Reassessment of Sphenoid Dysplasia Associated with Neurofibromatosis Type 1
Claude Jacquemina,
Thomas M. Bosleyb,
Don Liub,
Helena Svedbergb and
Amal Buhaliqab
a Diagnostic Imaging Department, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia
b Neuro-ophthalmology Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia
c Oculoplastics Division, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia
d Research Department, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia
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FIG 1. Series of axial CT scans.
A, Infiltration and decalcification of the sphenoid wing underlying neurofibromas.
B, Infiltration and decalcification of the temporal bone underlying neurofibromas.
C, Infiltration and decalcification of the occipital bone underlying neurofibromas.
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FIG 2. Axial contrast-enhanced CT scan shows bilateral facial soft-tissue tumor infiltration, bilateral enlarged middle cranial fossae (arrowheads), bilateral large globes (buphthalmos), and infiltration of both optic nerve sheaths.
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FIG 3. Enlargement of the left middle cranial fossa with temporal arachnoid cyst, absence of the left sphenoid wing, and flattening of the temporal bone. There is tumor invasion of the orbit with reduced orbital volume, and the left eye was enucleated. The insert shows an abnormal temporal squamosa suture (arrowheads) underlying tumor in the left superficial temporal fossa.
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FIG 4. Axial CT scan shows decalcification of sphenoid bone (arrowheads) adjacent to neurofibroma infiltration of the lateral rectus muscle (arrows) in the absence of middle cranial fossa enlargement. Tumor also enlarges the superior orbital fissure and extends into the cavernous sinus (curved arrow).
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FIG 5. CT scan of our one patient with minimal expansion of the middle cranial fossa and abnormally large decalcification of the ipsilateral sphenoid wing, with no detectable adjacent tumor. The left globe is also enlarged.
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FIG 6. Axial CT scan of a patient with smaller middle cranial fossa and flattened temporal bone ipsilateral to extracranial tumor (arrows).
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FIG 7. Series of axial CT scans (soft-tissue and bone windows) obtained during three scanning sessions over several years show progression of sphenoid bone abnormalities over time. Note that the soft-tissue mass increases over time (original, A and D; 5 years later, B and E; and 10 years later, C and F). and the ipsilateral middle cranial fossa becomes progressively more enlarged.
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