측두골에 발생한 섬유성 이형성증 1례

Fibrous dysplasia is a bone disorder of unknown etiology in which normal bone is replaced with fibrotic tissue and disorganized immature bone trabeculae. The disease has three variants ; monostotic, polyostotic, and Al- brighfs syndrome. Monostotic fibrous dysplasia especially involving mainly squama in temporal bone is rare. The clinical course of them is unpredictable. Monostotic form arises usually in childhood and often come to an arrest at puberty. Polyostotic form without endocrine dysfunction tends to appear at a slightly earlier age than the monostotic form and may progress into middle age. Mostly their involvement is unilateral. Monostotic form involving temporal bone is associated with conductive hearing loss attributable to compression of the external auditory canal. Potential problems include cholesteatoma, recurrence and malignant transformation. We report a case of monostotic fibrous dysplasia of temporal bone involving mainly squama, which is succe-ssfully treated with canaloplasty and skin graft. We also observed the eletron microscopic findings. (J Clinical Otolaryngol 2000;11:109–114)