Dentomaxillofacial variability of cleidocranial dysplasia: clinicoradiological presentation and systematic review

Abstract

The diagnosis of CCD can be difficult when typical features are not clearly expressed. Since the multiple case studies concentrated on specific clinical aspects, an overall ranking including all associated findings was not possible. Owing to their prevalence, we recommend referencing to the described list of clinical signs as major symptoms for the pathognomy in CCD, since they are infrequent in other conditions and in the general population. To categorize the expression of CCD, more interdisciplinary studies are necessary. Nevertheless, a subjective classification is possible according to the related restrictions in the patients' quality of life.

Die Bedeutung von RUNX2 in der postnatalen Entwicklung des Kondylus

“…Diese beinhalten Milchzahnpersistenz, Retention permanenter Zähne, verspäteten Zahndurchbruch, Verlagerung permanenter Zähne, Hyperdontie permanenter Zähne, strukturelle Anomalien der Zahnwurzeln und Zementdysplasien [10,19,21,29,33,46,54,72]. Einen typischen Befund stellt die Anwesenheit der bleibenden Molaren bei Persistenz der ersten Dentition und Platzüberschuss in der unteren Frontzahnregion dar [72,81].…”

“…There are numerous case studies and review articles on CCD's clinical symptoms [4,9,12,19,21,26,32,40,46]. It is a rare syndrome inherited as an autosomal dominant trait.…”

“…Zur klinischen Symptomatik liegen zahlreiche Fallbeschreibungen sowie Über-sichtsarbeiten vor [4,9,12,19,21,26,32,40,46]. CCD ist ein seltenes Syndrom mit einem autosomal-dominanten Erbgang.…”

“…These involve the persistence of deciduous teeth, retention of permanent teeth, delayed eruption, displacement of permanent teeth, hyperdontia of permanent teeth, structural abnormalities of tooth roots and cemental dysplasia [10,19,21,29,33,46,54,72]. Typical is the presence of permanent molars while the first dentition persists, and excess space in the lower anterior region [72,81].…”

Use of cone-beam CT in a patient with cleidocranial dysplasia dramatically reduced the operation time

“…It occurs in fewer than one person per million [1]. More than 90% of cases have prolonged retention of primary teeth, supernumerary teeth, and eruption failure.…”