Cleidocranial dysplasia : a case report

쇄골두개이골증의 증례

  • Kim Jae-Duk (Dept. of Dentistry, Graduate School, Chosun University) ;
  • Lee Chang-Yul (Dept. of Dentistry, Graduate School, Chosun University) ;
  • You Choong-Hyun (Dept. of Dentistry, Graduate School, Chosun University)
  • 김재덕 (조선대학교 대학원 치의학과 구강악안면방사선학교실) ;
  • 이창율 (조선대학교 대학원 치의학과 구강악안면방사선학교실) ;
  • 유충현 (조선대학교 대학원 치의학과 구강악안면방사선학교실)
  • Published : 2004.03.01

Abstract

Cleidocranial dysplasia is a rare and autosomal dominent disorder characterized by aplasia or hypoplasia of the clavicles, an open fontanelle, dental abnormalities, and short stature, A 17-year-old female who presented with short stature and subsequent delay in eruption of permanent teeth is described. she showed the abnormal hypermobility of the shoulder, ocular hypertelorism and concave nasal bridge. Radiographs revealed the underdeveloped maxilla, defect of the cranium in the fontanelle region, and aplasia of the clavicles. Characteristically, panoramic view revealed near parallel-sided borders of the ascending ramus and downward curvature of the zygomatic arch with hypoplasia. The prolonged retention of deciduous teeth with delayed eruption of permanent teeth and multiple embedded supernumerary teeth were striking. Radiographic and clinical investigations revealed Cleidocranial dysplasia.

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