Disóstosis cleidocraneal. Presentación de un caso clínico

Abstract

in 1897, is characterized by complete or incomplete clavicular aplasia, an increased transverse cranial diameter, delayed ossification of the sutures and fontanelles, bone malformations, dental anomalies and hereditary transmission. These clinical features condition the extraoral appearance of these patients: brachycephalia with frontal and parietal bulging, a depressed and broadened nasal dorsum, hypertelorism, pronounced nasolabial sulci and a low body height. The clavicular deficiency gives the neck an elongated appearance, with narrow shoulders that can be approximated across the chest. Maxillary and dental anomalies are a practically constant finding at oral level, and comprise delayed resorption of the roots of the deciduous dentition, delayed eruption of the permanent teeth with ectopic locations, and morphological anomalies of the latter, the presence of multiple retained teeth (mainly supernumerary), the development of dentigerous cysts around the unerupted molars, and a scant development of the upper jaw. Mandibular development is normal, though adult patients lack fusion of the mandibular symphysis -probably as a result of the generalized delay in skeletal development and ossification observed in these individuals. The dental treatment of such patients is slow and difficult, and often requires a multidisciplinary management approach. The case of an adult male with cleidocranial dysostosis is presented, with a description of the salient clinical, diagnostic and therapeutic characteristics at both dental and craniofacial level.