12 height were measured.

Results: T1-T12 (12.8 vs. 15.2 cm, p = .01) and T1-S1 (21.2 vs. 24.5 cm, p = .05) heights were significantly shorter for the SKD group at implantation, and kyphosis tended to be more severe in children with SKD (p = .80 and .07, respectively). Kyphosis did not improve with treatment. Scoliosis improved (p < .01), and ΔT1-T12 and ΔT1-S1 significantly increased in both groups (p < .01). Complication rates were similar between the two groups; however, patients with SKD had more intraoperative monitoring changes and hardware failures (p < .005).

Conclusion: Although patients with SKD start with shorter spine lengths, gains in spine length appear to be comparable to other forms of EOS. Neuromonitoring changes and implant failures are more common in the SKD group.

Significance: The effectiveness of growth-friendly techniques in promoting growth in early-onset spinal deformities in patients with skeletal dysplasia has not been previously studied. We report the first comprehensive review of this topic. Growth-friendly techniques are an appropriate treatment option in this patient population.

Keywords: Early-onset scoliosis; Growing rods; Kyphosis; Skeletal dysplasia.

Publication types

  • Comparative Study
  • Multicenter Study

MeSH terms

  • Bone Diseases, Developmental / therapy*
  • Child
  • Child Development
  • Child, Preschool
  • Female
  • Humans
  • Male
  • Orthopedic Procedures / instrumentation*
  • Retrospective Studies
  • Spine / abnormalities*
  • Spine / growth & development