An overview of the intraoral features and craniofacial morphology of growing and adult Japanese cleidocranial dysplasia subjects

Abstract

Background Orthodontic treatment for cleidocranial dysplasia (CCD) requires an understanding of the nature of the retained deciduous teeth, supernumerary teeth, delayed eruption of the permanent teeth, and craniofacial morphology from childhood to adulthood. This study aimed to provide an overview of the intraoral and craniofacial characteristics of growing and adult Japanese CCD subjects. Methods We assessed cross-sectionally the intraoral features of 28 CCD subjects (males, 15.3 ± 7.0 years; females, 15.2 ± 5.1 years) using orthopantomograms and photographs. Mean facial diagrams (profilograms) of 3 age groups (5–10 years, 11–14 years, over 15 years: adult) were constructed, and linear and angular measurements of 2 age groups (under 15 years, adult) were performed by using cephalograms. The data were compared with Japanese standards. Results A mean of 11.7 and 8.4 retained deciduous teeth, 10.4 and 15.8 erupted permanent teeth were observed in the adult males and females, and a mean of 6.8 and 5.3 supernumerary teeth were observed in all males and females, respectively. A positive correlation was found between the number of supernumerary teeth and the age at initial visit. Cephalometric analysis showed an average to anteriorly positioned maxilla, a tendency for counter-clockwise rotation of the ramus, and a prognathic mandible in all groups. Conclusions The number of supernumerary teeth increased with age. The maxilla was average to anteriorly positioned, and the mandible was counter-clockwise rotated and prognathic for all groups. These characteristic craniofacial morphologies and changes of intraoral conditions at different ages in CCD patients should be considered when proposing rational orthodontic treatment plans.

Treatment and genetic analysis of multiple supernumerary and impacted teeth in an adolescent patient

“…The patient presented with distinctive facial features and numerous supernumerary/impacted teeth, prompting suspicions of some potential syndromes, particularly CCD [ 21 – 23 ]. In light of this suspicion, chest radiography and genetic testing were conducted.…”

Amyloid-mediated remineralization for tooth hypoplasia of cleidocranial dysplasia

“…Typical clinical features include absent or hypoplastic clavicles, defective ossification of the anterior fontanelle, and supernumerary teeth (Sun et al, 2016;Zhang et al, 2022). In addition to clear systemic symptoms, dental anomalies such as skeletal class III tendency, high narrow arched palate, tooth hypoplasia, delayed eruption of permanent dentition and multiple impacted supernumerary teeth are characteristic abnormal oral manifestations, that severely affect the oral health of patients (Shih-Wei Cheng et al, 2022;Yamashiro et al, 2022). Since the delayed eruption of permanent dentition and arrest of multiple impacted supernumerary teeth in the replacement of primary and permanent teeth, studies on CCD…”

“…Cleidocranial dysplasia (CCD) is an autosomal dominant heritable skeletal disease that affects cranial sutures, teeth, and clavicles, and the putative causative gene is Runt-related transcription factor 2 (RUNX2) (Xuan et al, 2010;Hordyjewska-Kowalczyk et al, 2019;Komori, 2020). Typical clinical dental features include skeletal class III tendency, tooth hypoplasia, delayed eruption of the permanent dentition, and multiple impacted supernumerary teeth (Shih-Wei Cheng et al, 2022). Currently, therapeutic strategies for CCD are still based on symptomatic treatment.…”

A Clinical Odyssey Involving Cleidocranial Dysplasia: A Rare Case Report

“…CCD's most distinguishing dental features are retained primary teeth and multiple supernumerary teeth as seen in this case. According to a study conducted by Cheng et al, the number of supernumerary teeth increases with age in patients with CCD [ 9 ]. Primary tooth retention, skeletal class III malocclusion, midfacial hypoplasia, and overclosure, all cause functional and aesthetic issues [ 10 ].…”