A 28-year-old man had left orbital dystopia, ptosis, and pulsatile exophthalmos (Figure 1). Visual acuity was 20/20 OD (without correction) and 20/400 OS (with −18 diopter sphere). Ophthalmic examination disclosed bilateral Lisch nodules and myopic staphyloma in the left eye. Computed tomographic scans of the head revealed absence of the left greater sphenoid wing and temporal lobe herniation into the orbit (Figure 2 and Figure 3). A diagnosis of neurofibromatosis 1 (NF1) was made.1
Photograph showing craniofacial abnormalities in neurofibromatosis 1 with absence of greater sphenoid wing.
Axial computed tomographic scan of the head showing absence of the left greater sphenoid wing. Note the left temporal lobe, along with the surrounding cerebrospinal fluid and arachnoid space, prolapsing into the orbit (white asterisk). The black asterisk shows the normal right greater sphenoid wing.
A 3-dimensional computed tomographic scan of the cranial base shows absence of the left greater sphenoid wing, with ample communication between the left orbit and cranial vault. The greater sphenoid wing absence allows the temporal lobe to prolapse into the orbit. The black asterisks indicate the middle cranial fossae; white asterisk, the occipital foramen.
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