Orbit Deformities in Craniofacial Neurofibromatosis Type 1
Claude Jacquemina,
Thomas M. Bosleyb and
Helena Svedbergc
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 Research Department, King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia
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FIG 1. T1-weighted MR images.
A, Parasagittal image shows a temporal arachnoid cyst on the left that pushes the orbital contents, including the globe, forward (arrowheads), while a tumor mass in the superior quadrant of the orbit pushes the globe downward (arrow).
B, Axial image of the same patient with a temporal arachnoid cyst on the left (white arrowheads) shows shortening of the lateral wall of the orbit (black arrowhead) and flattening of the orbital angle. Also note the abnormal concave posterior aspect of the lateral orbital wall and the large wing of the sphenoid.
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FIG 2. Coronal CT scans.
A, Image of the classic egg-shaped, orbital enlargement and deformity. Note the phthisical eye (asterisk) and tumoral infiltration of superior rectus muscle (arrow) and orbital septum (arrowheads). The left paranasal sinuses are underdeveloped. The nasal septum and crista galli are deviated.
B, Image of the posterior orbit shows that the tumors in the pterygoid fossa (asterisk) infiltrates the left orbital apex, enlarging the inferior orbital fissure and distorting the posterior orbit.
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FIG 3. A, Sagittal T1-weighted, contrast-enhanced, fat-suppressed MR image shows infiltration of the superior extraconal space (small asterisk), superior rectus muscle, and levator palpebrae muscle by PNFs. Posterior extension of the tumor into the cavernous sinus is marked by the large asterisk. Intraconal sensory nerves are contrast enhanced and thickened because of tumor infiltration. Ipsilateral maxillary sinus is small compared with that on the contralateral side.
B, Coronal T1-weighted MR image of the same patient shows extension of the tumor into the cavernous sinus, with discrete remodeling of the sphenoid body (arrowheads).
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FIG 4. Axial CT image of the skull base shows expanded cranial foramina (foramen ovale, rotundum, and lacerum) on the left (asterisk) due to tumor enlargement of branches of the trigeminal nerve.
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FIG 5. Axial CT image through the orbits shows unilateral enlargement of the right optic canal due to tumor infiltration of the optic nerve sheath without an optic nerve glioma. The ipsilateral globe is buphthalmic, and the lateral rectus (asterisk) is enlarged as a result of tumoral infiltration. The greater wing of the sphenoid is partially decalcified and bowed forward (arrowheads), without an obvious arachnoid cyst.
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