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Photo Essay
October 1, 2005

Absence of the Greater Sphenoid Wing in Neurofibromatosis 1

Arch Ophthalmol. 2005;123(10):1454. doi:10.1001/archopht.123.10.1454

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