鎖骨頭蓋異骨症の1例および本邦における症例の文献的考察

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Cleidocranial dysplasia is characterized by abnormal ossification, especially in clavices and skull, oral and dental anomalies, together with hereditary characteristics. Martin first reported this disease in 1765, and since then more than 600 cases were reported abroad, and more than 100 cases in Japan. A 27-year-old man consulted us with complaints of oppressive pain, bleeding and mandibular fracture. The prognosis was fairly good after surgical treatment. Based on retrospective survey of 241 cases of cleidocranial dysplasia reported in Japanese literatures from 1918 to the 1986, the results are summarized as follows: 1) 82.2% were abnormalities of the clavicles. 2) 83.8% were disturbances of skull ossification. 3) 58.1% were oral and dental anomalies. 4) 23.7% were hereditary characteristics.