姉弟に生じた鎖骨頭蓋異骨症の1例

概要

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Cleidocranial dysplasia is a form of congenital systemic bone dysplasia, that has such clinical characteristics as aplastic or hypoplastic clavicle bilaterally, delayed ossification of the skull, delayed deciduous dentition and jaw bone development. In Japan, many cases have been reported but relatively few in siblings or structural findings on teeth. We recently experienced cleidocranial dysostosis in an 18-year-old girl and her 16-year-old brother. We detected signs of intraoral abnormality and recorded histological findings related to extracted teeth.<BR>Both cases showed hypoplastic development of the skull, upper jaw, clavicle, pelvis, and bones of the hand. The sister had 19 impacted teeth including 7 supernumerary teeth in her jaws. An impacted tooth extracted from the median upper jaw showed hypoplasia, namely enamellike tissues could be seen in the dentine, and there was very little cementum. Her brother had prolonged retention of the deciduous teeth and 28 impacted teeth including 10 supernumerary teeth. These impacted teeth were mainly seen in anterior teeth and premolars.<BR>In examination of the family, their father was thought to have had similar physical features. Therefore it is assumed that a prepotent factor was transmitted to his children.
社団法人日本口腔外科学会の論文