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A novel fixation technique using anterior C1 screw in a pediatric solitary cervical spinal juvenile xanthogranuloma

  • Rekhapalli Rajasekhar
  • Madhivanan KarthigeyanEmail author
  • Pravin Salunke
  • Kirti Gupta
Case Report

Abstract

Purpose

Juvenile xanthogranuloma (JXG) presenting as solitary vertebral body lesion is infrequently seen and usually limited to one or two levels. We report a case of an isolated JXG with extensive cervical spinal (bony and extradural) involvement in a 6-year-old child. There was a diagnostic dilemma as the radiologic and intraoperative picture resembled tuberculosis. The spinal reconstruction was also challenging due to involvement of multiple vertebral levels and necessitated an anterior C1 screw.

Methods

The lytic lesion was multicompartmental, involving the craniovertebral junction and the subaxial spine (till C6 vertebral body) and extending into the retropharyngeal space. Noticeably, an associated thoracic syringomyelia was also present. Near-total excision of the lesion and 360° spinal fixation was performed using fibular strut graft. The graft was cranially anchored to the C1 anterior arch, thereby sharing the load with the posterior occipito-cervical instrumentation in order to avoid a construct failure due to cantilever effect.

Results

At 12-month follow-up, the patient had good clinico-radiologic outcome with evidence of bony fusion and resolution of syrinx.

Conclusion

The report highlights the diagnostic dilemma of JXG lesion on both the radiology and surgery and discusses the challenges in the management and the relevant literature. The described technique can be a viable option in pediatric tumors with extensive C2 vertebral body involvement. Occasionally, extradural compression can have associated syrinx formation and the primary treatment per se could tackle the underlying syringomyelia.

Keywords

Juvenile xanthogranuloma Non-Langerhans cell histiocytosis Craniovertebral junction Cervical spine Fibular graft Vertebral body C1 screw 

Notes

Acknowledgements

We thank Mr. Dharamjit singh, medical illustrator at PGIMER, Chandigarh, for his illustrations.

Compliance with ethical standards

Conflict of interest

The authors declare that they have no conflict of interest.

Informed consent

Informed consent was obtained from the patient.

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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Department of NeurosurgeryPostgraduate Institute of Medical Education & Research (PGIMER)ChandigarhIndia
  2. 2.Department of HistopathologyPGIMERChandigarhIndia

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