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Bilateral optic pathway gliomas

Case contributed by Thandi E Buthelezi
Diagnosis certain

Presentation

A 7 month old baby girl who presented 3 months ago with left eye proptosis. Follow up MRI was done.

Patient Data

Age: 7 months
Gender: Female

MRI Brain with Contrast

mri

MRI Brain demonstrates avidly enhancing, T2 isointense to grey matter lesion along the optic pathway, from the left intraorbital optic nerve and right intracanalicular optic nerve, extending to involve the optic chiasm into the lateral geniculate bodies. MRI features in keeping with bilateral optic pathway glioma. There is evidence of left eye enucleation. 

Case Discussion

Histologically confirmed a pilocytic astrocytoma (WHO grade 1) of the optic nerve. The patient did not have neurofibromatosis type 1 stigmata.

Optic nerve gliomas are the most common tumor of the optic nerve 1. It is a low grade astrocytoma and is associated with neurofibromatosis type 1.

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