Reconstruction of the anterior craniocervical junction using an expandable cage after resection of a C1 chordoma in a 5-year-old child: case report

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Chordomas are histologically benign tumors with local aggressive behavior. They arise from embryological remnants of the notochord at the clivus, mobile spine, and sacrum. Chordomas are rare tumors in the pediatric age group. Their surgical management is difficult, given their propensity for inaccessible anatomical regions, and proximity to critical neurovascular structures. While en bloc resection with surgical margins has been advocated as the preferred approach for chordomas, tumor characteristics and violation of adjacent anatomical boundaries may not allow for safe en bloc resection of the tumor. Here, the authors present the case of a C1 chordoma in a 5-year-old boy with epidural and prevertebral extension. The patient’s treatment consisted of a far-lateral approach for resection of the tumor and C1 arch, followed by circumferential reconstruction of the craniocervical junction with an expandable cage spanning the skull base to C2, and posterior occipitocervical spinal instrumentation. At 42 months after surgery, the patient remains neurologically intact with stable oncological status, and no evidence of craniocervical junction instrumentation failure.

Article Information

Correspondence Andrew Jea: Indiana University School of Medicine, Riley Hospital for Children, Indianapolis, IN.

INCLUDE WHEN CITING Published online April 12, 2019; DOI: 10.3171/2019.2.PEDS18752.

Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.

© AANS, except where prohibited by US copyright law.



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    Axial (A) and sagittal (B) MR images with gadolinium of the craniocervical junction at the level of C1, demonstrating a large extraaxial mass with significant spinal cord compression at the cervicomedullary junction.

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    Axial (A) and coronal (B) MR images with gadolinium of the craniocervical junction at the level of C1, demonstrating a near-total resection with stable residual disease in the left anterolateral neck (white arrows) at 32 months after surgery.

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    Coronal (A) and sagittal (B) CT scans of the cervical spine obtained 42 months after surgery, demonstrating arthrodesis between the occiput and cervical spine with chronic remodeling of the bone at the craniocervical junction. There is no evidence of failure of the spinal instrumentation. The expandable cage remains in place, spanning the base of the skull and the C2 lateral mass, and the alignment at the craniocervical junction is maintained.

  • View in gallery

    Flexion (A) and extension (B) cervical spine radiographs showing no evidence of spinal instrumentation failure, loss of spinal alignment, or instability.


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