Neurofibromatosis type 1 (NF1) is an autosomal dominant, multisystem disorder affecting approximately one in three thousand people. Neurofibromas of the eyelid are its wellrecognized manifestation. Several different presentations have been described. Some of the more common orbital and eyelid findings in NF1 are plexiform neurofibromas, causing mechanical ptosis and proptosis, optic nerve gliomas and sphenoid wing dysplasia. Plexiform neurofibroma is a non-circumscribed, thick and irregular benign tumor of the peripheral nerve sheath. It is a virtually pathognomonic and often disabling feature of NF type I. Lid margin of ptotic eyelid displays a peculiar sinuous configuration, lid border possessing an inner portion arches upwards whereas outer half assumes a downward convexity. We present a case report of neurofibroma of lid, with the inverted configuration which alters the approach of surgery. It was reviewed and diagnosed as NF1 clinically, radiologically and on histopathology.

Eyelid Plexiform Neurofibroma: Atypical Presentation and Management

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