[Compressive optic neuropathy caused by fibrous dysplasia].

We report a case of compressive optic neuropathy caused by fibrous dysplasia in a 28-year-old woman. The patient had no significant medical history. Her best-corrected visual acuity was 20/20 in the right eye and 20/400 in the left eye. There was an afferent pupillary defect in the left eye. Slit-lamp examination was unremarkable. Funduscopy showed a normal optic disc bilaterally and congenital hypertrophy of the retinal pigment epithelium in the right eye. Systemic evaluation disclosed facial asymmetry and mucocutaneous lentiginosis involving the face and the limbs. Goldmann visual field testing showed a cecocentral scotoma in the left eye. Imaging studies demonstrated extensive changes of craniofacial fibrous dysplasia involving the sphenoid bone, with compression of the left optic nerve by a cystic structure. Results of gastrointestinal fibroscopy were unremarkable. The patient was given systemic steroids. After 4 days of treatment, her visual acuity had improved to 20/40, with resolution of the afferent pupillary defect and visual field improvement. Debulking of the tumor was recommended, but the patient declined the procedure. She has remained stable over 13 months of follow-up. Compressive optic neuropathy should be considered as a leading cause of visual loss in patients with craniofacial fibrous dysplasia. Early diagnosis, close follow-up, and appropriate management are mandatory to improve or maintain the visual function in such patients.