A CASE OF DYSOSTOSIS GENERALISATA

DOI 7 References
  • ABUKAWA Harutsugi
    Department of Dentistry and Oral Surgery, Hirosaki University School of Medicine
  • KOMATSU Kenichi
    Department of Dentistry and Oral Surgery, Hirosaki University School of Medicine
  • FUKUI Roh
    Department of Dentistry and Oral Surgery, Hirosaki University School of Medicine
  • NAKAYAMA Katsunori
    Department of Dentistry and Oral Surgery, Hirosaki University School of Medicine
  • ENDOH Yumi
    Department of Dentistry and Oral Surgery, Hirosaki University School of Medicine
  • KIMURA Hiroto
    Department of Dentistry and Oral Surgery, Hirosaki University School of Medicine

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  • 全身性骨形成不全症の一例

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Abstract

Cleidocranial dysostosis (CD) is a rare congenital osseous disease which is characterised by anomaly of the clavicle and skull with dental abnormalities. The most common deformity is the absence of the central clavicular segment and the dental abnormalities are prolonged retention of primary teeth, late eruption of permanent teeth, supernumerary teeth and so on.<BR>Since the first description of this disease was made by Martin in 1765, more than 150 cases were reported in Japan. On the other hand in 1936 Haneda proposed to define CD with a systemic bone anomaly in the whole body as dysostosis generalisata (DG). The authors experienced a young boy with dental abnormalities and a large extent of bone anomaly at the clavicles, skull, rib, pelvis and carpal bone. We diagnosed this case as DG, therefore, clinical findings and a review of the literature are reported here.

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