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Natural history of primary paediatric optic nerve sheath meningioma: case series and review
  1. Daniel S Narayan1,
  2. Ghislaine L Traber2,
  3. Edwin Figueira1,
  4. Adnan Pirbhai1,
  5. Klara Landau1,
  6. Garry Davis1,
  7. John Crompton1,
  8. Dinesh Selva1
  1. 1 Department of Ophthalmology, South Australian Institute of Ophthalmology, Adelaide, South Australia, Australia
  2. 2 Department of Ophthalmology, University Hospital Zurich, University of Zurich, Zurich, Switzerland
  1. Correspondence to Daniel S Narayan, South Australian Institute of Ophthalmology Level 8, Royal Adelaide Hospital Adelaide, South Australia Australia, 5000; daniel.s.narayan{at}


Purpose To study the natural history, clinical and radiological characteristics of primary paediatric optic nerve sheath meningioma (PPONSM).

Methods Retrospective study of eight paediatric patients who were treated between 1994 and 2016 at the University Hospital Zurich, Switzerland and the Royal Adelaide Hospital, Australia. Clinical records and imaging studies were reviewed.

Results The mean age at presentation was 11 years (range: 6–17 years). There were six female patients and two male patients. 2/8 patients had associated neurofibromatosis type 2. Patients were followed up for 71–297 months (mean 156±70 months). 6/8 patients were observed through the course of their disease and 2/8 patients were treated with radiotherapy. 2/8 patients who were observed had minimal change in vision and did not experience tumour growth after long-term follow-up.

Conclusions This is the largest PPONSM case series with long-term data on patients treated conservatively. We highlight that a small subset of these tumours are indolent and can be managed using observation alone.

  • optic Nerve
  • orbit
  • vision

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  • Contributors DS conceived the study. DN, KL, JC and GD initiated the design of the study. DN, GT, AP and EF helped with the implementation of the study. DN and GT did the data collection, analysis and interpretation. DN, GT, AP and EF performed the drafting of the manuscript. All authors contributed to revision of the manuscript and final approval of the version to be published.

  • Competing interests GT is supported by ‘Filling the Gap’ of the University of Zurich, Zurich, Switzerland.

  • Patient consent Detail has been removed from this case description/these case descriptions to ensure anonymity. The editors and reviewers have seen the detailed information available and are satisfied that the information backs up the case the authors are making.

  • Provenance and peer review Not commissioned; internally peer reviewed.

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