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Sphenoid wing dysplasia

Case contributed by Huda B. Gharbia
Diagnosis almost certain

Presentation

Left proptosis, swollen left eyelid.

Patient Data

Age: 4 years
Gender: Female
mri

There is asymmetry of the middle cranial fossae, enlarged on left side with ipsilateral sphenoid wing dysplasia, causing deformity of left orbital cavity, and splaying the left optic canal.

Also, there is left eyelid subcutaneous soft tissue lesion, which appears slightly hyperintense in T2W and shows homogenous enhancement on post-contrast images.

Case Discussion

Left sphenoid wing dysplasia, with left subcutaneous eyelid soft tissue likely a neurofibroma, suggestive neurofibromatosis type1. The patient also has multiple cutaneous cafe au lait spots. No brain parenchymal abnormalities on current examination.

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