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

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Abstract

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.

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Author information

Authors and Affiliations

  1. Department of Orthopedics and Sports Medicine, Seattle Children’s Hospital, 4800 Sand Point Way OA.9.120, 98105, Seattle, WA, USA

    Klane K. White MD, MSc, Viviana Bompadre PhD & Walter F. Krengel III MD

  2. Department of Orthopaedics and Sports Medicine, University of Washington, 1959 NE Pacific St, 98195, Seattle, WA, USA

    Klane K. White MD, MSc & Walter F. Krengel III MD

  3. Department of Orthopedic Surgery, Nemours Alfred I. DuPont Hospital for Children, 1600 Rockland Rd, 19803, Wilmington, DE, USA

    Suken A. Shah MD & William G. Mackenzie MD

  4. Division of Pulmonary and Sleep Medicine, Seattle Children’s Hospital, 4800 Sand Point Way, OC.7.720, 98105, Seattle, WA, USA

    Gregory J. Redding MD

Authors
  1. Klane K. White MD, MSc
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  2. Viviana Bompadre PhD
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  3. Suken A. Shah MD
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  4. Gregory J. Redding MD
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  5. Walter F. Krengel III MD
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  6. William G. Mackenzie MD
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Consortia

Children’s Spine Study Group, Growing Spine Study Group

Corresponding author

Correspondence to Klane K. White MD, MSc.

Additional information

IRB approval: This study was reviewed and approved by Seattle Children’s Research Institute IRB ID# PIROSTUDY14864.

Author disclosures: KKW (none), VB (none), SAS (none), GJR (none), WFK III (none), WGM (none), Children’s Spine Study Group, Growing Spine Study Group.

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White, K.K., Bompadre, V., Shah, S.A. et al. Early-Onset Spinal Deformity in Skeletal Dysplasias: A Multicenter Study of Growth-Friendly Systems. Spine Deform 6, 478–482 (2018). https://doi.org/10.1016/j.jspd.2018.01.002

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  • DOI: https://doi.org/10.1016/j.jspd.2018.01.002

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