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Concept: Hyperdontia

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BACKGROUND: Cleidocranial dysplasia (CCD) is a rare congenital autosomal dominant skeletal disorder. The disorder is caused by heterozygosity of mutations in human RUNX2, which is present on the short arm of chromosome 6p21. The incidence of CCD is one per million births. CCD appears spontaneously with no apparent genetic cause in approximately 40% of affected patients, and one in three patients has unaffected parents. The most prevalent features associated with CCD are aplastic or hypoplastic clavicles, supernumerary teeth, failed eruption of permanent teeth, and a hypoplastic maxilla. CASE PRESENTATION: A 13-year-old Caucasian boy presented with a chief complaint of delayed eruption of the permanent anterior teeth. The patient was subsequently diagnosed with CCD based on the clinical examination, panoramic X-ray, anterior-posterior and lateral cephalogram, and chest radiograph findings. The details of this case are herein reported because of the extremely low incidence of this disorder. CONCLUSIONS: CCD is of clinical importance in dentistry and medicine because it affects the bones and teeth and is characterized by many changes in skeletal patterning and growth. Particularly in dentistry, CCD is of great clinical significance because is associated with delayed ossification of the skull sutures, delayed exfoliation of the primary teeth, lack of permanent teeth eruption, multiple supernumerary teeth, and morphological abnormalities of the maxilla and mandible. Patients with CCD seek treatment mainly for dental problems. Knowledge of the pathogenesis, clinical characteristics, and diagnostic tools of CCD will enable clinicians to render the appropriate treatment to improve function and aesthetics. Early diagnosis of CCD is crucial for timely initiation of an appropriate treatment approach.

Concepts: Medical terms, Medical statistics, Chromosome, Teeth, Hyperdontia, Dental anatomy, Cleidocranial dysostosis, Permanent teeth

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Supernumerary teeth are an uncommon occurrence that have the ability to arise anywhere in the dental arch. Mesiodens is a specific type of supernumerary tooth that occurs in the vicinity of the maxillary central incisors. Mesiodens are the most common among all supernumerary teeth, almost always impacted, and may also be seen in various positions based on the eruption status. The formation of mesiodens is a much-debated topic and many theories have been proposed. The reports also include its common radiographic findings and a pertinent literature review. This study reports a case of an impacted, inverted mesiodens incidentally discovered on a preimplant examination of maxilla. This article demonstrates the scope of eruption of an impacted and inverted mesiodens. Various methods of treatment plan are discussed and a new classification system of mesiodens is proposed.

Concepts: Medical imaging, Teeth, Maxillary central incisor, Hyperdontia, Oral pathology, Supernumerary body parts

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A 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.

Concepts: Medical imaging, Auditory system, Otology, Teeth, Hyperdontia, Deciduous teeth, Fontanelle, Cleidocranial dysostosis

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Cleidocranial dysplasia (CCD, MIM 119600) is a rare autosomal dominant disorder affecting bone, cartilage, craniofacial growth, and tooth formation leading to supernumerary teeth. Few reports delineate the genotype-phenotype correlations related to the variations in craniofacial morphology and patterning of the dentition and the complexity of treating patient’s malocclusion. Successful management of the craniofacial deformities in patients with CCD requires a multidisciplinary team of healthcare specialists. Approximately 70% of patients are due to point mutations in RUNX2 and <20% due to copy number variations with the remainder unidentified. There is no literature to date, describing the orthognathic management of CCD patients with deletion in one of the RUNX2 alleles. The purpose of this study was to evaluate the craniofacial morphology and dental patterning in a 14-year-old Caucasian female with CCD resulting from a novel microdeletion of RUNX2 in 1 allele. The CCD patient with RUNX2 haploinsufficiency due to microdeletion had decreased craniofacial bone and ankyloses in the permanent dentition. An altered extraction protocol of supernumerary teeth was followed in this patient. Craniofacial growth and morphologic analysis demonstrated atypical skull shape, persistent metopic suture, and decreased mandibular size.

Concepts: Gene, Genetics, Allele, Teeth, Genetic disorders, Haploinsufficiency, Hyperdontia, Cleidocranial dysostosis

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This study aimed to compare the developmental timing and mineralization quality of mesiodentes, i.e., supernumerary teeth located mainly in the midline of the maxilla between the central incisors, with the developmental timing and mineralization quality of permanent and primary central incisors.

Concepts: The Central, Hyperdontia, Supernumerary body parts

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In this review, we aimed to depict the clinical, radiological, and genetic features of cleidocranial dysplasia (CCD) and to suggest management guidelines, based on our experience of 8 cases, with an emphasis given to dental complications.The most common craniofacial features of CCD that stand out are a patency of the anterior fontanelle, an inverted pear-shaped calvaria, a hypertelorism, a general midface retrusion, and a mandible prognathism, associated with an excessive mobility of the shoulders, a short stature, and teeth abnormalities such as supernumerary teeth and failure of eruption, in particular. RUNX2 is the only gene in which mutation is known to cause CCD, but mutations are detected in only 65% of all patients with a clinical diagnosis of CCD. Panoramic radiography is a valuable adjunct in confirming the diagnosis of CCD.Our experience allowed us to conclude that orthodontically aided eruption should always be attempted. However, to stabilize the occlusion and to improve facial esthetics, we recommend associated orthognathic surgery. When orthodontic treatment is partially efficient, prosthetic treatment options bring satisfactory results, in terms of occlusion. Nevertheless, when orthodontic treatment fails, we recommend to preserve as many native teeth as possible, and to combine orthognathic preprosthetic surgery and implant-supported prosthesis.In any case, an individualized treatment protocol, depending on the needs and demand of the patient, the age at diagnosis and social and economic circumstances, should be put forward.

Concepts: Genetics, Cancer, Oral and maxillofacial surgery, Orthodontics, Haploinsufficiency, Hyperdontia, Fontanelle, Cleidocranial dysostosis

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Mesiodentes are the most frequently encountered supernumerary teeth. Recently, reports of double mesiodens have increased, which may be due to the availability of better diagnostic methods. This article discusses 2 cases of double mesiodens with varying clinical presentations. In addition, the literature related to the etiology, clinical presentation, diagnosis, and management of mesiodentes is reviewed.

Concepts: Greek loanwords, Hyperdontia, Supernumerary body parts

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Numerical anomalies, either addition or deletion, are quite a common findings in human dentition. However, it is extremely rare to find both hypodontia and hyperdontia simultaneously in the same individual. This condition is referred as concomitant hypohyperdontia (CHH). Aetiology of this condition is still obscure. The prevalence of CHH has been reported to be between 0.002% and 3.1%. This case report highlights a rare occurrence of bimaxillary CHH represented by the absence of both mandibular central incisors and presence of two supernumerary teeth in the maxillary anterior segment. The rarity of such condition of mixed hypodontia as well as hyperdontia in single human dentition prompted the author to report the case.

Concepts: Teeth, Tooth, Maxillary central incisor, Hyperdontia, Mandibular central incisor, Hypodontia, Oral pathology, Supernumerary body parts

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Most mesiodens remain impacted and can affect the growth and development of adjacent permanent teeth. Impacted mesiodens are usually located in an intraosseous position associated with complicated anatomical structures, necessitating minimally invasive surgical approaches. This article demonstrates a simple customized surgical stent for extraction of impacted mesiodens. Its use and advantages are described.

Concepts: Medicine, Surgery, Physician, Minimally invasive, Anatomy, Hyperdontia, Supernumerary body parts

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Teeth in excess number than the normal count are called supernumerary teeth. They can be either unilateral or bilateral and single or multiple, found at any place in the dental arch, but most commonly found in the anterior maxillary region. Supernumerary teeth are commonly associated with syndromes when present in more numbers, but can be idiopathic. The present study shows a case report of a 16-year-old female patient who reported with overretained deciduous teeth. A panoramic radiograph showed multiple impacted supernumerary teeth. Based on clinical features and radiographic examination, a diagnosis of idiopathic hyperdontia was given. In such types of patients, management must be planned by a multidisciplinary approach.

Concepts: Medical terms, Medical imaging, Number, Teeth, Tooth development, Hyperdontia