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



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.


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.


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


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.


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



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.


  1. 1.
    Janssen D, Harms D (2005) Juvenile xanthogranuloma in childhood and adolescence: a clinicopathologic study of 129 patients from the kiel pediatric tumor registry. Am J Surg Pathol 29:21–28CrossRefGoogle Scholar
  2. 2.
    Dehner LP (2003) Juvenile xanthogranulomas in the first two decades of life: clinicopathologic study of 174 cases with cutaneous and extracutaneous manifestations. Am J Surg Pathol 27:579–593CrossRefGoogle Scholar
  3. 3.
    Cao D, Ma J, Yang X, Xiao J (2008) Solitary juvenile xanthogranuloma in the upper cervical spine: case report and review of the literatures. Eur Spine J 17(Suppl 2):S318–S323CrossRefGoogle Scholar
  4. 4.
    Inoue H, Seichi A, Yamamuro K, Kojima M, Kimura A, Hoshino Y (2011) Dumbbell-type juvenile xanthogranuloma in the cervical spine of an adult. Eur Spine J 20(Suppl 2):S343–S347CrossRefGoogle Scholar
  5. 5.
    Jain A, Mathur K, Khatri S, Kasana S, Jain SK (2011) Rare presentation of juvenile xanthogranuloma in the thoracic spine of an adult patient: case report and literature review. Acta Neurochir (Wien) 153:1813–1818CrossRefGoogle Scholar
  6. 6.
    Shenoy A, Singhal SS, Shinde S (2018) Solitary intraspinal juvenile xanthogranuloma in an infant. Asian J Neurosurg 13:172–175CrossRefGoogle Scholar
  7. 7.
    Emile JF, Abla O, Fraitag S, Horne A, Haroche J, Donadieu J et al (2016) Histiocyte Society. Revised classification of histiocytoses and neoplasms of the macrophage-dendritic cell lineages. Blood 127:2672–2681CrossRefGoogle Scholar
  8. 8.
    Pirillo V, Prontera A, Rizzo P, Cecchi PC, Maffei M, Schwarz A (2017) A rare case of intramedullary solitary juvenile xanthogranuloma of the lumbar spine in the adult: a case report. J Spine Surg 3:504–508CrossRefGoogle Scholar
  9. 9.
    Levine DN (2004) The pathogenesis of syringomyelia associated with lesions at the foramen magnum: a critical review of existing theories and proposal of a new hypothesis. J Neurol Sci 220:3–21CrossRefGoogle Scholar
  10. 10.
    Menezes AH, Ahmed R (2014) Primary atlantoaxial bone tumors in children: management strategies and long-term follow-up. J Neurosurg Pediatr 13:260–272CrossRefGoogle Scholar
  11. 11.
    Salunke P, Sahoo SK, Sood S, Mukherjee KK, Gupta SK (2016) Focusing on the delayed complications of fusing occipital squama to cervical spine for stabilization of congenital atlantoaxial dislocation and basilar invagination. Clin Neurol Neurosurg 145:19–27CrossRefGoogle Scholar
  12. 12.
    Štulík J, Kozák J, Šebesta P, Vyskočil T, Kryl J, Klezl Z (2010) Total spondylectomy of C2: report of three cases and review of the literature. J Spinal Disord Tech 23:e53–e58CrossRefGoogle Scholar
  13. 13.
    Classen CF, Minkov M, Lehrnbecher T (2016) The non-Langerhans cell histiocytoses (rare histiocytoses)—clinical aspects and therapeutic approaches. Klin Padiatr 228(6–07):294–306Google Scholar
  14. 14.
    Agabegi SS, Iorio TE, Wilson JD, Fischgrund JS (2011) Juvenile xanthogranuloma in an adult lumbar spine: a case report. Spine (Phila Pa 1976) 36:E69–E73CrossRefGoogle Scholar
  15. 15.
    Castro-Gago M, Gómez-Lado C, Alvez F, Alonso A, Vieites B (2009) Juvenile xanthogranuloma of the cauda equina. Pediatr Neurol 40:123–125CrossRefGoogle Scholar
  16. 16.
    Irmola T, Laitinen MK, Parkkinen J, Engellau J, Neva MH (2018) Solitary juvenile xanthogranuloma in the spine pretreated with neoadjuvant denosumab therapy followed by surgical resection in a 5-year-old child: case report and literature review. Eur Spine J. Google Scholar

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

Personalised recommendations