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

Restricted access

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. ajea@goodmancampbell.com.

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.

Headings

Figures

  • View in gallery

    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.

  • View in gallery

    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.

  • View in gallery

    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.

References

  • 1

    Bailey CSFisher CGBoyd MCDvorak MF: En bloc marginal excision of a multilevel cervical chordoma. Case report. J Neurosurg Spine 4:4094142006

  • 2

    Barrenechea IJPerin NITriana ALesser JCostantino PSen C: Surgical management of chordomas of the cervical spine. J Neurosurg Spine 6:3984062007

  • 3

    Guppy KHChakrabarti IIsaacs RSJun JH: En bloc resection of a multilevel high-cervical chordoma involving C-2: new operative modalities: technical note. J Neurosurg Spine 19:2322422013

  • 4

    Hsieh PCGallia GLSciubba DMBydon AMarco RARhines L: En bloc excisions of chordomas in the cervical spine: review of five consecutive cases with more than 4-year follow-up. Spine (Phila Pa 1976) 36:E1581E15872011

  • 5

    Jeszenszky DFekete TFMelcher RHarms J: C2 prosthesis: anterior upper cervical fixation device to reconstruct the second cervical vertebra. Eur Spine J 16:169517002007

  • 6

    McMaster MLGoldstein AMBromley CMIshibe NParry DM: Chordoma: incidence and survival patterns in the United States, 1973-1995. Cancer Causes Control 12:1112001

  • 7

    Neil JAGarber STDailey ATCouldwell WT: Management of complex pediatric chordoma: transnasal and bilateral far-lateral approach for resection with O-C4 fusion: 3-dimensional operative video. Oper Neurosurg (Hagerstown) 12:3922016

  • 8

    Ortega-Porcayo LACabrera-Aldana EEArriada-Mendicoa NGómez-Amador JLGranados-García MBarges-Coll J: Operative technique for en bloc resection of upper cervical chordomas: extended transoral transmandibular approach and multilevel reconstruction. Asian Spine J 8:8208262014

  • 9

    Ozpinar ALiu JJWhitney NLTempel ZJChoi PAAndersen PE: Anterior spinal reconstruction to the clivus using an expandable cage after C2 chordoma resection via a labiomandibular glossotomy approach: a technical report. World Neurosurg 90:3723792016

  • 10

    Patel AJGressot LVCherian JDesai SKJea A: Far lateral paracondylar versus transcondylar approach in the pediatric age group: CT morphometric analysis. J Clin Neurosci 21:219422002014

  • 11

    Patel SKLiu JK: Staged bilateral far-lateral approach for bilateral cervicomedullary junction neurenteric cysts in a 10-year-old girl. J Neurosurg Pediatr 12:2742802013

  • 12

    Stephens BHWright NM: Reconstruction of the C-1 lateral mass with a titanium expandable cage after resection of eosinophilic granuloma in an adult patient. J Neurosurg Spine 26:2522562017

  • 13

    Tsitouras VWang SDirks PDrake JBouffet EHawkins C: Management and outcome of chordomas in the pediatric population: The Hospital for Sick Children experience and review of the literature. J Clin Neurosci 34:1691762016

  • 14

    Verburg JMSeco J: Dosimetric accuracy of proton therapy for chordoma patients with titanium implants. Med Phys 40:0717272013

  • 15

    Wewel JTNunna RSTan LAKasliwal MKO’Toole JE: Novel reconstruction of the anterior craniocervical junction using an expandable cage with integrated fixation after total C2 spondylectomy for chordoma. J Clin Neurosci 30:1571602016

  • 16

    Yamada YLaufer ICox BWLovelock DMMaki RGZatcky JM: Preliminary results of high-dose single-fraction radiotherapy for the management of chordomas of the spine and sacrum. Neurosurgery 73:6736802013

TrendMD

Metrics

Metrics

All Time Past Year Past 30 Days
Abstract Views 317 317 95
Full Text Views 49 49 14
PDF Downloads 27 27 9
EPUB Downloads 0 0 0

PubMed

Google Scholar