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Perineural gliomatosis associated with isolated optic nerve gliomas
  1. S N Yeung1,
  2. M K S Heran2,
  3. A Smith2,
  4. V A White1,3,
  5. J Rootman1,2,3
  1. 1
    Department of Ophthalmology and Visual Sciences, University of British Columbia, Vancouver, BC, Canada
  2. 2
    Department of Radiology, University of British Columbia, Vancouver, BC, Canada
  3. 3
    Department of Pathology and Laboratory Medicine, University of British Columbia, Vancouver, BC, Canada
  1. Dr S N Yeung, Department of Ophthalmology and Visual Sciences, University of British Columbia, 2550 Willow Street, Vancouver, BC, Canada V5Z 3N9; sonia.y{at}gmail.com

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Optic pathway gliomas represent 2 to 5% of all brain tumours in children,1 and are the most common central nervous system tumours in patients with neurofibromatosis type 1 (NF1).2 3 They can involve the nerve proper, chiasm, hypothalamus and optic tracts, and are classified as grade I pilocytic astrocytomas.

Previous reports have suggested perineural gliomatosis, defined as extraneural growth of glioma into the surrounding subarachnoid space, as a characteristic of NF1-associated optic nerve gliomas, whereas isolated optic nerve gliomas typically display intraneural growth.48 We report three patients with non-NF optic nerve gliomas with perineural gliomatosis, which has not previously been reported in the context of combined MRI and clinicopathological evaluation.

Representative case report

A 2½-year-old girl was referred to the Orbit Service with gradual right proptosis over 3 months. Visual acuity in the right eye was light perception and 6/6 in the left eye. A right 1.9 afferent pupillary defect was noted, with 3 mm of proptosis and 3 mm of downward displacement. Ocular movements were normal. There was a congested right disc with venous dilatation. Cycloplegic refraction was +3.00 in the right …

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