scholarly journals Genetic Pattern, Orthodontic and Surgical Management of Multiple Supplementary Impacted Teeth in a Rare, Cleidocranial Dysplasia Patient: A Case Report

Medicina ◽  
2021 ◽  
Vol 57 (12) ◽  
pp. 1350
Author(s):  
Alessio Danilo Inchingolo ◽  
Assunta Patano ◽  
Giovanni Coloccia ◽  
Sabino Ceci ◽  
Angelo Michele Inchingolo ◽  
...  
Keyword(s):  
Permanent Teeth ◽  
Cleidocranial Dysplasia ◽  
Supernumerary Teeth ◽  
Tooth Formation ◽  
Impacted Teeth ◽  
Surgery Outcome ◽  
Molecular Features ◽  
Tissue Healing ◽  
Related Transcription Factor ◽  
Delayed Closure

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.

scholarly journals Orthodontic Treatment in a Patient with Cleidocranial Dysostosis

2009 ◽  
Vol 79 (1) ◽  
pp. 178-185 ◽  
Author(s):  
Giampietro Farronato ◽  
Cinzia Maspero ◽  
Davide Farronato ◽  
Silvia Gioventù
Keyword(s):  
Ethnic Group ◽  
Orthodontic Treatment ◽  
Primary Dentition ◽  
Permanent Teeth ◽  
Supernumerary Teeth ◽  
Impacted Teeth ◽  
Cleidocranial Dysostosis ◽  
Skeletal Disorder ◽  
Maxilla And Mandible ◽  
Delayed Closure

Abstract Cleidocranial dysostosis is a rare congenital skeletal disorder, associated with clavicular hypoplasia or aplasia, delayed closure of cranial fontanels, brachycephalic skull, delayed exfoliation of primary dentition, eruption of permanent teeth, and multiple supernumerary and morphologic abnormalities of the maxilla and mandible. The disorder is caused by mutation in the CBFA1 gene, on the short arm of chromosome 6p21. The prevalence of cleidocranial dysostosis is estimated one per million, without sex or ethnic group predilection. The purpose of this paper is to describe the orthodontic treatment in a patient with cleidocranial dysostosis. Therapy may include removal of supernumerary teeth, surgical exposure of impacted teeth, and orthodontic treatment.

scholarly journals Cleidocranial dysplasia-dental disorder treatment and audiology diagnosis

Open Medicine ◽  
2018 ◽  
Vol 13 (1) ◽  
pp. 1-8 ◽  
Author(s):  
Teresa Matthews-Brzozowska ◽  
Dorota Hojan-Jezierska ◽  
Wawrzyniec Loba ◽  
Marta Worona ◽  
Artur Matthews-Brzozowski
Keyword(s):  
Hearing Loss ◽  
Conductive Hearing Loss ◽  
Case Reports ◽  
Permanent Teeth ◽  
Deciduous Teeth ◽  
Cleidocranial Dysplasia ◽  
Supernumerary Teeth ◽  
The Many ◽  
Delayed Closure ◽  
Conductive Hearing

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.

scholarly journals Cleidocranial Dysplasia Causing Respiratory Distress in Neonates: A Case Report and Literature Review

2021 ◽  
Vol 12 ◽  
Author(s):  
Ru Xue ◽  
Guoqing Zhang ◽  
Xiafang Chen ◽  
Xiuxia Ye
Keyword(s):  
Respiratory Distress ◽  
Pectus Excavatum ◽  
Cleidocranial Dysplasia ◽  
Inherited Disease ◽  
Supernumerary Teeth ◽  
Adequate Treatment ◽  
Related Transcription Factor ◽  
Ear Infections ◽  
Standard Medical Treatment ◽  
Delayed Closure

Cleidocranial dysplasia (CCD; OMIM 119600) is a rare autosomal dominant skeletal dysplasia, which is mainly characterized by persistently open or delayed closure of fontanelle, patent skull sutures, abnormal clavicles, pectus excavatum, short stature, supernumerary teeth, and sinus and middle ear infections. It is caused by Runt-related transcription factor 2 (RUNX2; OMIM 600211) mutations. Herein, we present a rare case of CCD with neonatal respiratory distress, who had abnormal midfacial features and wide fontanelle. Also, pectus excavatum was noted. He was transferred to our department, administered standard medical treatment, and discharged after 4 weeks. Therefore, we recommend the early suspicion and identification of this rare inherited disease to adequate treatment.

Progressive Development of Supernumerary Teeth in Cleidocranial Dysplasia

1989 ◽  
Vol 16 (2) ◽  
pp. 103-106 ◽  
Author(s):  
K. Frame ◽  
R. I. W. Evans
Keyword(s):  
Treatment Period ◽  
Permanent Teeth ◽  
Cleidocranial Dysplasia ◽  
Supernumerary Teeth ◽  
Progressive Development ◽  
The Future

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.

scholarly journals Supernumerary teeth in patients with cleft lip and palate: the tooth germs do not separate

2021 ◽  
Vol 26 (4) ◽  
Author(s):  
Alberto CONSOLARO ◽  
Maria Carolina Malta MEDEIROS ◽  
Dario Augusto Oliveira MIRANDA ◽  
Ingrid Araújo de OLIVEIRA
Keyword(s):  
Cleft Lip ◽  
Cleft Lip And Palate ◽  
Permanent Teeth ◽  
Supernumerary Teeth ◽  
Human Teeth ◽  
Tooth Formation ◽  
Dental Lamina ◽  
The Face ◽  
Embryonic Origin ◽  
Tooth Germs

ABSTRACT Introduction: Supernumerary teeth in cases of cleft lip and palate do not result from the division of normal germs before the formation of hard tissue. Deciduous and permanent teeth odontogenesis begins after the face has formed, either with or without the cleft. Discussion: The most acceptable hypothesis to enable understanding of the presence of supernumerary teeth on one or both sides of the cleft palate is hyperactivity of the dental lamina in its walls. This hyperactivity, with the formation of more tooth germs, must be attributed to mediators and genes related to tooth formation, under strong influence of local epigenetic factors, whose developmental environment was affected by the presence of the cleft. Conclusion: The current concepts of embryology no longer support the fusion of embryonic processes for the formation of the face, but rather the leveling of the grooves between them. All human teeth have a dual embryonic origin, as they are composed of ectoderm and mesenchyme/ectomesenchyme, but this does not make it easy for them to be duplicated to form supernumerary teeth.

scholarly journals Non-syndromic multiple impacted teeth: two case reports

2018 ◽  
Vol 5 (11) ◽  
pp. 2798-2801
Author(s):  
Mohadeseh Delavarian ◽  
Fatemeh Delavarian ◽  
Mohammad Moslem Imani
Keyword(s):  
Case Reports ◽  
Systemic Disease ◽  
Interdisciplinary Approach ◽  
Permanent Teeth ◽  
Systemic Diseases ◽  
Supernumerary Teeth ◽  
Facial Aesthetics ◽  
Impacted Teeth ◽  
Rare Finding ◽  
Unerupted Teeth

Multiple impacted permanent teeth is a rare finding and often associated with systemic diseases or some rare syndromes. This article reports two Iranian cases with multiple impacted teeth without systemic disease or syndromes. The first patient, a 17-year-old male, had 18 unerupted teeth and 9 unerupted supernumerary teeth. The second patient, a 16-year-old female, had 14 unerupted teeth. In these cases, an interdisciplinary approach between orthodontist, oral surgeon and prosthodontist is required to achieve optimum facial aesthetics and functional occlusion.

scholarly journals Hypodontia and Delayed Dentition as the Primary Manifestation of Cleidocranial Dysplasia Presenting with a Diagnostic Dilemma

2012 ◽  
Vol 2012 ◽  
pp. 1-4 ◽  
Author(s):  
Radhika Chopra ◽  
Mohita Marwaha ◽  
Payal Chaudhuri ◽  
Kalpana Bansal ◽  
Saurabh Chopra
Keyword(s):  
Family History ◽  
Tooth Development ◽  
Cleidocranial Dysplasia ◽  
Supernumerary Teeth ◽  
Diagnostic Dilemma ◽  
Girl Child ◽  
Delayed Closure

Cleidocranial dysplasia is a rare autosomal disorder which manifests as partial or complete absence of clavicles, multiple supernumerary teeth, and delayed closure of fontanelle. Classical cases of cleidocranial dysplasia are easily diagnosed very early in the life. However, cases with partial manifestation of the syndrome and noncontributory family history are difficult to diagnose. Here, we report a case of 8.5-year-old girl child who presented with delayed tooth development (without any supernumerary teeth), anterior open fontanelle, and normal clavicles, thus resulting in a diagnostic dilemma.

scholarly journals Clinical and radiological findings in a severe case of cleidocranial dysplasia

2018 ◽  
pp. bcr-2018-226671 ◽  
Author(s):  
Priti P Lotlikar ◽  
Adriana G Creanga ◽  
Steven R Singer
Keyword(s):  
Autosomal Dominant ◽  
Treatment Guidelines ◽  
Severe Case ◽  
Permanent Teeth ◽  
Deciduous Teeth ◽  
Cleidocranial Dysplasia ◽  
Supernumerary Teeth ◽  
Radiological Findings ◽  
Recommended Treatment ◽  
Dominant Condition

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.

scholarly journals Cleidocranial dysplasia. A molecular and clinical review.

2018 ◽  
Vol 8 (1) ◽  
pp. 35-38
Author(s):  
Andrea Avendaño ◽  
Francisco Cammarata-Scalisi ◽  
Mochamad Fahlevi Rizal ◽  
Sarworini Bagio Budiardjo ◽  
Margaretha Suharsini ◽  
...  
Keyword(s):  
Transcription Factor ◽  
Molecular Mechanisms ◽  
Target Genes ◽  
Autosomal Dominant ◽  
Cleidocranial Dysplasia ◽  
Supernumerary Teeth ◽  
Phenotypic Characteristics ◽  
Autosomal Dominant Disorder ◽  
Dental Abnormalities ◽  
Related Transcription Factor

Cleidocranial dysplasia (CCD) is a rare autosomal dominant disorder characterized by skeletal and dental abnormalities primarily, short stature, aplasia or hypoplasia of clavicles, open fontanelles and supernumerary teeth. Heterozygous mutations of the runt related transcription factor 2 (RUNX2) gene have been found in approximately 60-70% of cases leaving a large number of cases with no defined genetic cause which led us to delve into molecular mechanisms underlying CCD and thus to detect potential target genes to be explored in these patients. In this review we also highlight very broadly the phenotypic characteristics of previously reported patients with CCD.

scholarly journals Multiple Impacted Teeth: Report of 3 Cases

2008 ◽  
Vol 02 (01) ◽  
pp. 73-78 ◽  
Author(s):  
Gürkan Raşit Bayar ◽  
Kerim Ortakoḡlu ◽  
Metin Sencimen
Keyword(s):  
Young Male ◽  
Rare Condition ◽  
Radiographic Examination ◽  
Cleidocranial Dysplasia ◽  
Supernumerary Teeth ◽  
Impacted Teeth ◽  
Young Males ◽  
Edentulous Mandible ◽  
Unerupted Teeth ◽  
Histopathological Studies

ABSTRACTWhile impaction of tooth is widespread, multiple impacted teeth by itself is a rare condition and often found in association with syndromes such as cleidocranial dysplasia or Gardner’s syndrome. A light of radiographic examination, we describe three Turkish young males with multiple impacted teeth who didn’t possess any systemic conditions or syndromes involving both jaws. The first patient, a 21-year-old young male, had 16 unerupted teeth and 5 unerupted supernumerary teeth. The second patient with totally edentulous mandible, a 20-year-old young male, had 31 unerupted teeth. The third patient, a 21-year-old young male, had 22 unerupted teeth and 4 unerupted supernumerary teeth. Based on the clinical presentation, radiographic examination and histopathological studies, this paper discusses the differential diagnosis and management of such cases. (Eur J Dent 2008;2:73-78)

Sign in / Sign up

Export Citation Format

Share Document