側頭骨線維性骨異形成症の2症例

Fibrous Dysplasia of the Temporal Bone:Report of 2 Cases

　はじめに

　線維性骨異形成症（Fibrous dysplasia）は線維性組織と骨組織の増殖が主体の良性の骨病変である。単骨性に出現するmonostoticの型と，多骨性に出現するpolyostoticの型があり，後者のうち皮膚の異常色素沈着，内分泌異常を伴うものをAlb-right症候群と呼んでいる。臨床の場において頭頸部領域で遭遇することは少なく，その中でも上顎骨，下顎骨に多く認められ，側頭骨にみられるのは比較的稀である。

　今回われわれは，外耳道狭窄，難聴が主訴で，側頭骨に生じた線維性骨異形成症の症例に対して，外耳道形成術を施行し，長期経過観察し得た2症例を経験したので，その症例を報告するとともに，治療方法，術後経過などについて若干の文献的考察を加えて検討した。

The first case was a 25-year-old man who complained of a hearing loss and stenosis of the external auditory canal. The right external canalo-plasty was performed. Fourteen months after surgery, the external canal was patent with no evidence of recurrence. The second case was a 49-year-old female who had removal of a swelling of bone from the right zygoma at age 45. The histological diagnosis was fibrous dys-plasia. She complained of otorrhea and stenosis of the external canal and underwent right extre-nal canaloplasty. Five years after surgery, the external canal was patent with no evidence of recurrence.

Review of 37 reported cases in the literature (1946-1990) were made. More males than females were affected. All of the patients had some characteristic symptoms : (1) a progressive hear-ing loss, usually conductive, (2) earfullness, (3) progressive occlusion of the external auditory canal. The age of onset ranged from 5 months to 54 years, the mean age was 19 years. The age of the first clinical examination ranged from 9 months to 78 years, the mean age was 27 years. Conserv-ative surgery is recommended for fibrous dys-plasia of the temporal bone.