두개저, 부비동, 하비갑개, 비중격에 광범위하게 침범된 다골성 섬유성 이형성증 1례

Fibrous dysplasia is considered as a benign disease of the bone having an unknown etiology, characterized by a slow progression. Medullary bone that is replaced by mildly to highly cellular tissue composed of irregular trabeculae of woven bone and spindle-shaped fibroblasts. Craniofacial involvement frequently affects the maxilla and frontal bone and induced local facial swelling, facial asymmetry and exophthalmos. Currently three general subtypes of disease are recognized：monostotic, polyostotic, and McCune-Albright syndrome. We present 15-year-old male with left proptosis, superior eyelid swelling and nasal obstruction. Our case demonstrated an extensive bony lesion involving bilateral frontal, ethmoid, sphenoid sinuses, left maxillary bone, inferior turbinate, septum, and skull base. Hence we report a case of polyostotic fibrous dysplasia with extensive bony involvement. (J Clinical Otolaryngol 2002;13:220-224)