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Sphenoid wing dysplasia

Case contributed by Dalia Ibrahim
Diagnosis certain

Presentation

Facial asymmetry and left eye proptosis.

Patient Data

Age: 8 years
Gender: Female
mri

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

ct

A gaping bony defect is seen in the posterior aspect of the left orbit giving the "empty orbit sign"

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

Absent soft tissue masses.

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