대한구강악안면방사선학회

Korean Academy of Oral and Maxillofacial Radiology

학회지

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저자 : 정선진, 홍순기
2000년|30권 3호|Page : 229-234|조회수 296
Full Text : 2000_229.pdf (912.0K)
Korean J Oral Maxillofac Radiol. 2000 Sep;30(3):229-234. Korean.

Cleidocranial Dysplasia: Report of a Case.

Jeong SJ, Hong SK.

Department of Oral and Maxillofacial Radiology, College of Dentistry, Chosun University, Korea. djsj6042@hanmail.net

Cleidocranial dysplasia(previously known as cleidocranial dysostosis) is a well-known, rare and hereditary skeletal disorder characterized by a variety of dental abnormalities and as its name implies, striking involvement of the cranial vaults and clavicles. A 17-year-old female who presented with short stature and prolonged retention of deciduous teeth, subsequent delay in eruption of permanent teeth is described. She could touch her shoulders together at the midline anteriorly. Diagnostic procedures showed hypoplasia of the maxillary and zygomatic bones, open fontanelles and sutures, and aplasia of the clavicles. The paranasal sinuses were absent or underdeveloped. Characteristically, she had near parallel-sided borders in the ascending ramus of the mandible and abnormal-shaped, the slender pointed coronoid process. The zygomatic arches had a downward bend and discontinuity at the zygomaticotemporal suture area. Radiographic and clinical investigations of her cranial and skeletal abnormalities revealed features of cleidocranial dysplasia.

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