섬유형성이상 환자의 눈 소견

Ocular Findings in Patients with Fibrous Dysplasia

Purpose: Fibrous dysplasia is an uncommon disease that replaces normal bone tissue with fibrous and osteoid tissue. We evaluated patients with fibrous dysplasia involving orbital bones to identify the ocular characteristic findings. Methods: We recruited 6 patients with fibrous dysplasia of the craniofacial bone. Best corrected visual acuity and facial asymmetry were measured. Slit lamp biomicroscopy, tonometry, strabismus examination, and funduscopy were performed. Results: Strabismus, exophthalmos and optic neuropathy were found in 5, 4, and 2 patients, respectively. One patient underwent a strabismus surgery. One patient had McCune-Albright syndrome, exhibiting a caf?-au-lait spot of the skin and precocious puberty. All 6 patients had facial asymmetry and higher superior orbital rims of the affected side in 5 patients. Conclusions: Ophthalmologic examination may be mandatory for patients with fibrous dysplasia involving the orbit, especially to prevent optic neuropathy. If optic atrophy, strabismus or exophthalmos is found in patients with facial asymmetry, the possibility of fibrous dysplasia must be considered.