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Optic nerve glioma

Oculoplasty

Key Points

  • •Optic nerve glioma is a pilocytic astrocytoma (WHO Grade I) - the most common optic nerve tumor; 25–50% associated with NF1; presents in children < 10 years
  • •MRI shows fusiform enlargement of the optic nerve with kinking/buckling; NO calcification (calcification suggests meningioma instead)
  • •NF1-associated gliomas are often indolent and may regress; bilateral optic nerve gliomas are virtually pathognomonic for NF1
  • •First-line treatment for progressive disease: carboplatin + vincristine chemotherapy; radiation is AVOIDED in NF1 patients (risk of secondary malignancies)
  • •Look for NF1 features: café-au-lait macules, Lisch nodules, axillary freckling, neurofibromas, sphenoid wing dysplasia

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Keep reading Optic nerve glioma

17 more sections are included with the Clinical plan.

  • Definition
  • Epidemiology
  • Aetiology and causes
  • Pathogenesis
  • Classification
  • Risk factors and associations

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