Bare right orbit - sphenoid dysplasia due to neurofibromatosis (type 1)
Presentation
Known NF1. Right proptosis ? mass in optic nerve. Normal acuity.
Patient Data
Well-defined defect in the right sphenoid allowing the inferior right frontal lobe to herniate into the right orbit and resulting in extrinsic compression of the optic nerve. This can cause a pulsatile exophthalmos. Note the absence of the lesser wing of sphenoid line in the right orbit as compared with the left (arrows). This is responsible for the "bare" orbit.
Case Discussion
This is a bone dysplasia allowing the frontal lobe to herniate, not a bone erosion due to en plaque meningioma, neurofibroma or other tumor. Note in this case the innominate line (tangential greater wing of sphenoid) remains intact. It is the lesser wing of sphenoid (arrow on the left) that is dysplastic letting the frontal rather than the more typical temporal lobe to herniate.
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