Sphenoid wing dysplasia

Case contributed by Dr Dalia Ibrahim

Presentation

Right proptosis.

Patient Data

Age: 20-year-old
Gender: Male
Modality: CT

Absence of the right sphenoid wing and the lateral orbital wall. Consequently the right temporal lobe with the surrounding CSF and enlarged arachnoid space are prolapsing into the right orbit producing distorsion of the optic nerve and subsequent proptosis.

A gapping bony defect is seen in the posterior aspect of the right orbit giving the "Empty orbit sign"

Absent anatomical landmarks of the optic nerve canal,anterior clinoid, superior and inferior orbital fissures.

Right upper eyelid subcutaneous soft tissue thickening, likely representing plexiform neurofibromatosis.

Diffuse atrophic changes of the right cerebral hemisphere.

Cortical irregularity of the right orbital roof and parietal bone secondary to old trauma and surgical intervention.

Case Discussion

The patient is known to have "Neurofibromatosis type I"

PlayAdd to Share

Case Information

rID: 51596
Case created: 27th Feb 2017
Last edited: 28th Feb 2017
System: Head & Neck
Inclusion in quiz mode: Included

Updating… Please wait.
Loadinganimation

Alert accept

Error Unable to process the form. Check for errors and try again.

Alert accept Thank you for updating your details.