Conservative orthodontic and multidisciplinary approaches for patients with cleidocranial dysplasia in late adolescence or young adulthood.

This case series describes conservative orthodontic and multidisciplinary approaches for treating two patients diagnosed with cleidocranial dysplasia in late adolescence and young adulthood. Most of the impacted permanent teeth erupted spontaneously within 3 to 4 years after surgical extraction of the deciduous and supernumerary teeth. The remaining unerupted permanent teeth were facilitated with traction or extracted followed by implantation or restoration. Repositioning of the maxilla and mandible via orthognathic surgery was also applied to correct skeletal and occlusal discrepancies and lead to satisfying results.

Microdont Developing Outside the Alveolar Process and Within Oral Diffuse and Plexiform Neurofibroma in Neurofibromatosis Type 1.

BACKGROUND/AIM: Numerical aberrations of permanent dentition and dystopic tooth eruption are part of the phenotype of the tumor predisposition syndrome neurofibromatosis type 1 (NF1). In these cases, surplus tooth germs usually develop in the alveolar processes of the jaw. This report attests to the dystopic development of a dysplastic supernumerary tooth in NF1 arising outside the jaw. CASE REPORT: The 8-year-old male patient developed a microdont outside the bone and above the occlusal plane of the retained maxillary right second molar. The supernumerary tooth was completely embedded in oral soft tissue. Hyperplastic oral soft tissue in the molar region and microdont were excised. Specimen of the mucosa surrounding the teeth was interspersed with diffuse and plexiform neurofibroma. The retained upper right first molar emerged spontaneously within a few months after surgery. The upper right second molar did not change position. CONCLUSION: Odontogenesis can take place within tumorous oral mucosa in NF1. Surgical removal of the tumorous mucous membrane facilitates tooth eruption in some cases.

A rare case of accessory maxilla: a case report and literature review of Tessier no. 7 clefts.

Bilateral Tessier no. 7 clefts are rarely reported in the literature. Here, we describe the presence of accessory maxilla with supernumerary teeth in a patient who exhibited bilateral Tessier no. 7 clefts; the diagnosis was established based on the patient's history, clinical presentation, and computed tomography images. A review of the available literature revealed 24 patients with Tessier no. 7 clefts from 2000 to 2020, including our patient. The most common clinical manifestation in patients with Tessier no. 7 clefts comprises bilateral facial clefts. Additionally, Tessier no. 7 clefts are more frequently found in boys or men, rather than in girls or women. The presence of an accessory maxilla with supernumerary teeth in a patient with bilateral Tessier no. 7 clefts is extremely rare. Early detection of craniofacial abnormalities is important, because it may influence patient prognosis and management.

Three-dimensional evaluation of morphology and position of impacted supernumerary teeth in cases of cleidocranial dysplasia.

Cleidocranial dysplasia (CCD) is a congenital anomaly characterized by the presence of impacted supernumerary teeth and delayed eruption of permanent teeth. However, there has been no detailed investigation on supernumerary teeth in patients with CCD using three-dimensional (3D) imaging techniques. The purpose of this study was to elucidate the morphology and position of supernumerary teeth using 3D images reconstructed from cone-beam computed tomography (CBCT) data in a group of five Japanese subjects (male, 3; female, 2; age, 15.0-25.4 years) with CCD. All five subjects exhibited supernumerary teeth (39 in total; average, 7.8; range, 1-15). All supernumerary teeth were impacted and existed as pairs with adjacent permanent teeth. Comparison of the size (the crown and dental-root lengths, the crown mesiodistal and buccolingual diameters), the number of cusps and dental roots, the position, and direction of supernumerary teeth in relation to the adjacent permanent teeth was analyzed. The results of relationship analyses revealed that, at sites other than the molar region, supernumerary teeth were positioned on the lingual and distal sides and supernumerary teeth resembled the morphology of their adjacent permanent teeth in terms of the number of cusps but were smaller than the adjacent permanent teeth. In the molar region, supernumerary teeth were microdontia, which were apparently small and obscure morphologically. In addition, while all adjacent permanent teeth exhibited normal direction, five supernumerary teeth exhibited inverse direction. The findings of this study will improve our understanding of the characteristics of CCD and provide important information for the pathophysiology and clinical treatment.

Endoscopically Assisted Modified Caldwell-Luc Approach to Enucleation of Dentigerous Cyst With Ectopic Tooth From the Maxillary Sinus.

Dentigerous cysts in the craniomaxillofacial complex are a benign pathology commonly associated with ectopically placed teeth. Owing to slow and asymptomatic growth, they often attain considerable size before diagnosis that may compromise adjacent anatomical structures. Various techniques for removal of dentigerous cysts from the maxillary sinus have been described. This case report demonstrates a hybrid endoscopically assisted modification of the Caldwell-Luc approach to provide the technical advantages of both techniques.

Characteristics of Supernumerary Teeth in Nonsyndromic Population in an Urban Dental School Setting.

PURPOSE: The purpose of this study was to analyze the clinical records of all patients evaluated for supernumerary teeth at the University of Illinois-Chicago Department of Oral and Maxillofacial Surgery postgraduate clinic from January 1, 2010 to October 31, 2015 to determine the demographics of impacted supernumerary teeth among different ethnicities in the city of Chicago. MATERIALS AND METHODS: A retrospective case series was completed on patients eligible for inclusion in the study who had a diagnosis of at least 1 full bony impacted supernumerary tooth (D7240). Other variables evaluated included date of surgical intervention, age at time of treatment, gender, ethnicity, location and number of supernumerary teeth present, and family history of supernumerary teeth. RESULTS: There were 459 male and 308 female patients (male-to-female ratio, 1.5:1). Patient ages ranged from 4 to 51 years (average, 13.1) at the time of surgery. Five hundred seventy-three patients (74.7%) were of Hispanic descent. Supernumerary teeth were found most frequently in the maxilla (69.9%), specifically in the central incisor region (526 teeth; 51.2%). Of the responding patient sample (258 patients), 53 patients (20.5%) reported a family history of supernumerary dentition. Of these 53 patients who reported a family history of supernumerary dentition, 37 (69.8%) were of Hispanic descent. Most of these Hispanic patients (83.8%) originated from Mexico. CONCLUSIONS: The incidence of supernumerary teeth is higher in male patients (male-to-female ratio, 1.5:1), and these teeth are most frequently found in the maxilla (69.9%), specifically the central incisor region (51.2%). A large percentage of patients (20.5%) also had first-degree relatives with a supernumerary dentition, and this fact might warrant further investigation into the possible genetics responsible for the formation of supernumerary teeth.

Bimaxillary concomitant hypohyperdontia in a 10-year-old child.

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.

Making extra teeth: Lessons from a TRPS1 mutation.

A Thai mother and her two daughters were affected with tricho-rhino-phalangeal syndrome type I. The daughters had 15 and 18 supernumerary teeth, respectively. The mother had normal dentition. Mutation analysis of TRPS1 showed a novel heterozygous c.3809_3811delACTinsCATGTTGTG mutation in all. This mutation is predicted to cause amino acid changes in the Ikaros-like zinc finger domain near the C-terminal end of TRPS1, which is important for repressive protein function. The results of our study and the comprehensive review of the literature show that pathways of forming supernumerary teeth appear to involve APC and RUNX2, the genes responsible for familial adenomatous polyposis syndrome and cleidocranial dysplasia, respectively. The final pathway resulting in supernumerary teeth seems to involve Wnt, a morphogen active during many stages of development. © 2016 Wiley Periodicals, Inc.

Syndromes with supernumerary teeth.

While most supernumerary teeth are idiopathic, they can be associated with a number of Mendelian syndromes. However, this can also be a coincidental finding, since supernumerary teeth occur in 6% or more of the normal population. To better define this relationship, we analyzed the evidence for specific associations. We excluded conditions with a single affected patient reported, supernumerary teeth adjacent to clefts or other forms of alveolar disruption (as secondary rather than primary findings), and natal teeth, which can involve premature eruption of a normal tooth. Since, the cause of supernumerary teeth shows considerable heterogeneity, certain findings are less likely to be coincidental, such as five or more supernumerary teeth in a single patient, or locations outside of the premaxilla. We found only eight genetic syndromes with strong evidence for an association: cleidocranial dysplasia; familial adenomatous polyposis; trichorhinophalangeal syndrome, type I; Rubinstein-Taybi syndrome; Nance-Horan syndrome; Opitz BBB/G syndrome; oculofaciocardiodental syndrome; and autosomal dominant Robinow syndrome. There is also suggestive evidence of an association with two uncommon disorders, Kreiborg-Pakistani syndrome (craniosynostosis and dental anomalies), and insulin-resistant diabetes mellitus with acanthosisnigricans. An association of a Mendelian disorder with a low frequency manifestation of supernumerary teeth is difficult to exclude without large numbers, but several commonly cited syndromes lacked evidence for clear association, including Hallermann-Streiff syndrome, Fabry disease, Ehlers-Danlos syndrome, Apert and Crouzon syndromes, Zimmermann-Laband syndrome, and Ellis-van Creveld syndrome. © 2016 Wiley Periodicals, Inc.

[Supernumerary nasal tooth a case report].

This case report describes a male patient, 5 years old, with recurrent nose congestion and fetid discharge from the right nasal cavity. The suspected clinical diagnosis of a supernumerary nasal tooth was confirmed by computed tomography (CT). Laboratory examination showed normal. After endoscopic removal in general anesthesia, the tooth was examined by pathological examination. A literature search identified 22 supernumerary nasal teeth in 21 patients.

Dientes supernumerarios en la región del tercer molar inferior: etiología y manejo quirúrgico: presentación de dos casos / Supernumerary teeth in the lower third molar region: etiology and surgical management: report of two cases

Los dientes supernumerarios en la región del tercer molar inferior sonpoco frecuentes. Cuando se presentan en posición distal se les denominadistomolares o distodens. Algunos autores los denominan cuarto molar; ésta denominación es la menos aceptada, ya que el cuarto molarno forma parte de la fórmula dental permanente, sino que es un dientesupernumerario. Cuando éste se presenta paralelo a los molares, en lacara externa o interna de la mandíbula, se denomina paramolar. El manejo quirúrgico suele ser complejo y requiere consideraciones especiales. Presentamos dos casos de dientes supernumerarios en la región del tercer molar: distomolar y paramolar; describimos su diagnóstico, etiología y la técnica quirúrgica empleada.

Supernumerary teeth in the lower third-molar region are rare. When these are located in the region posterior to the third molar, they are known as ®distomolars¼ or ®distodens¼. Some authors refer to them as ®fourth molars¼, a term that is somewhat less accepted due to the fact that the fourth molar is not part of the permanent dentition, but rather a supernumerary tooth. When a distomolar occurs parallel to the molars, either on the outer or inner surface of the jaw, it is known as a ®paramolar¼. Surgical management is often complex and requires special considerations. We report two cases of supernumerary teeth in the third-molar region, one distomolar and one paramolar, and describe their diagnosis, etiology, and the surgical technique employed.

[Supernumerary teeth in the maxilla and mandible-an interdisciplinary challenge. Part 1: epidemiology, etiology, classification and associated complications]. / Überzählige Zähne im Ober- und Unterkiefer – eine interdisziplinäre Herausforderung.

Supernumerary teeth develop in addition to the normal dentition and are a therapeutic challenge for pedodontists, orthodontists and oral surgeons alike. Therefore, interdisciplinary treatment concepts are needed for the benefit of the patient. In the present, two-parted literature review on supernumerary teeth, current classification, associated complications, diagnostic steps and different therapeutic approches are presented and discussed. Supernumerary teeth are diagnosed in the primary and permanent dentition. Supernumerary incisors, canines, premolars, and molars, as well as distomolars and mesiodentes can be seen according to the location of the supernumerary teeth. Furthermore, based on the morphology of the supernumeraries four different types can be differentiated: conical, tuberculate, supplemental, and odontoma. Supernumerary teeth often remain asymptomatic, and are only diagnosed incidentally during a routine dental visit on radiographs (often intraoral). Nevertheless, various complications have been reported in the literature for supernumerary teeth such as disturbed eruption, malpositioning/rotation, root resorption of neighbouring teeth, and development of dentigerous cysts in impacted supernumeraries. Root resorption of neighbouring teeth can be radiographically diagnosed in up to 22.8% of the cases.

Supernumerarios / Supernumerary teeth

En este artículo se describen los supernumerarios, las diversas teorías sobre su etiología, su frecuencia y lasalteraciones que pueden producir. Además, se dan pautas para su tratamiento. Los supernumerarios son anomalías del desarrollo que se encuentran con poca frecuencia, del 0,30 por ciento al 3,80 por ciento de la población, pero en pacientes con labio ypaladar fisurado pueden llegar al 28 por ciento. Se los puede definir como dientes adicionales a la serie dental normal. Se los encuentra raramente en ladentición primaria, en esta la distribución por sexo es similar; mientras que en la dentición permanente el sexo masculino prevalece en una proporción de 2 a 1. El mesiodens es el que se encuentra con mayor frecuencia. Constituye, según Shafer y Cols, más del50 por ciento de todos los supernumerarios...

[Nasal cavity supernumerary tooth and maxillary sinusitis: one case reported].

Supernumerary tooth is a rare case. This report described a case of nasal cavity supernumerary tooth association with maxillary sinusitis. A 28-year-old male patient reported with the chief complaint of nasal obstruction, headache and purulent secretion for the past three months. Clinic examination and CT examination showed that there was a supernumerary tooth in the right nasal bottom, and maxillary sinus was infected in the same side. This patient was performed supernumerary tooth removing and given antibiotics for 3 days. Ten days after the operation, there was no clinical symptoms, and nasal bottom mucosa was normal. After 3 months of follow-up, reexamination of coronal CT scan appeared normal.

A unique phenotype in a patient with a rare triplication of the 22q11.2 region and new clinical insights of the 22q11.2 microduplication syndrome: a report of two cases.

BACKGROUND: The rearrangements of the 22q11.2 chromosomal region, most frequently deletions and duplications, have been known to be responsible for multiple congenital anomaly disorders. These rearrangements are implicated in syndromes that have some phenotypic resemblances. While the 22q11.2 deletion, also known as DiGeorge/Velocardiofacial syndrome, has common features that include cardiac abnormalities, thymic hypoplasia, characteristic face, hypocalcemia, cognitive delay, palatal defects, velopharyngeal insufficiency, and other malformations, the microduplication syndrome is largely undetected. This is mainly because phenotypic appearance is variable, milder, less characteristic and unpredictable. In this paper, we report the clinical evaluation and follow-up of two patients affected by 22q11.2 rearrangements, emphasizing new phenotypic features associated with duplication and triplication of this genomic region. CASE PRESENTATION: Patient 1 is a 24 year-old female with 22q11.2 duplication who has a heart defect (ostium secundum atrial septal defect) and supernumerary teeth (hyperdontia), a feature previously not reported in patients with 22q11.2 microduplication syndrome. Her monozygotic twin sister, who died at the age of one month, had a different heart defect (truncus arteriousus). Patient 2 is a 20 year-old female with a 22q11.2 triplication who had a father with 22q11.2 duplication. In comparison to the first case reported in the literature, she has an aggravated phenotype characterized by heart defects (restrictive VSD and membranous subaortic stenosis), and presented other facial dysmorphisms and urogenital malformations (ovarian cyst). Additionally, she has a hemangioma planum on the right side of her face, a feature of Sturge-Weber syndrome. CONCLUSIONS: In this report, we described hyperdontia as a new feature of 22q11.2 microdeletion syndrome. Moreover, this syndrome was diagnosed in a patient who had a deceased monozygotic twin affected with a different heart defect, which corresponds to a phenotypic discordance never reported in the literature. Case 2 is the second clinical report of 22q11.2 triplication and presents an aggravated phenotype in contrast to the patient previously reported.

Lateral or oblique facial clefts associated with accessory maxillae: Review of the literature and report of a case.

INTRODUCTION: Lateral or oblique facial clefts associated with accessory alveolar processes are very rare congenital anomalies; only a few clinical cases have been reported in published literature. CASE REPORT AND LITERATURE REVIEW: Here, we present a retrospective review of 22 cases of lateral facial clefts and six cases of oblique facial clefts associated with accessory alveolar processes, and a case report of 23-year-old woman from China with bilateral transverse facial clefts and accessory alveolar processes. We present a summary of their clinical features, along with bone and teeth abnormalities. DISCUSSION: We identified 24 cases from published English literature and four cases from Chinese literature. Accessory alveolar processes in lateral or oblique facial clefts occurred most frequently in the molar region, with pterygoid processes and maxillary tuberosities as the most common site. The maxilla displayed a full complement of deciduous and permanent teeth appropriate to his age. Supernumerary teeth resembled deciduous and permanent molars developed in accessory alveolar processes. There was no cleft in the posterior maxillary alveolus. Some were cases of orbital and mandibular deformation. CONCLUSION: The maxillary prominence was the main area of the lesion. Accessory alveolar processes may be an isolated pathology in lateral or oblique facial cleft.

Dentes supranumerários impactados: relato de caso clínico / Supernumerary impacted teeth: case report

Dentes supranumerários impactados representam um desafio para os Odontopediatras. Estes dentes podem causar atraso na erupção do dente permanente, alteração do crescimento ósseo e posicionamento ectópico do dente permanente. O presente artigo relata a resolução de um caso de dois dentes supra numerários, em ambiente de consultório odontológico, sob sedação e com excelente sucesso clínico radiográfico.

Supernumerary impacted teeth represent a challenge to pediatric dentists. Their presence can cause failure of eruption of permanent teeth, growth and development alterations and displacement of teeth. The case reported in this paper presents the resolution of a case of two supernumerary impacted teeth in a dental office environment, under sedation and with excellent radiographic clinical success.