Presentation
Head shaking and bilateral nystagmus (spasmus mutans) noticeable at 7 months of age.
Patient Data
Bilateral symmetrical fusiform enlargement and kinking of the optic nerves indicating optic nerve glioma. It extends intracranially involving the optic chiasm, optic tracts and lateral thalami bilaterally. It is isointense on T1, hyperintense on T2 and FLAIR. The lesions demonstrate heterogeneous enhancement following IV contrast administration. No cystic component is seen within. The lesions are bright on DWI images. No blooming artifact on GRE to suggest calcification or microhemorrhage.
Incidental findings of a small left temporal extra-axial arachnoid cyst.
Findings are suggestive of bilateral optic pathway gliomas (OPGs) involving optic nerves, optic chiasm, optic tracts and both lateral thalami.
Case Discussion
The involvement and distribution of the lesions are characteristic of optic pathway glioma (OPGs). Posterior extension involving the optic chiasm and optic pathways is more common in sporadic cases compared to NF 1 related glioma. The extension indicates more aggressive disease and poor clinical outcomes.
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