Type II Pfieffer misdiagnosed as Crouzon syndrome with additional features of supernumerary teeth and localized symmetrical gigantism: a case report.

BACKGROUND: Pfieffer syndrome is among the syndromes seen in the recognized variant of the FGFR2 gene. There are several conditions related to this variant and a very closely related condition is Crouzon syndrome. This case is important to report because the neonate was a delayed referral from another region, without clear counseling and information on the gravity of situation. We describe additional features , not previously described in Pfieffer or Crouzon syndrome, supernumerary teeth and localized symmetrical gigantism of thumbs and great toes on both sides. That a genetic testing is essential to further manage and counsel to avoid lost opportunities for future births. Several cases are seen in this unit annually, and there is need for a more consolidated and comprehensive counseling and genetic testing. Once early diagnosis is done and the case is recognized to be untreatable, it was avert the need to refer. CASE PRESENTATION: A 2-week-old male African neonate referred from outside the region, presented with massive proptosis soon after delivery, with signs of pan-ophthalmitis and neonatal sepsis. The infant had additional multiple malformations and features initially diagnosed as Crouzon syndrome , but later confirmed after genetic testing to be Type II Pfieffer syndrome. A through clinical evaluation and genetic testing would prevent undue referral to a tertiary center, or if needed, the baby should have been referred much earlier. The uniqueness of this case is the presence of supernumerary teeth. CONCLUSION: A complicated, difficult to remedy case, referred to tertiary center, investigated, and sent back home with no significant intervention. Genetic test confirmed the typical findings of Pfieffer Type II. Presented for describing additional unique features of supernumerary teeth and localized gigantism and ethical challenges in management.

The Prevalence and Morphology of Supernumerary Teeth in Children With Nonsyndromic Cleft Lip and Palate.

INTRODUCTION: Cleft lip with or without cleft palate (CL/P) is congenital deformity associated with hyperdontia. OBJECTIVE: To determine the prevalence and characteristics of supernumerary teeth in patients with CL/P. DESIGN: Retrospective descriptive and correlation clinical study. PATIENTS: One hundred thirteen children with cleft (age ranged 9.3-19.2; 67 males and 46 females) treated in Clinic of Congenital Facial Deformities Medical University of Lublin were included in the study. METHODS: Records evaluation was conducted regarding age, gender, cleft type (Q36, Q37-International Classification of Diseases 10th revision), cleft side, and incidence of supernumeraries. In all supernumerary teeth, size, shape, and developmental degree were analyzed and correlation between the incidence of hyperdontia with different variables was checked. Correlations were detected using chi-square and the Yates correction. RESULTS: The majority of the examined group were males-59.29% with Q37 (67.26%) and the cleft on the left side (62.83%). Hyperdontia was noted in 26.55%. Only upper lateral incisors were affected. They usually had atypical shape (56.67%), reduced size (83.33%), and delayed development (56.67%). CONCLUSIONS: The prevalence of supernumerary permanent teeth in patients with cleft was higher than in the general population. Anomaly was more frequent in male patients and occurred mainly on the cleft side. The severity of the cleft did not influenced the frequency of supernumerary teeth, their shape, size, and developmental degree. Supernumerary teeth were characterized by reduced crown size, abnormal structure, incorrect inclination, and delayed development phase.

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.

Supernumerary Teeth: A Retrospective Cross-Sectional Study from Palestine

Abstract Objective: To investigate the prevalence and characteristics of supernumerary teeth among patients attending the students' dental clinics at the Faculty of Dentistry of the Arab American University, Jenin, Palestine. Material and Methods: This is a descriptive, cross-sectional, retrospective study of 1970 digital panoramic radiographs of patients, age ranging between 12 and 50 years. The radiographs and dental records were reviewed for supernumerary teeth. For each patient with supernumerary teeth, were recorded the demographic variables, number, location, eruption status, type, and morphology, the developmental stages. Descriptive statistics were performed and results were tabulated. Results: Supernumerary teeth were detected in 17 subjects (0.86%), of which 13 were males and 4 were females with a male to female ratio of 3.25:1. Of the 17 patients, 64.7% had one supernumerary tooth while the others had two. The majority (78.3%) of the supernumeraries were located in the maxilla. Around 56.5% of the supernumeraries found in this study were located in the premaxilla. Half of the supernumeraries (52.2%) were erupted, and 47.8% were impacted. Mesiodens was the most frequently seen supernumerary teeth followed by distomolars and premolars. Most of the supernumeraries were fully developed, while 21.7% displayed only crown formation. Conclusion: The prevalence of supernumerary teeth in the study sample was found to be 0.86% and the most frequent type was mesiodens. Supplemental morphology was found to be the most common form of supernumerary teeth.

Main genetic entities associated with supernumerary teeth. / Principales entidades genéticas asociadas con dientes supernumerarios.

Supernumerary teeth represent a common human dental anomaly, defined as presence of extra teeth-more than the normal number foreseen in primary or permanent dentition. The prevalence has been reported between 0.2 to 3%, and is more frequent in males than females. The etiology is heterogeneous, highly variable and most of the cases are idiopathic. However, the presence of multiple impacted or erupted supernumerary teeth is rare and associated with some genetic syndromes: cleidocranial displasia, familial adenomatous polyposis, trichorhinophalangeal syndrome type I, Rubinstein-Taybi syndrome, Nance-Horan syndrome, Opitz G/BBB syndrome, oculofaciocardiodental syndrome and Robinow syndrome (autosomal dominant). The supernumerary teeth should be considered in order to possibly diagnose these entities with the aim of offering an interdisciplinary management and treatment, as well as offer adequate family genetic counseling.

Los dientes supernumerarios representan una anomalía dental frecuente en los seres humanos. Esta afección se define como la presencia de una mayor cantidad de dientes que los previstos en cuanto a los dientes de leche o a los permanentes. Según se ha notificado, la prevalencia varía entre el 0,2 % y el 3 % y es más frecuente en los varones que en las mujeres. Su etiología es heterogénea, sumamente variable y, en la mayoría de los casos, idiopática. Sin embargo, la presencia de múltiples dientes supernumerarios retenidos o erupcionados es infrecuente y está asociada con ciertos síndromes genéticos, como displasia cleidocraneal, poliposis adenomatosa familiar, síndrome tricorrinofalángico de tipo I, síndrome de Rubinstein-Taybi, síndrome de Nance-Horan, síndrome de Opitz G/BBB, síndrome oculofaciocardiodental y síndrome de Robinow (autosómico dominante). Se deben considerar los dientes supernumerarios para diagnosticar estas entidades a fin de ofrecer un abordaje interdisciplinario, además de brindar asesoramiento genético familiar adecuado.

Supernumerary Incisors in CB6F1 Mice Conditioned with Chemotherapy and Total Body Irradiation before Bone Marrow Transplantation.

Multiple adult female CB6F1 mice presented with supernumerary incisors after preconditioning with chemotherapy and total body irradiation for bone marrow transplantation (BMT). Mice received nonmyeloablative total body irradiation (3 Gy) and either cyclophosphamide or bendamustine, followed by BMT and posttransplantation cyclophosphamide or bendamustine. Here we describe the clinical presentation, µCT findings, and histopathologic evaluation of the affected mice. These analyses confirmed the gross diagnosis and revealed details of the abnormal tooth morphology. We surmise that the combination of total body irradiation and chemotherapy resulted in the abnormal formation of supernumerary incisors. Supernumerary teeth should be considered as a potential confounding factor in tracking weight loss after BMT. These conditions can be managed to allow animals to reach their intended scientific endpoint.

Secondary Dentition Characteristics in Children With Nonsyndromic Unilateral Cleft Lip and Palate: A Retrospective Study.

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.

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.

Crouzon syndrome with multiple supernumerary teeth.

Crouzon syndrome (CS) is an autosomal dominant disorder characterized by craniofacial deformities caused by the early closure of cranial sutures. It is diagnosed by the presence of a flat sphenoid bone, protrusion of eyeballs (exophthalmos), and midfacial hypoplasia. Although hypodontia is usually present in cases with CS, supernumerary teeth are rarely seen. A 16-year-old male patient with CS was referred to our clinic. He had a high forehead, beaked nose, hypertelorism, palpebral ptosis, and asymmetrical orbits. Bilateral multiple supernumerary teeth were observed in his upper and lower jaws. Early diagnosis of CS is helpful in dental and craniofacial treatment. Because of multiple facial and oral problems, this patient required a multidisciplinary treatment approach.

A rare case of mandibulary parapremolars in siblings: case report and literature review.

Supernumerary teeth in permanent dentition are influenced by hereditary and environmental, having clinical variations such as location, number and morphology. Parapremolars can be found as a single supernumerary tooth or as multiple, usually in the lower jaw, sometimes in association with other types of supernumerary teeth. Familial inheritance often involves more than one generation. This paper presents a case of two brothers, one with only one parapremolar and a good oral status and hygiene, the other with poor hygiene, multiple parapremolars and one mesiodens, causing various complications. The aim of this paper is to show the presence of parapremolars in brothers with no other medical relevant history, located in the same site, and to point the influence of genetic factors in the etiology of supernumerary teeth. Along with a review of the literature, this article shows the importance of an early diagnosis and follow-up using successive panoramic radiographs of such cases, in order to limit or prevent the inflammatory, carious, periodontal and occlusal complications that can seriously affect both the jaws and the normal dentition.

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.

Prevalência de dentes supranumerários em pacientes na faixa etária de 12 a 20 anos em Manaus (AM) / Prevalence of supernumerary teeth in patients aged from 12 to 20 years in Manaus (AM)

A pesquisa avaliou a prevalência de dentes supranumerários na cidade de Manaus (AM), visando obter dados científicos que possam corroborar com outros estudos. Foram analisadas 1.000 radiografias panorâmicas de pacientes na faixa etária de 12 a 20 anos, observando-se a prevalência dos dentes supranumerários, de acordo com o sexo, arco dentário e as regiões mais acometidas dos pacientes. Os dados obtidos foram tabulados, as medidas de tendência central e dispersão foram estimadas, e a prevalência foi calculada. Para a comparação entre os sexos, foi aplicado o teste de Qui-quadrado, ao nível de 5% de probabilidade. A prevalência de dentes supranumerários foi de 3,2%, não houve diferença significante entre os sexos, a mandíbula foi o osso gnático mais acometido, houve maior frequência de dentes supranumerários na região de pré-molares, e a maior parte dos pacientes possuía um único supranumerário. A prevalência de dentes supranumerários foi condizente com outros estudos, podendo corroborar com outras pesquisas.

This study evaluated the prevalence of supernumerary teeth in the city of Manaus (AM), to obtain scientific data that can corroborate with other studies One thousand panoramic radiographs were evaluated from patients aged at 12-20 years-old and the prevalence classified according to gender, dental arch/ region. Data were with central tendency and dispersion values calculated. The Chi-square test was used to compare prevalence between genders (5% level of significance). The prevalence of supernumerary teeth was 3.2%, with no differences between genders, being the premolar mandibular region the most affected. Most of patients presented one supernumerary tooth. The results corroborate those already published by other articles.

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.

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...

Excess NF-κB induces ectopic odontogenesis in embryonic incisor epithelium.

Nuclear factor kappa B (NF-κB) signaling plays critical roles in many physiological and pathological processes, including regulating organogenesis. Down-regulation of NF-κB signaling during development results in hypohidrotic ectodermal dysplasia. The roles of NF-κB signaling in tooth development, however, are not fully understood. We examined mice overexpressing IKKß, an essential component of the NF-κB pathway, under keratin 5 promoter (K5-Ikkß). K5-Ikkß mice showed supernumerary incisors whose formation was accompanied by up-regulation of canonical Wnt signaling. Apoptosis that is normally observed in wild-type incisor epithelium was reduced in K5-Ikkß mice. The supernumerary incisors in K5-Ikkß mice were found to phenocopy extra incisors in mice with mutations of Wnt inhibitor, Wise. Excess NF-κB activity thus induces an ectopic odontogenesis program that is usually suppressed under physiological conditions.

Multiple, supernumerary retained teeth in the course of cleido-cranial dysplasia. A case report.

Cleido-cranial dysplasia, often referred to as Scheuthauer-Marie-Sainton syndrome, is an autosomal dominant disorder of the musculo-skeletal system. Patients with cleido-cranial dysplasia are characterized by short stature, frequent varus or valgus hip, kyphoscoliosis, underdevelopment of the scapulas and the sternum, incorrect number of ribs. The most characteristic feature is unilateral or bilateral, partial or total underdevelopment of clavicles. Mental development is not affected in this syndrome. Malocclusion, occlusal irregularities, multiple supernumerary teeth, impacted teeth, and persistent milk teeth are found in the stomatognathic system. Teeth often have abnormal anatomy. Gothic palate, cleft hard and soft palate are diagnosed. The aim of this paper is to present a case of a 12-year-old boy diagnosed with irregularities in the masticatory system involving an additional number of retained teeth. The boy was referred by an orthodontist for surgical and orthodontic team therapy. The case presented confirms the observations of other authors that only the multi-specialty collaboration of a pediatrician, a geneticist, an orthopedist, an orthodontist, a maxillofacial surgeon, an implant prosthetic surgeon and a physiotherapist can provide proper diagnosis and treatment.

Odontomas and supernumerary teeth: is there a common origin?

The aim of the present work is to analyze all scientific evidence to verify whether similarities supporting a unified explanation for odontomas and supernumerary teeth exist. A literature search was first conducted for epidemiologic studies indexed by PubMed, to verify their worldwide incidence. The analysis of the literature data shows some interesting similarities between odontomas and supernumerary teeth concerning their topographic distribution and pathologic manifestations. There is also some indication of common genetic and immuno-histochemical factors. Although from a nosological point of view, odontomas and supernumeraries are classified as distinct entities, they seem to be the expression of the same pathologic process, either malformative or hamartomatous.

Non-syndromic multiple supernumerary teeth in permanent dentition: a rare phenomenon.

Hyperdontia or supernumerary teeth in the absence of associated systemic condition or syndrome is an uncommon phenomenon. Non-syndromic supernumerary teeth need to have periodical radiographic observation. In the case of asymptomatic condition, as they impacted in the jaw, a careful examination is necessary because they may develop into pathological status such as dentigerous cysts. Surgical removal of such teeth is indicated if evidence of any pathologies, such as cystic lesion, resorption, delayed eruption, altered eruption and displacement of adjacent teeth, is evident or have occurred.