목적 : 섬유형성이상은 정상 골조직이 섬유조직과 비정상적인 골양조직으로 대체되는 드문 질환으로, 눈 주위의 뼈가 침범되는 경우 눈에 합병증이 발생할 수 있다. 안와 섬유형성이상 환자의 눈 소견을 알아 보고자 하였다. 대상과 방법 : 안와의 섬유형성이상 환자 6명을 대상으로 전향적으로 얼굴의 비대칭을 조사하였고 외안부 검사, 시력검사, 안압측정, 굴절검사, 사시검사, 입체시 검사, 안저검사 등을 시행하였다. 결과 : 안구돌출이 4명에서 있었고, 사시가 5명에서 있었으며 이중 1명은 사시수술을 받았다. 2명에서 환측의 시력저하가 있으면서 구심동공운동장애와 시신경위축이 있었다. 1명은 피부의 caf&eacute;-au-lait 반점과 조기성숙이 함께 나타나는 McCune-Albright 증후군이었다. 6명 모두에서 얼굴의 비대칭이 있었고 이환측의 상안와연이 높았다. 6명 중 5명이 두개성형술을 받았다. 결론 : 안와를 침범하는 섬유형성이상에서는 안구돌출, 사시, 시신경병증이 발생할 수 있어 안과검사가 필수적이다. 얼굴이 비대칭인 환자에서 시신경위축, 사시, 안구돌출 등을 발견할 때 섬유형성이상의 가능성을 염두에 두어야 하겠다. <한안지 47(5):787-798, 2006>

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&eacute;-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. J Korean Ophthalmol Soc 47(5):787-798, 2006

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&eacute;-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. J Korean Ophthalmol Soc 47(5):787-798, 2006

Fibrous dysplasia, Optic atrophy, Strabismus