Neurofibromatosis type 1: orbital manifestations

Case contributed by Ahmed Abdrabou
Diagnosis certain


Right orbital swelling of long duration and headache.

Patient Data

Age: 30 years old
Gender: Female

Dysplasia of the right sphenoid wing with associated enlargement of the right orbit in comparison to the left one.

A preseptal and septal soft tissue mass involving the upper and lower eye lid of the right orbit with intraorbital extraconal extension along the superior aspect of the right orbit.

Case Discussion

The patient has manifestations of neurofibromatosis type 1 as evident by sphenoid wing dysplasia (bare orbit sign) as well as plexiform neurofibromata involving the preseptal, septal and postseptal regions of the right orbit.

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