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Extensive cervicothoracic posterior arch defects from C1 to T6 leading to myelopathy due to thoracic kyphosis in an adolescent boy

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Abstract

Purpose

Limited dorsal myeloschisis, a form of cervical spinal dysraphism, is a rare anomaly and is typically associated with spinal cord tethering. The objective is to illustrate a rare dysraphic anomaly in the cervicothoracic spine causing myelopathy, not due to tethering but secondary to progressive kyphosis. To our knowledge, such an anomaly has not been described in the literature.

Study design

Case report

Methods

A 16-year-old boy presented with lower extremity spastic paraparesis due to progressive cervicothoracic deformity. The imaging studies revealed extensive posterior arch defects from C1 to T6. The cervical spinal cord and meninges had herniated out of the spinal canal in the hyperlordotic cervical spine, and the thoracic spinal cord was stretched and compressed over the T4/5 kyphotic apex. Free-floating spinous processes were found compressing the cord at the T4-5 level. Tethering was not detected.

Results

The patient underwent a posterior vertebral column resection at T5 and excision of the free-floating spinous processes. The patient made a complete neurological recovery. At 8 year follow-up, he was asymptomatic and his deformity was stable.

Conclusion

We present a rare congenital cervical dystrophic anomaly causing myelopathy secondary to progressive kyphosis. We speculate that this anomaly was due to the sclerotomal cells’ failure to migrate dorsally to the neural tube and fuse in the midline.

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Funding

No funding was received for this work. This manuscript has not been previously published or presented, in whole or in part, or submitted elsewhere for review.

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Authors and Affiliations

  1. Department of Orthopaedics, Lokmanya Tilak Municipal General Hospital and Lokmanya Tilak Municipal Medical College, Sion, Mumbai, India

    Ashok Rathod

  2. Department of Orthopaedics, Aneurin Bevan University Health Board, Newport, UK

    Rishi Aggarwal

  3. Department of Orthopaedics, King Edward VII Memorial Hospital and Seth GS Medical College, Parel, Mumbai, India

    Vinod J. Laheri

  4. Department of Orthopaedics, PD Hinduja Hospital and Medical Research Center, Mumbai, Maharashtra, India

    Kshitij Chaudhary

Authors
  1. Ashok Rathod
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  2. Rishi Aggarwal
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  3. Vinod J. Laheri
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  4. Kshitij Chaudhary
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Contributions

AR: conception, acquisition and analysis of data, drafted the work and revised the draft critically, approved the version to be published, accountable for all aspects of the work. RA: acquisition and analysis of data, drafted the work, approved the version to be published, accountable for all aspects of the work. VL: analysis and interpretation of data, revised the draft critically, approved the version to be published, accountable for all aspects of the work. KC: acquisition of data, analysis and, interpretation of data, drafted the report and revised the draft critically, approved the version to be published, accountable for all aspects of the work.

Corresponding author

Correspondence to Kshitij Chaudhary.

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The authors have no competing or conflicting interests.

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Consent of the patient was obtained for publishing this case report.

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Supplementary Information

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Supplementary file1 Supplemental Video File: Intraoperative video of T5 posterior vertebral column resection. (MP4 20425 KB)

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Rathod, A., Aggarwal, R., Laheri, V.J. et al. Extensive cervicothoracic posterior arch defects from C1 to T6 leading to myelopathy due to thoracic kyphosis in an adolescent boy. Spine Deform 10, 189–195 (2022). https://doi.org/10.1007/s43390-021-00383-7

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  • DOI: https://doi.org/10.1007/s43390-021-00383-7

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