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Congenital cervicothoracic dissociation: report of two cases

Abstract

Purpose

Congenital cervicothoracic dissociation (CCTD) of the spine is a rare condition while having major impacts on stability and neurological function. Surgical treatment includes decompression and instrumented fusion. Only few cases of CCTD have been reported in children. This report intends to demonstrate the complexity of this condition and its surgical management options based on two cases.

Methods

Retrospective illustration of two cases with CCTD treated with instrumented occipito-thoracic fusion. Timing and options of surgical management are discussed.

Results

Two patients aged 9 and 12 were treated. Patient 1 presented with asymmetric chronic weakness of the upper extremity and unstable neurogenic bladder. Imaging showed a CCTD with severe distortion of the posterior facets and a dysplastic spinal cord. Patient 2 presented with mild spasticity and unilateral weakness of the upper extremity. A low energy trauma resulted in tetraplegia by increasing the amount of antero-posterior displacement. Both patients were treated with Halo vest application, followed by posterior decompression, and instrumented occipito-thoracic fusion. Additional upfront Halo traction was applied in patient 2 for distraction and sagittal alignment improvement. The last follow-up confirmed solid fusion and minor residual neurological impairment in both patients at 5, and 6 years, respectively.

Conclusion

CCTD is a rare congenital condition, which can be successfully managed by posterior instrumented fusion. A sudden cervicothoracic displacement with neurological deterioration may respond to careful Halo traction with preoperative sagittal alignment correction. Timing of surgery depends on the actual instability in neurologically stable patients.

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Abbreviations

CD:

Congenital dislocation

CT:

Computed tomography

CCTD:

Congenital cervicothoracic dissociation

FU:

Follow-up

MRI:

Magnetic resonance imaging

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Acknowledgements

We would like to thank our neuromonitoring and orthotics team for their cooperation and support in the clinical management of the reported patients.

Funding

No funding was obtained for the study.

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Authors and Affiliations

Authors

Contributions

LNL: made substantial contributions to the acquisition of data; drafted the work, approved the version to be published; and agreed 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. JD: made substantial contributions to the conception or design of the work; revised it critically for important intellectual content; approved the version to be published; and agrees 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. RZ: made substantial contributions to the conception or design of the work; revised it critically for important intellectual content; approved the version to be published; and agrees 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.

Corresponding author

Correspondence to Laura Nanna Lohkamp.

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None of the authors has conflicts of interests or competing interests.

Compliance with ethical standards

The study was conducted in accordance with the guidelines of the ethics committee (REB) at The Hospital for Sick Children. Informed consent was waived due to the retrospective, observational format of the study.

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Lohkamp, L.N., Drake, J. & Zeller, R.D. Congenital cervicothoracic dissociation: report of two cases. Spine Deform (2022). https://doi.org/10.1007/s43390-022-00581-x

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Keywords

  • Cervicothoracic dissociation
  • Posterior cervicothoracic fusion
  • Pediatric
  • Bone graft
  • Autologous