쇄골두개 이형성증 환아의 치아발육이상

Abstract

Cleidocranial Dysplasia(CCD) is an autosomal dominant human bone disease characterized by abnormal clavicles, patent sutures and fontanelles, and dental anomalies. Among dental anomalies, it is characterized that permanent dentition is severly disturbed due to multiple supernumerary teeth and abnormalities of tooth morphology. A eight-year-old female patient diagnosed as cleidocranial dysplasia visited in our hospital. Upon clinical oral exam, retained deciduous teeth, constriction of dental arch, anterior cross bite, and multiple dental caries were observed. In the dental panoramic radiograph, retained deciduous teeth and multiple supernumerary teeth in the maxilla and the mandible were found. In the cephalometric radiograph, open sutures and wormian bones were seen. In the chest P-A view absence of clavicles was observed. The cleidocranial dysplasia patients have eruption problems in permanent dentition both in regions with and without supernumerary teeth. The severely delayed or arrested eruption of permanent teeth has been ascribed to various factors : 1) The presence of multiple supernumerary teeth, 2) malformed roots with lack of cellular cementum, 3) the jaw bone being too dense, and 4) abnormal resorption of bone and primary teeth. Formation and maturation of primary teeth in cleidocranial dysplasia are normal, whereas the permanent dentition has various anomalies. Therefore, dentists should understand the development of dentition in cleidocranial dysplasia, and treat them in proper time.