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Background: The authors report on a 30-year-old man who presented with progressive bilateral exophthalmos over a 2-year period., Findings: Computed tomography showed large heterogeneous masses in the superior aspect of both orbits. Excisional biopsy via bilateral lateral orbitotomies showed the tumors to be well-circumscribed, relatively avascular, localized neurofibromas. The patient had several features suggestive of multiple endocrine neoplasia type IIB, including Marfanoid habitus, enlarged corneal nerves, thickened lips, and mucosal neuromas., Conclusion: Localized neurofibromas are rare in the orbit and, unlike plexiform neurofibromas, are not typically associated with von Recklinghausen's neurofibromatosis. Bilaterality of such localized neurofibromas has not been previously reported. Recognition of ophthalmic lesions suggestive of multiple endocrine neoplasia IIB should prompt evaluation for systemic manifestations of this disorder.

The association between induced contralateral upper eyelid retraction and blepharoptosis, although well known, has not been well analyzed. The authors prospectively studied 50 consecutive patients with blepharoptosis. Interpalpebral fissure measurements of the contralateral "normal" or relatively less blepharoptotic eyelids were made in the resting position, with the blepharoptotic eye occluded, manually elevated, and after instillation of phenylephrine 2.5%. Ocular dominance also was tested. Contralateral interpalpebral fissure height decreased greater than or equal to 1 mm in 10 of 50 patients (20%) after manual elevation. Blepharoptosis was present or greater in the dominant eye in 7 of 10 (70%) patients in this group, but in only 7 of 40 (18%) patients in the group not showing such a response (P less than 0.001). Of 12 patients with congenital blepharoptosis, none demonstrated this response. In patients with acquired blepharoptosis, contralateral decrease in eyelid position also was directly associated with severity of blepharoptosis in the opposite eye. These findings suggest that contralateral induced eyelid elevation or retraction is frequently associated with blepharoptosis and is more apparent as visual impairment secondary to blepharoptosis increases. Detection of contralateral eyelid retraction is important in the preoperative evaluation of blepharoptosis.