Unequaled overgrowth inside and out—an exceptional example o
Plexiform neurofibroma is classically associated with neurofibromatosis type I (NF1). However, there are relatively few cases that involve isolated neurofibroma of the orbit of varying pathologies (myxoid, plexiform, diffuse) in the absence of clinical criteria of NF1.

A 10-year-old girl presented to the neurology clinic with an 8-year history of progressive hypertrichosis with associated discoloration and swelling in the right periorbital region . The hypertrichosis covered most of the right half of her face, excluding only the mandibular, malar, and nasolabial regions. The coarse terminal hair was dense with a uniform, thick distribution. The swelling was confined to the periorbital region and did not have associated proptosis of the eye.

Five years prior, ophthalmology had evaluated her, and magnetic resonance imaging of the brain showed an extensive periorbital lesion compressing the optic nerve and extending posteriorly into the right orbit, retromaxillary, and suprazygomatic spaces. Characteristic of plexiform neurofibroma, the magnetic resonance imaging showed the lesion to be infiltrative, ropelike, and hyperintense on T2-weighted images with a target-like appearance. In addition, possible sphenoid wing dysplasia was found.

Source: https://www.jaadcasereports.org/article/S2352-5126(19)30163-8/pdf
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