Spheno-orbital dysplasia associated with plexiform neurofibroma in Von Recklinghausen neuro-fibromatosis

We report a case of plexiform neurofibroma of the upper eyelid associated w ith spheno-orbital dysplasia in a Is-year-old woman with von Recklinghausen neurofibromatosis. Visual acuity was 20/40 in the right eye and 20/20 in t he left. Plexiform neurofibroma involving the right upper eyelid was associ ated with mild ptosis and ipsilateral facial hypertrophy. Biomicroscopic ex amination showed lisch nodules. Funduscopic examination, visual field and n eurologic examinations were normal. Cafe au lait spots involved the trunk w ith neck plexiform neurofibroma. Computed tomography disclosed spheno-orbit al dysplasia. The patient's status remained unchanged at 6 months follow-up . Cranial features of von Recklinghausen neurofibromatosis are found in 3 to 7% of patients. In patients with plexiform neurofibroma of the eyelid the o phthalmologist should look for associated spheno-orbital dysplasia.