Ocular Findings in Patients with Fibrous Dysplasia.

Kim, Nam Ju; Wang, Kyu Chang; Kim, Suk Wha; Son, Woo Gil; Hwang, Jeong Min

Original Article

Journal of the Korean Ophthalmological Society 2006;47(5):787-798.
Published online May 31, 2006.

Ocular Findings in Patients with Fibrous Dysplasia.

Nam Ju Kim, Kyu Chang Wang, Suk Wha Kim, Woo Gil Son, Jeong Min Hwang

¹Department of Ophthalmology, Seoul National University College of Medicine, Seoul National University Bundang Hospital, Gyeonggido, Korea. hjm@snu.ac.kr
²Seoul National University Children's Hospital and Neurological Research Institute, SNUMRC, Seoul, Korea.
³Department of Reconstructive Plastic Surgery, Seoul National University College of Medicine, Division of Pediatric Plastic Surgery, Seoul National University Children's Hospital, Seoul, Korea.

섬유형성이상 환자의 눈 소견

김남주¹,왕규창²,김석화³,손우길³,황정민¹

Department of Ophthalmology, Seoul National University College of Medicine, Seoul National University Bundang Hospital¹, Gyeonggido, Korea Seoul National University Children`s Hospital & Neurological Research Institute, SNUMRC², Seoul, Korea Department of Reconstructive Plastic Surgery, Seoul National University College of Medicine, Division of Pediatric Plastic Surgery, Seoul National University Children`s Hospital³, Seoul, Korea

Correspondence: Nam Ju Kim, M.D.¹

Abstract

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 cafe-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.

Key Words: Fibrous dysplasia;Optic atrophy;Strabismus