4138455.pdf

P. Lukinmaa, B. Jensen, I. Thesleff, J. Andreasen and S. Kreiborg (1995) Histological observations of teeth and peridental tissues in cleidocranial dysplasia imply increased activity of odontogenic epithelium and abnormal bone remodeling Journal of Craniofacial Genetics and Developmental Biology, 15(4):212–221. Abstract Cleidocranial dysplasia (CCD) is a heritable generalized bone dysplasia presenting with a variety of dental abnormalities. To delineate morphological features of the dental tissues, we have analyzed histologically the structure of teeth and peridental tissues obtained from four CCD patients. Dentin appeared regular, except when formed in response to caries, physiological attrition or root resorption. Both acellular and cellular cementum were present in deciduous teeth, whereas in permanent teeth, cellular cementum was virtually lacking and acellular cementum was partially hyperplastic. Enamel pearls were seen in the furcations of one deciduous and one permanent molar.