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Case report: vertebral body tethering for idiopathic scoliosis in a patient with bilateral phocomelia

Abstract

Purpose

The purpose of this study is to present a case report of a patient with bilateral upper extremity phocomelia with progressive scoliosis, who underwent vertebral body tethering (VBT).

Methods

This is a case report on the use of VBT in a patient with scoliosis and bilateral congenital phocomelia, with 5 year follow-up.

Results

A male patient with bilateral phocomelia had early onset scoliosis that progressed to 45° at age 10. Surgical options were discussed, including traditional VBT, posterior spinal fusion, growing rods, magnetically controlled growing rods, and vertical expandible prosthetic titanium ribs. These options would limit the flexibility of the spine. Given these pitfalls, VBT was chosen, as it would address the scoliosis while maintaining trunk flexibility. Preoperatively, he had 45° right main thoracic curve, bending to 22°; he was Risser 0 with open triradiate cartilage. He underwent T6-T11 thoracoscopic VBT, with postoperative correction to 37°. Postoperatively, the patient was able to continue to use his lower extremities for writing, feeding, and personal grooming. He had no postoperative complications. At 3 years, his curve was 21°, and at 5 years was 19°.

Conclusion

This case describes a novel technique for treating scoliosis in patients with bilateral phocomelia. Other forms of scoliosis surgical treatment limit motion of the spine. Due to this, we present VBT as an option for this unique set of patients for correcting scoliosis, while also preserving trunk flexibility for its role in feeding and self-care.

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Availability of data and material

The data is available if requested.

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Funding

This study did not receive any funding.

Author information

Authors and Affiliations

Authors

Contributions

JHH, SLM, ASS, PJC: Conception of design or work; JHH, GF, SLM: Data Collection; JHH, GF: Writing-Original Draft Preparation; JHH, GF, SLM, ASS, PJC: Revision of work; JHH, GF, SLM, ASS, PJC: Approval of final version of manuscript; JHH, GF, SLM, ASS, PJC: Agree to be accountable for the work.

Corresponding author

Correspondence to Jessica H. Heyer.

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Conflicts of interest

The authors have no conflicts of interest to declare that are relevant to the content of this article.

Ethics approvals

Patient/parent consent was obtained for this case report. IRB approval was not required, as it is an observational study.

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Informed consent was obtained from legal guardians.

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The participant has consented to the submission of the case report to the journal.

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Heyer, J.H., Fryhofer, G.W., Mitchell, S.L. et al. Case report: vertebral body tethering for idiopathic scoliosis in a patient with bilateral phocomelia. Spine Deform (2022). https://doi.org/10.1007/s43390-022-00562-0

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  • DOI: https://doi.org/10.1007/s43390-022-00562-0

Keywords

  • Phocomelia
  • Amelia
  • Scoliosis
  • Vertebral body tethering