J Korean Ophthalmol Soc.  2006 May;47(5):787-798.

Ocular Findings in Patients with Fibrous Dysplasia

• Kim NJ
• Wang KC
• Kim SW
• Son WG
• Hwang JM

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.

Keyword

Fibrous dysplasia; Optic atrophy; Strabismus

MeSH Terms

Bone and Bones
Cafe-au-Lait Spots
Exophthalmos
Facial Asymmetry
Fibrous Dysplasia, Polyostotic
Humans
Manometry
Optic Atrophy
Optic Nerve Diseases
Orbit
Puberty, Precocious
Skin
Strabismus
Visual Acuity