PDF: Cleidocranial Dysostosis, a Case Report

Cleidocranial dysostosis is an autosomal dominant inherited condition transmitted by either sex and affecting men or women with equal frequency. The classic feature is at least one clavicle partly or completely absent.

Cleidocranial-Dysostosis


The skull has a brachycephalic appearance, and the closure of the sutures and fontanelles is delayed compared with normal. The fontanelles exhibit secondary ossification centers forming wormian bones. 

► Read also: PDF: Orthokeratinized Odontogenic Cyst: a report of two cases in the mandible

The skull can have parietal and frontal bossing. The patient tends to be short in stature with a narrow chest and sloping shoulders.

Unerupted permanent teeth, supernumerary teeth, and overretained deciduous teeth as well as delayed eruption of teeth, malocclusion, and impacted teeth arc the causes of aberrations in the development of the dentition. 

Many of the permanent teeth fail to erupt, although permanent teeth that have no deciduous predecessors, such as the molars, usually erupt normally. There is almost total absence of cellular or secondary cementum in all examined teeth. 

This may be related to the failure of eruption so frequently seen. Some of the impacted teeth may be associated with follicular cysts. There is apparently no predilection for race. Solitary cases with no apparent hereditary influence are occasionally reported as mutations (Lubowitz, 1968; Kelly & Nakamoto, 1974; Harris et al., 1977).



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