Cleidocranial dysplasia: maxillary alterations on the transverse plane. Presence of crown-radicular anomalies and multidisciplinary approach of a clinical case.

Abstract

Cleidocranial Dysplasia (CCD) is a rare inherited autosomal dominant congenital syndrome that occurs in approximately one out of every one million individuals worldwide; it primarily affects bones that undergo intra-membranous ossification, generally the skull and clavicles. Other bones may be affected such as the long bones, spine, pelvis, bones of hands and feet showing hypoplasia of distal phalanges. Indispensable is the role of the gene Runx2, necessary for the differentiation of odontoblasts and osteoblasts; it regulates the expression of many genes related to the development of dental hard tissues. The aim of this study was to appraise the connection between the Cleidocranial Dysplasia and the appearance of skeletal and dental anomalies not much deepen to this day. With particular emphasis, it wants to describe the multidisciplinary therapeutic approach. The patient showed multiple skeletal features of CCD. A distinctive feature was the failed or delayed exfoliation of deciduous dentition and a delayed eruption of permanent teeth. The goal of the treatment is the improvement of both aesthetic and functional aspects. This objective can be achieved through an appropriate multidisciplinary treatment plan that arranges the orthodontic and surgical measures. Because of the involvement of facial bones, the altered mode of tooth eruption and the presence of numerous included supernumerary teeth, CCD is a pathology that all dentists should be familiar with. The patients have small faces compared to the skull and the hypoplasia of maxillary, tear, nasal and zygomatic bones. The orthodontic approach in literature seems to be reduced to the guided eruption of bad-positioned and impacted teeth. Knowledge of the clinical features of CCD allows for the early planning of the procedures necessary to resolve the dental pathologies observed in CCD patients.