Sphenoid wing dysplasia (bare orbit)

Case contributed by Dr Mohammad A. ElBeialy

Presentation

4 years old male child presenting with right pulsating proptosis and squint with right temporal swelling since birth. Past history of irreducible ileocolic intussusception and surgical reduction was done.

Patient Data

Age: 4 years
Gender: Male

Defective greater wing of the right sphenoid bone and the lateral orbital wall with enlargement of the right superior orbital fissure and the right orbital cavity as well as focal indentation of the right lateral rectus muscle by the enlarged anterior temporal extra-axial CSF space with consequent mild right proptosis.

CT examination of the orbit confirmed the right bare orbit with defective and dysplastic right greater wing of sphenoid.

Case Discussion

Dysplastic and defective right greater wing of sphenoid bone; consistent with right bare orbit; suggesting the possibility of neurofibromatosis type I (NFI).  Clinical examination of this child revealed skin nodules and cafe au lait patches as well as axillary freckling. The previous ileo-colic intussusception might have been related to neurofibromatosis of the ileum.

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