C leidocranial dysplasia (CCD) is a rare genetic disorder that affects the development of teeth and bones. It has also been referred to as Scheuthauer-Marie-Santon syn- drome, Pierre Marie-Sainton syndrome, and cleidocranial dysos- tosis. CCD occurs in approximately one in 1 million individuals, with no specific race or gender preference.1,2 Dental anomalies are very common among individuals with CCD. Oral health professionals should be knowledgeable about CCD characteristics, pathophysiology, treatment, and oral consid- erations to provide optimal care for people with this condition. CCD was initially thought to exclusively involve bones of mem- branous origin such as the face, skull, and clavicle. However, med- ical discoveries have found CCD is a generalized skeletal dysplasia affecting the entire skeleton rather than just the clavicles and skull. Hence, CCD is considered a dysplasia rather than a dysostosis (dis- order of an individual bone).2 Characteristics Clinical features of CCD are typically discernable in early child- hood, and include incompletely formed or missing clavicles, delayed closure of fontanelles (soft spots on infant’s head where cranial bones have not fused together), short stature, pigeon or conical shape of chest, abnormally long neck, and abnormal teeth development.2–4 An individual with CCD usually has a prominent forehead with a vertical groove down the middle. Narrow and sloping shoulders may also be present. By Leena Balicat, RDH, BSDH, MS; Amber W. Hunt, BSDH, MS, RDH; and Brenda Bradshaw, BSDH, MS, RDH EDUCATIONAL OBJECTIVES After reading this course, the participant should be able to: 1. Identify the clinical characteristics and pathophysiology of cleidocranial dysplasia (CCD). 2. List the common dental anomalies associated with CCD. 3. Discuss the surgical-orthodontic approaches for CCD treatment, importance of patient education, and postoperative care considerations. XIXINXING / ISTOCK / GETTY IMAGES PLUS 28 Dimensions OF DENTAL HYGIENE • January/February 2024 dimensionsofdentalhygiene.com DIMENSIONS CE EARN 2 CE UNITS THIS SELF-STUDY CE COURSE IS WRITTEN FOR DENTISTS, DENTAL HYGIENISTS, AND DENTAL ASSISTANTS. DIMENSIONS PEER-REVIEWED CONTENT SECTION 1 An In-Depth Look at Cleidocranial Dysplasia In order to provide the best possible patient care, oral health professionals need to be aware of the characteristics, pathophysiology, treatment, and oral considerations of this genetic condition.
