title: Differing therapeutic approaches to cleidocranial dysplasia (CCD).
abstract: Cleidocranial dysplasia (CCD) (MIM 119600) is a genetic skeletal disorder characterised by skeletal alterations at numerous bone segments (cranium, clavicles, pelvis) and a typical hyperdontia. It is a hereditary disease of the dominant autosomal type with wide variability of expression. The constant presence of numerous supernumerary teeth poses two orders of problems. On one hand, if these supplementary teeth are impacted, they may constitute a mechanical obstacle to the eruption of normal teeth. If on the contrary they have erupted, they cause aesthetic and functional damage. Surgical therapy is indispensable to restore a correct architecture to the alveolar-dental arches, and it is recommended during childhood to avoid or at least reduce complications in adulthood. Two cases of cleidocranial dysplasia are here reported. The adult patient (Case 1), reached our observation with a poor oral situation, manifested relapsing odontogenic abscesses at several impacted supernumerary teeth. The surgical treatment was complex and invasive, requiring percutaneous access in order to remove a tooth embedded in the mandible and the extraction of almost all residual teeth. This outcome was avoided in the second case, through combined surgical-orthodontic treatment that entailed extraction of the supernumerary teeth and subsequent orthodontic treatment, with restoration of a normal occlusion. In conclusion, management of patients suffering from very rare pathologies should be carefully evaluated in relation to clinical characteristics and possible local and systemic complications. The aim of this study is to illustrate two cases managed in a paradigmatically opposite way. It is paramount for the surgeon to intercept cases of hyperodontia typical of CCD at an early phase. It is possible, through correct surgical-orthodontic treatment, not only to avoid local and general complications, but also to achieve proper occlusion.
authors.affiliation: Department of Medical Science, University of Eastern Piedmont B, Amedeo Avogadro Faculty of Medicine, Novara, Italy. carmenmortellaro@hotmail.com
authors.forename: C
authors.initials: C
authors.lastname: Mortellaro
authors.forename: A
authors.initials: A
authors.lastname: Greco Lucchina
authors.forename: E
authors.initials: E
authors.lastname: Prota
mesh_terms: D000328:Adult; D002648:Child; D002973:Cleidocranial Dysplasia / Q000175:diagnosis / Q000601:surgery*; D057210:Delayed Diagnosis; D003951:Diagnostic Errors; D004954:Esthetics; D006801:Humans; D008297:Male; D019647:Oral Surgical Procedures / Q000379:methods*; D014078:Tooth Eruption; D014081:Tooth Extraction; D014095:Tooth, Impacted / Q000235:genetics / Q000601:surgery; D014096:Tooth, Supernumerary / Q000235:genetics / Q000601:surgery*; D016896:Treatment Outcome