Presentation
Right-sided proptosis.
Patient Data
Absence of the right sphenoid wing and the posterolateral orbital wall. The ipsilateral temporal lobe is enlarged as well as the surrounding CSF space prolapsing into the right orbit with distortion of the optic nerve with subsequent proptosis.
Hypodensities of the globi pallid mainly on the left.
Abnormal hyperintensities on FLAIR and T2 involving mainly the dentate nuclei, brainstem and globi pallidi with nodular enhancement of the lesion located in the pons.
Right sphenoid wing dysplasia with enlarged temporal lobe and adjacent CSF space prolapsing into the orbit causing proptosis and distortion of the optic nerve.
Case Discussion
The CT and MRI features of focal areas of signal intensity (FASI) with sphenoid wing dysplasia in a child with numerous café au lait spots (>6) are highly suggestive of Neurofibromatosis type 1.
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