Cleidocranial Dysplasia: Diagnostic Criteria and Combined Treatment

Abstract

Cleidocranial dysplasia (CCD) is an uncommon, generalized skeletal disorder characterized by delayed ossification of the skull, aplastic or hypoplastic clavicles, and serious, complex dental abnormalities. There are many difficulties in the early diagnosis of CCD because a majority of the craniofacial abnormalities becomes obvious only during adolescence. In the present case, a hypoplastic midface, a relative prognathia of the mandible, and close approximation of the shoulders in the anterior plane were the conspicuous extraoral findings. Prolonged exfoliation of the primary dentition, unerupted supernumerary teeth, and the irregularly and partially erupted secondary dentition produced occlusional anomalies. The presence of the second permanent molars together with the primary dentition and wide spacing in the lower incisor area were typical dental signs. Gradual extraction of the supernumerary teeth and over-retained primary teeth was the first step of oral surgery. This was followed by a surgical exposure of the unerupted teeth by thinning of the cortical bone. Orthodontic treatment was aimed at parallel growth of the jaws. Removable appliances were used to expand the narrow maxillary and mandibular arches, and a Delaire mask compensated for the lack of sagittal growth of the upper jaw. Temporary functional rehabilitation was solved by partial denture. When the jaws have been fully developed, implant insertions and bridges are the therapeutic measures. The reported case and the literature data support the importance of the early diagnosis and interdisciplinary treatment of CCD.

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]. Bedingt durch überzählige Zähne können Parodontopathien, chronische Abszesse und Schmerzen im Kieferbereich begünstigt werden.…”

“…Aus diesen Gründen stellt die CCD eine für die Zahnmedizin und insbesondere die Kieferorthopädie relevante Erkrankung dar. Eine hohe Dunkelziffer an nicht diagnostizierten Fällen ist zu vermuten, auch weil sich bei vielen Patienten die kraniofazialen Symptome erst während der Adoleszenz bemerkbar machen [20,81].…”

“…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]. The presence of supernumerary teeth may encourage the development of periodontal diseases, chronic abscesses and pain in the jaw region.…”

“…This is why CCD is relevant to dentistry and orthodontics in particular. It is reasonable to suspect that many cases go undiagnosed, partly because the craniofacial symptoms only become noticeable during adolescence in many patients [20,81].…”

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

“…Dental team management involving an orthodontist, Fig. 5 All unerupted teeth were evaluated by MPR images from cone-beam CT after the first operation oral and maxillofacial surgeon, oral radiologist, and prosthodontist is required with good communication and cooperation from the patient [10,11]. We thought that the operation time for impacted teeth would be reduced if the oral surgeon fully understood the condition of the impacted teeth before the operation.…”