Classical eyelid border sign of neurofibromatosis.

Neurofibromatosis is a well-documented entity, having been first recorded by von Reck-linghausen (I882). When involvement of the orbit and eyelid occurs in this disease, the presentation may be manifold. Blepharoptosis commonly occurs and may be variable in degree. Where elephantiasis neuromatosa occurs in association with this, the drooping eyelid characteristically has the sensation of a bag of worms when palpated. In addition exophthalmos may or may not be present. Sometimes, where orbital bony defects exist, the globe has a pulsatile character; bone hypertrophy, however, may be the rule rather than bone destruction. The typical ocular and systemic manifestations of neurofibro-matosis can also be associated with involvement of the orbit and these are well described in the literature (Reese, I963). Attention is drawn here to an important physical sign in the eyelid which is diagnostic of neurofibromatosis of the orbit. In this condition the lid margin of the ptotic eyelid displays a peculiar sinuous configuration, the lid border possessing an inner portion which arches upwards whereas the outer half assumes a downwards convexity (Fig. IA and B).