Progressive Development of Supernumerary Teeth in Cleidocranial Dysplasia

1989 ◽  
Vol 16 (2) ◽  
pp. 103-106 ◽  
Author(s):  
K. Frame ◽  
R. I. W. Evans

A 9-year-old boy suffering from cleidocranial dysplasia presented with multiple unerupted permanent teeth and five unerupted supernumerary teeth. During a 4-year observation and treatment period he developed nine more supernumerary teeth at a time when normally new teeth would no longer be forming. By the age of 13 years and 5 months a total of fourteen supernumerary teeth had been produced and it is possible that more teeth may develop in the future.

Medicina ◽  
2021 ◽  
Vol 57 (12) ◽  
pp. 1350
Author(s):  
Alessio Danilo Inchingolo ◽  
Assunta Patano ◽  
Giovanni Coloccia ◽  
Sabino Ceci ◽  
Angelo Michele Inchingolo ◽  
...  

Background: Cleidocranial dysplasia (CCD) is a rare, autosomal dominant skeletal dysplasia with a prevalence of one per million births. The main causes of CCD are mutations in the core-binding factor alpha-1 (CBFA1) or runt-related transcription factor-2 (RUNX2), located at the 6p21 chromosomal region. RUNX2 plays important roles in osteoblast differentiation, chondrocyte proliferation and differentiation, and tooth formation. The disease is characterized by clavicular aplasia or hypoplasia, Wormian bones, delayed closure of cranial suture, brachycephalic head, maxillary deficiency, retention of primary teeth, inclusion of permanent teeth, and multiple supernumerary teeth. Materials and Methods: A 22-year-old girl suffering from cleidocranial dysplasia with short stature, narrow shoulders, craniofacial manifestations (short face, broad forehead, etc.) and dental anomalies (different lower dental elements under eruption, supernumerary and impacted multiple teeth, etc.) was examined at our service (Complex Operative Unit of Odontostomatology of Policlinico of Bari). RX Orthopantomography (OPG) and cone beam computed tomography (CBCT) were requested to better assess the position of the supernumerary teeth and their relationships with others and to evaluate the bone tissue. Results: Under eruption was probably caused by dental interferences with supernumerary teeth; hence, extractions of supernumerary upper canines and lower premolars were performed under general anaesthesia. Surgery outcome was excellent with good tissue healing and improvements in the therapeutic possibilities with future orthodontics. Conclusions: The objective of this article is to give an update about radiological, clinical, and molecular features of CCD and to alert the health team about the importance of establishing an early diagnosis and an appropriate treatment in these patients to prevent impacted teeth complications and to offer them a better quality of life.


2018 ◽  
pp. bcr-2018-226671 ◽  
Author(s):  
Priti P Lotlikar ◽  
Adriana G Creanga ◽  
Steven R Singer

Cleidocranial dysplasia (CCD) is a rare congenital autosomal dominant condition, causing hypoplasia of the clavicle, abnormal formation of teeth, skeletal and craniofacial bones. CCD is caused by the mutation of RUNX2/CBFA1 present in the short arm of chromosome 6 at position 21.1, a transcription factor essential for the formation of teeth, cartilage and bone. Patients with CCD show the classical features of excessive mobility of the shoulder bone, lack of resorption of the deciduous teeth, failure to erupt permanent teeth, multiple impacted and supernumerary teeth, and open fontanelle and sutures of the skull. In this article we report a case of CCD in a 16-year-old male patient, with an aim to highlight the clinical, radiological and recommended treatment guidelines.


2020 ◽  
Vol 9 (9) ◽  
pp. e916998052
Author(s):  
Eleonor Álvaro Garbin Júnior ◽  
Anna Carolina Jaccottet Oliveira ◽  
Niviane Dorigan Vidor ◽  
Mauro Carlos Agner Busato ◽  
Geraldo Luiz Griza ◽  
...  

Cleidocranial dysplasia (CCD) is a rare syndrome, occurring at a rate of 1:10,000,000 in the form presented in this clinical case. This syndrome occurs due to an autosomal dominant inheritance characterized by changes in skeletal formation and development, as a result of a mutation associated with the RUNX2 gene, the main regulatory gene for osteoblastic differentiation, resulting in manifestations such as hypoplastic or aplastic clavicles, brachycephaly, hypoplasia of the middle third of the face, and delay or non-closure of fontanelles. Specifically, in the intraoral region, atypical dental manifestations occur, such as failure in the exfoliation of the primary dentition, delayed eruption of permanent teeth, and multiple supernumerary teeth. Thus, this study aims to present a case report of a patient with the pathognomonic characteristics of the syndrome in its rarest form, as well as the radiographic, imaging and clinical manifestations that allow its diagnosis and a discussion on the common manifestations in such patients, forms of treatment, and the conduct of treatment according to the specific needs of that case..


Open Medicine ◽  
2018 ◽  
Vol 13 (1) ◽  
pp. 1-8 ◽  
Author(s):  
Teresa Matthews-Brzozowska ◽  
Dorota Hojan-Jezierska ◽  
Wawrzyniec Loba ◽  
Marta Worona ◽  
Artur Matthews-Brzozowski

AbstractA review of numerous case reports was made, in order to demonstrate the possibilities for treatment of dental disorders in patients with Cleidocranial dysplasia (CCD). In this paper, our own report, including a diagnosis of the effect on the auditory system, is presented. In addition to the triad of CCD symptoms that include hypoplastic or aplastic clavicles, impacted and supernumerary teeth, delayed closure of fontanelles and cranial sutures, impairment of the hearing system resulting in conductive hearing loss also occurs. Our own report is based on the case of a 12-year-old CCD patient, in whom Cone Beam Computed Tomography (CBCT) revealed the presence of 12 supernumerary teeth. Furthermore, a clinical examination pointed to the presence of retained deciduous teeth and a delayed eruption of permanent teeth. Orthodontic-surgical procedures were implemented, in accordance with the literature. During the course of the orthodontic treatment, a decrease in auditory sensitivity was observed, for which reason hearing tests were also performed. Conductive hearing loss was detected. As such, it is important to remember that in such cases, auditory check-ups need to be performed between the many surgical and orthodontic interventions, which usually last a few years.


2018 ◽  
Vol 55 (4) ◽  
pp. 582-589 ◽  
Author(s):  
Elaine Li Yen Tan ◽  
Meaw Charm Kuek ◽  
Hung Chew Wong ◽  
Serene Ai Kiang Ong ◽  
Mimi Yow

Objective: Children with cleft lip and palate are reported to be commonly associated with higher prevalence of dental anomalies such as hypodontia, supernumeraries, and abnormalities in tooth size, shape, and position. This study investigated the prevalence of dental anomalies in a longitudinal cohort of children with unilateral cleft lip and palate (UCLP). Design: The study was a retrospective analysis of radiographs, study models, and treatment notes. Patients: Sixty patients with repaired UCLP aged 13 years old with complete dental records dating from 5 years of age were included. Methods: Study casts, dental panoramic, anterior maxillary occlusal, and periapical radiographs of the patients were examined for cleft-sidedness, congenitally missing permanent teeth, supernumerary teeth, microdontic, and macrodontic teeth in the anterior maxillary region, presence of malformed permanent cleft-sided lateral incisor and its morphology (peg-shaped, conical shaped, canine-formed), positions of the permanent lateral incisors relative to the cleft side and presence of rotated cleft-sided central incisors. Results: Of the 60 patients studied, 63.3% had hypodontia, 21.7% had supernumerary teeth, 69.6% had microdontia, and 12.5% had macrodontia. All of the cleft-sided permanent lateral incisors had associated anomalies, with a large proportion (43.1%) missing; and when present in 31 subjects, the majority (90.3%) was positioned distal to the cleft. Most of the cleft-sided permanent central incisors were rotated if present, and prevalent at 86.7%. Conclusion: A high prevalence of dental anomalies was observed in this sample of children with UCLP.


Author(s):  
João Paulo Schwartz ◽  
Daniela Gamba Garib

Summary Background/Objectives This retrospective study evaluated the prevalence of dental anomalies of number in different subphenotypes of isolated cleft palate. Materials/Methods The sample comprised 26 individuals with submucous cleft palate (group S) and 68 individuals with complete cleft palate (group C) aged between 9 and 12 years from a single centre. Panoramic radiographs were evaluated regarding the presence of dental anomalies of number in permanent teeth. Intergroup comparison was performed using chi-square tests (P < 0.05). Results Tooth agenesis was found in 34.61 and 36.76 per cent of group S and group C, respectively. The most commonly missing teeth were the maxillary second premolar, maxillary lateral incisor, and mandibular second premolar. Supernumerary teeth were found in none and 1.47 per cent of the individuals with submucous and complete cleft palate, respectively. No statistically significant difference was found between groups for the frequency of tooth agenesis and supernumerary teeth. Limitations Only dental anomalies of number were evaluated. Conclusions/Implications Individuals with submucous and complete cleft palate showed similar prevalence for tooth agenesis and supernumerary teeth. Dental anomalies frequency seems not to be a discriminator for subphenotypes of cleft palate.


2015 ◽  
Vol 5 (20) ◽  
pp. 229-231
Author(s):  
Atanas Vlaykov ◽  
Dian Sharlanov ◽  
Dilyana Vicheva

Abstract Background. Supernumerary teeth are described as an excess of the normal teeth number of 20 deciduous and 32 permanent teeth and can occur in any dental region. Material and methods. The authors present the case of a 12-year-old female child with a paramolar in the maxilla, discovered accidentally during an orthopantomogram, emphasising the treatment modality and the complications that can appear. Conclusion. Supernumerary teeth can be present in any region of the oral cavity. Both practitioners and clinicians should be aware of the various types of paramolars and make a treatment plan after an accurate clinical and radiographic examination.


2011 ◽  
Vol 36 (2) ◽  
pp. 207-210 ◽  
Author(s):  
Rekhalakshmi Kamatham ◽  
J Sharada ◽  
Abinash Mohapatra ◽  
Sivakumar Nuvvula

Full blown cases of cleidocranial dysplasia (CCD) have been reported earlier, but a case with a rarity of 60 teeth associated with bony malformations, is seldom observed. Because of the oral findings this condition has been diagnosed at an early age, thus helping to achieve a better oral harmony. This article reports an atypical case with 16 supernumerary teeth associated with bony malformations.


2019 ◽  
Vol 43 (4) ◽  
pp. 244-251 ◽  
Author(s):  
Tamar Finkelstein ◽  
Yehoshua Shapira ◽  
Aikaterini Maria Pavlidi ◽  
Shirley Schonberger ◽  
Sigalit Blumer ◽  
...  

Background: Supernumerary teeth are one of the most common anomalies in the human dentition, found most frequently in the maxillary anterior region causing impaction or displacement of the adjacent permanent teeth. Aim :The purpose of this retrospective study was to determine the prevalence and characteristics of supernumerary teeth in orthodontically treated patients. Study design: Pre-treatment facial and intraoral photographs, study models, panoramic and periapical radiographs of 3,000 consecutively treated orthodontic patients (mean age 12.2 years) from the Department of Orthodontics Tel Aviv University, were examined to detect supernumerary teeth in both arches. They were recorded according to gender, age, number, location, position and morphology. Results: Thirty-six patients, 22 (61%) males and 14 (39%) females with 50 supernumerary teeth, of which 42 (84%) were found in the maxillary anterior region, and 8 (16%) in the mandible, presenting a prevalence of 1.2%. Conclusions: A prevalence of 1.2% was found in our study. The most common supernumerary tooth is mesiodens located at the maxillary anterior region. The characteristics of supernumeraries were based on their morphology, location and position. The most frequent complications caused were rotations, displacement and arrested eruption of maxillary incisors.


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