Cleidocranial dysostosis is a rare congenital skeletal disorder, associated with clavicular hypoplasia or aplasia, delayed closure of cranial fontanels, brachycephalic skull, delayed exfoliation of primary dentition, eruption of permanent teeth, and multiple supernumerary and morphologic abnormalities of the maxilla and mandible. The disorder is caused by mutation in the CBFA1 gene, on the short arm of chromosome 6p21. The prevalence of cleidocranial dysostosis is estimated one per million, without sex or ethnic group predilection. The purpose of this paper is to describe the orthodontic treatment in a patient with cleidocranial dysostosis. Therapy may include removal of supernumerary teeth, surgical exposure of impacted teeth, and orthodontic treatment. (Angle Orthod. 2009;79:178-185.)
The genetic data obtained in this study in children with Class III malocclusion and sleep-related breathing disorders provide new information useful to the genetic characterization of this pathology. The PHOX2B gene silent mutations can lead to structural and functional modification of their product providing to a group of children with Class III malocclusion similar features to those of CCHS (sleep apnea episodes and craniofacial malformations).
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