Challenges in Diagnosis and Management of Optic Nerve Glioma: A Case Report

Orbit And Oculoplasty

Abstract

Introduction: Optic nerve glioma is a slowly growing, pilocytic astrocytoma that typically affects children (median age 6.5 years)1 .  About 30% of patients have associated Neurofibromatosis type 1 and those have better prognosis.2

Case Report: A 13yr old boy presented with a gradual, painless visual loss in the left eye with associated bulging of the eyeball which  started about 7 years before presentation. Surgery was done at a peripheral hospital (nature of surgery unknown) 1 year after onset of  symptoms, however tumor regrowth over the past 6 years was noticed. Examination revealed a large ulcerated left orbital mass  measuring 8x7x5cm, firm, not tender, the mass was not attached to the orbital rim or skin, no dilated vessels, no bruit, and no eyeball  seen. Right Eye had a visual acuity of 6/24 (unaided) and 6/18 with pinhole, normal anterior segment but pale disc, CDR=0.3, flat retina.  Magnetic resonance imaging (MRI) done 3 months before presentation showed a well circumscribed lesion of mixed  consistency at suprasella region compressing the left ventricle with extension into left orbit. A diagnosis of optic pathway (optic nerve  & optic chiasma) glioma was made. Orbital and intracranial tumour excision was done in conjunction with neurosurgeons. Histology  revealed a pilocytic astrocytoma.