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An original preoperative orthopaedic management of a complex spinal deformity in a 16-year-old patient with spondyloepiphyseal dysplasia congenita

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Abstract

Purpose

Spinal deformities in patients with Spondyloepiphyseal Dysplasia congenita are particularly challenging to treat. Addressing these deformities requires a holistic surgical strategy.

The aim of this case report is to highlight an original preoperative protocol combining atlantoaxial instability stabilization by halo jacket with progressive correction of a thoracic kyphoscoliosis using continuous elongation by tension plaster cast as described by Stagnara.

Method

A 16-year-old patient with spondyloepiphyseal dysplasia congenita presenting a severe thoracic kyphoscoliosis associated with atlantoaxial instability causing cervical myelopathy was managed through a preoperative protocol combining a halo jacket with a Stagnara elongation cast allowing progressive correction of the kyphoscoliosis while stabilizing the cervical instability.

Results

The preoperative protocol allowed a safer and a more effective surgical spine fusion, reducing neurological risks, improving the final correction, and offering better postoperative recovery. Clinical and radiological solid fusion was observed at 3-year-follow-up with significant improvement of the patient’s quality of life.

Conclusion

This case report highlights the effectiveness of the preoperative preparation combining a halo jacket with a Stagnara elongation cast to safely stabilize the cervical spine while correcting the thoracic kyphoscoliosis. This strategy can serve as a valuable tool for spine teams when addressing complex spinal deformities particularly in this patient with SEDc.

Level of evidence

Level IV.

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Data availability

The data that support the findings of this study are available from the corresponding author, Toumia M.L, upon reasonable request.

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Funding

This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

Author information

Authors and Affiliations

  1. Department of Pediatric Orthopaedic Surgery, Children’s Hospital “Bechir HAMZA”, Medical School of Tunis, University Tunis-El Manar, Tunis, Tunisia

    Mohamed Laroussi Toumia, Ahmed Msakni, Ahmed Amine Mohseni, Mohamed Zairi, Sami Bouchoucha, Walid Saied, Mohamed Nabil Nessib & Rim Boussetta

Authors
  1. Mohamed Laroussi Toumia
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  2. Ahmed Msakni
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  3. Ahmed Amine Mohseni
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  4. Mohamed Zairi
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  5. Sami Bouchoucha
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  6. Walid Saied
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  7. Mohamed Nabil Nessib
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  8. Rim Boussetta
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Contributions

MLT: made substantial contributions to the conception of the work, drafted the original manuscript. AM: made substantial contributions to the acquisition, analysis, and interpretation of data. AAM: made substantial contributions to the acquisition, analysis, and interpretation of data. MZ: revised the article critically for important intellectual content. SB: revised the article critically for important intellectual content. WS: revised the article critically for important intellectual content. MNN: revised the article critically for important intellectual content. RB: agree to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved, approved the paper for publication.

Corresponding author

Correspondence to Mohamed Laroussi Toumia.

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Conflict of interest

The authors declare no competing interest.

Ethical approval

All procedures performed in this study involving human participants were in accordance with the ethical standards of the institutional and national research committees.

Informed consent

Informed consent was obtained from the patients involved in the study and his parents.

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Toumia, M.L., Msakni, A., Mohseni, A.A. et al. An original preoperative orthopaedic management of a complex spinal deformity in a 16-year-old patient with spondyloepiphyseal dysplasia congenita. Spine Deform (2023). https://doi.org/10.1007/s43390-023-00781-z

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  • DOI: https://doi.org/10.1007/s43390-023-00781-z

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