Skip to main content

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



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).


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


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°.


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.

This is a preview of subscription content, access via your institution.

Fig. 1
Fig. 2
Fig. 3

Availability of data and material

The data is available if requested.


  1. Kato K (1924) Congenital absence of the radius: with review of the literature and report of three cases. J Bone Joint Surg 6:589–626

    Google Scholar 

  2. Makley JT, Heiple KG (1970) Scoliosis associated with congenital deficiencies of the upper extremity. J Bone Joint Surg 52:279–287

    CAS  Article  Google Scholar 

  3. Powers TA, Haher TR, Devlin VJ, Spencer D, Millar EA (1983) Abnormalities of the spine in relation to congenital upper limb deficiencies. J Pediatr Orthop 3:471–474

    CAS  Article  Google Scholar 

  4. Lester DK, Painter GL, Berman AT, Skinner SR (1986) “Idiopathic” scoliosis associated with congenital upper-limb deficiency. Clin Orthop Relat Res 202:205–210

    Article  Google Scholar 

  5. Herring JA, Goldberg MJ (1985) Amelia and scoliosis. J Pediatr Orthop 5:605–609

    CAS  Article  Google Scholar 

  6. AlMarshad A, AlMazrua I, Al-Haidey R, AlZayed Z (2020) Functional outcomes in bilateral upper limb Amelia patient with scoliosis post vertical expandable prosthetic titanium rib (VEPTR) application: a case report. Int J Surg Case Rep 70:193–196

    Article  Google Scholar 

  7. Olgun ZD, Demirkiran G, Polly D Jr, Yazici M (2018) Congenital unilateral abense of the upper extremity may give rise to a specific kind of thoracolumbar curve. J Pediatr Orthop B 28:180–183

    Article  Google Scholar 

Download references


This study did not receive any funding.

Author information

Authors and Affiliations



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.

Ethics declarations

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.

Consent to participate

Informed consent was obtained from legal guardians.

Consent for publication

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

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Rights and permissions

Reprints and Permissions

About this article

Verify currency and authenticity via CrossMark

Cite this article

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).

Download citation

  • Received:

  • Accepted:

  • Published:

  • DOI:


  • Phocomelia
  • Amelia
  • Scoliosis
  • Vertebral body tethering