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A Journal on Dentistry and Maxillofacial Surgery

Official Journal of the Italian Society of Odontostomatology and Maxillofacial Surgery
Indexed/Abstracted in: CAB, EMBASE, Index to Dental Literature, PubMed/MEDLINE, Scopus, Emerging Sources Citation Index


Minerva Stomatologica 2010 March;59(3):117-27


Cleidocranial dysplasia: etiology and stomatognathic and craniofacial abnormalities

D’alessandro G., Tagariello T., Piana G.

Department of Dental Science, Dental School, Bologna University, Bologna, Italy

Cleidocranial dysplasia (CCD) is a rare disorder which is inherited as an autosomal genetic trait. It is characterized 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. The purpose of this review is to collect and analyze data in the literature on orofacial typical manifestations of the syndrome and to present knowledge of the eziopatogenics mechanisms of the CCD. Clinical, genetic, aetiopathogenetic studies on this syndrome were compliled through a systematic approach using Medline. This review reports the cranio-facial features and dental characteristics of the CCD on the basis of all data in the literature. This review pays particular attention on the eziopatogenics mechanisms of CCD and summarises the results of the most recent studies. Access to detailed review of the etiopathogenic mechanisms of CCD is a fundamental support for clinicians as it can allow to make an informed assessment regarding the most effective choice of therapy. The review shows how an interdisciplinary approach is necessary for an appropriate treatment since CCD patients suffer from a skeletal third class, transverse deficiency of the maxilla, supernumerary permanent teeth and deficient eruption of impacted permanent teeth.

language: English, Italian


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