Craniofacial changes and treatment of the stomatognathic system in subjects with Cleidocranial Dysplasia

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ABSTRACT

Aim

Cleidocranial dysplasia (CCD) is a rare disorder that is inherited as an autosomal genetic trait. It is
characterised by defective ossification, delayed bone and tooth development, stomatognathic and craniofacial abnormalities
and it is caused by mutations in the RUNX2 gene that is responsible for osteoblast differentiation. CCD is a dental disease
that needs complex rehabilitation and the assistance of several specialists. In most cases, this disease precipitates towards
the end of childhood with the progressive morbidity of the deciduous dentition, thus leading to edentulism at a young age
and giving patients an older appearance. Several therapeutic approaches have been proposed within literature. The aim of
this paper is to revise the literature on the proposed therapeutic approaches for the functional and aesthetical rehabilitation
of the typical defects caused by CCD in the cephalic region, and to identify the most effective therapy currently available.

Conclusion

The most effective therapeutic approach for the early treatment of the orthodontic and orthopaedic diseases
in CCD patients would start with an orthopaedic treatment of the upper jaw followed by orthodontic and surgical treatments.
When growth is completed a reintervention for maxillofacial surgery and the orthodontic treatment may be required. Finally, if
some teeth are missing. function and aesthetics can be achieved replacing them with prosthesis.

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