Prevalence of Peg-Shaped Lateral Incisors in Non-Syndromic Subjects: A Multi-Population Study

Abstract Objective: To evaluate the prevalence and gender-wise distribution of peg-shaped maxillary permanent lateral incisors among populations in Saudi Arabia representing different geographical locations (Saudi, Jordan, Egypt, Syria, Philippine, Pakistan, India and Bangladesh). Material and Methods: Panoramic radiographs of 9945 patients attending outpatient university dental clinics of College of Dentistry, Jouf University, Saudi Arabia between February 2014 and January 2018 were collected from the archives randomly. Two calibrated investigators examined the data, which were collected from the dental radiology department archives with prior permission from the authorities. The anomalies of maxillary lateral incisors (right and left) were investigated. Results: Among all geographic locations, the prevalence of peg laterals was more in males in comparison to females except in Pakistani and Philippine populations. Among the Saudi population, peg laterals' prevalence was more in case of right lateral incisor than the left incisor. Conclusion: A higher prevalence of peg laterals was found in Saudi. Conclusion: A higher prevalence of peg laterals was found in Saudi region, followed by Egypt. Among all geographic locations, the prevalence of peg laterals was higher in males than females except for Pakistan and Philippines populations. Among Saudi population prevalence of peg laterals was found to be higher in case of right lateral incisor when compared to the left.

COVID-19 Pandemic: Oral Repercussions and its Possible Impact on Oral Health

Abstract Many viral infections cause oral manifestations, including disorders in odontogenesis, resulting in dental malformations. In this review, based on current knowledge, we will discuss the likely dental and oral consequences of COVID-19. In this article, we review currently available data associated with vertical transmission of COVID-19 and odontogenesis, oral manifestations, and the impact of COVID-19 pandemic on a diagnosis of oral diseases. Owing to the severity of the pandemic, the population's anxiety and fear of becoming infected with COVID-19 may underestimate the signs and symptoms of serious illnesses, besides discourage patients from seeking health, medical or dental services to determine the diagnosis of oral lesions. Thus, the COVID-19 pandemic could be an additional and aggravating factor for the delay of serious illness diagnosis, such as oral squamous cell carcinoma resulting in higher morbidity and worse prognosis. Several changes and oral lesions have been described as oral manifestations of COVID-19, such as dysgeusia, oral ulcers, petechiae, reddish macules, desquamative gingivitis, among others. Besides, it can cause major systemic changes and predispose opportunistic infections. As with other viral infections, oral manifestations, including dental anomalies, can occur as a direct result of SARS-CoV-2 infection. However, further studies are needed to guide and clarify possible oral changes.

Clinical and x-ray oral evaluation in patients with congenital Zika Virus

Abstract Objective: The aim of this study was to investigate possible malformations in the soft, bone and/or dental tissues in patients with congenital Zika Virus (ZIKV) by clinical and x-ray evaluation. Methodology: Thirty children born with ZIKV and 30 children born without ZIKV (control group) were included in the study. Patients were evaluated over 24 consecutive months according to the variables: sex, age, cleft palates, soft tissue lesions, alveolar ridge hyperplasia, short labial and lingual frenums, inadequate posture of the lingual and perioral muscles at rest, micrognathia, narrow palatine vaults, changes in the teeth shape and/or number, sequence eruption, spasms, seizures and eruption delay were evaluated. Chi-square test, Student's t-test and nominal logistic regression were used (p<0.05). Results: Among the 30 babies examined, the mean age of the first dental eruption was 10.8±3.8 with almost two-thirds of the children (n=18, 60%) experiencing eruptions of their first tooth after 9 months of age, nine children (30%) had inadequate lingual posture at rest, more than half of the children (n=18, 60%) had short labial or lingual frenums. ZIKV babies showed a high prevalence of clef palate (p<0.001), inadequate lingual posture at rest (p=0.004), micrognathia (p=0.002), changes in the shape and/or number of teeth (p=0.006), alteration in sequence of dental eruption (p<0.001) and muscles spasms (p=0.002). The delay eruption was associated with inadequate lingual posture at rest (p=0.047), micrognathia (p=0.002) and changes in the shape and/or number of teeth (p=0.021). The delayed eruption (p=0.006) and narrow palatine vaults (p=0.008) were independently associated with ZIKV. Moreover, female patients showed the most narrow palatine vaults (p=0.010). Conclusions: The children with ZIKV showed a greater tendency to have delayed eruption of the first deciduous tooth, inadequate lingual posture and short labial and lingual frenums.

Are Environmental Factors Effective in Congenitally Missing Teeth? A Multicenter Study

Abstract Objective: To examine the prevalence of congenital absence of permanent teeth other than third molar teeth in non-syndromic children in the Black Sea and Mediterranean Regions of Turkey, and the correlation between gender and distribution sites in the jaws. Material and Methods: Panoramic radiographs and clinical records of 9831 children (5025 girls, 4806 boys) in the Mediterranean Region and 11372 children (5540 girls, 5832 boys) in the Black Sea region were examined. All panoramic radiographs were evaluated by a dentist. The permanent tooth, which was not seen in radiography, was recorded as a congenitally missing tooth, and the fact that the tooth was not extracted was confirmed by treatment records. The data were statistically evaluated by Chi-Square and t-test Results: The prevalence of congenitally missing permanent teeth was 2.8% (3.52% in girls, 2.5% in boys) in the Mediterranean region and 1.63% (1.82% in girls and 1.45% in boys) in the Black Sea region. Congenitally missing permanent teeth were observed more in the maxilla compared to mandible. The teeth that most frequently have congenitally missing permanent teeth in the Mediterranean Region are mandibular second premolars, maxillary lateral incisors and maxillary second premolars, respectively. The teeth that most frequently have congenitally missing permanent teeth in the Black Sea region are mandibular second premolars, maxillary second premolars and maxillary lateral incisors, respectively. Most of the patients had one or two teeth missing, and the lack of three or more teeth was rare Conclusion: In this study, which is performed in different climatic characteristics of Black Sea and Mediterranean Regions, the frequency of congenitally missing teeth is similar.

Prevalence of Dental Anomalies in Indonesian Individuals with Down Syndrome

Abstract Objective: To determine the frequency distribution of dental anomalies in people with Down syndrome. Material and Methods: This cross-sectional study was developed in Jakarta, Indonesia, and evaluated 174 individuals with Down syndrome aged 14-53 years. Were collected information regarding the tooth number, tooth size, shape, and structure. Descriptive statistics were used to calculate the absolute and relative frequencies. The Pearson chi-square test was used in bivariate analysis. The significance threshold was set at 5% Results: There were 70 female subjects (40.2%) and 104 male subjects (59.8%) with an average age of 19.2 years. In terms of anomalies of tooth number, hypodontia (80.9%), supernumerary teeth (12.4%), and combined hypodontia and supernumerary teeth (12.4%) were identified. In terms of anomalies of tooth size, microdontia (98.8%) is the most common anomaly. Anomalies of tooth shape included fusion (66.67%) and talon's cusp (33.3%), whereas anomalies of tooth structure included enamel hypoplasia (70.8%), enamel hypocalcification (4.2%), combined enamel hypoplasia and hypocalcification (12.5%), and tooth discoloration (12.5%) Conclusion: Individuals with Down syndrome in Jakarta showed a high prevalence of dental anomalies, with hypodontia and microdontia being the most common anomalies showing a tendency to occur predominantly in males.

Alterações dentárias em crianças com microcefalia associada à Síndrome Congênita do Zika Vírus e outras infecções congênitas / Dental changes in children with microcephaly associated with Congenital Zika Virus Syndrome and other congenital infections

As consequências da microcefalia associada à Síndrome Congênita do Zika Vírus (SCZ) e outras infecções congênitas no desenvolvimento dentário da criança afetada ainda não são bem conhecidas. Os objetivos deste estudo foram avaliar a frequência de alterações dentárias em crianças com microcefalia, analisar se há associação das alterações dentárias com a SCZ e verificar se a microcefalia é fator de risco para as alterações dentárias. Para isso, foram realizados dois estudos observacionais transversais e um estudo do tipo caso-controle. Um único examinador calibrado (Kappa > 0,8) avaliou a presença de alterações dentárias de número, forma e tamanho, alterações na cronologia e sequência de irrupção dentária e alterações no desenvolvimento do esmalte dentário em crianças com microcefalia, associada à SCZ e outras infecções congênitas, e em crianças normoreativas. Informações relacionadas à gestação da mãe e ao nascimento da criança foram coletadas e um questionário socioeconômico foi aplicado. Os dados foram avaliados descritivamente e, como testes de associação, foram utilizados o teste do Qui-quadrado e Exato de Fisher, considerando um nível de significância de 5% (estudos 2 e 3). A amostra do primeiro estudo foi composta por 49 crianças entre 7 e 35 meses de idade apresentando microcefalia associada à SCZ. As alterações mais prevalentes foram as relacionadas à cronologia de irrupção (93,9%; IC95%= 89­99%), às alterações no desenvolvimento do esmalte dentário (76,1%; IC95%= 64­88%) e sequência de irrupção dentária (71,7%; IC95%= 60­84%). No segundo estudo, 62 crianças, com idade entre 7 e 35 meses, portadoras de microcefalia associada à SCZ e outras infecções congênitas compuseram a amostra. Não houve associação estatisticamente significativa entre a SCZ e a presença de alteração na cronologia (p = 1,00) e sequência de irrupção dentária (p = 0,16) e de desenvolvimento do esmalte dentário (p = 1,00). No estudo de caso-controle, 81 crianças entre 30 e 35 meses de idade, normoreativas e portadoras de microcefalia, fizeram parte da amostra, a qual, após identificadas as frequências de cada uma das alterações dentárias, foi emparelhada pelo sexo e idade, na proporção 1:1, e alocadas nos grupos caso (presença de alterações dentárias) e controle (ausência de alterações dentárias). A presença de microcefalia mostrou-se estatisticamente associada ao atraso na irrupção dentária (p < 0,001), à presença de alterações na sequência de irrupção dentária (p < 0,001) e de defeitos no esmalte dentário (p < 0,001). Concluiu-se que as crianças com microcefalia associada à SCZ apresentaram atraso na irrupção dentária, alterações na sequência irruptiva e opacidade do esmalte dos dentes decíduos, no entanto, a infecção pelo vírus Zika não foi associada à ocorrência dessas alterações dentárias. A microcefalia, independente de sua etiologia, é fator de risco para alterações relacionadas ao processo de irrupção dentária e ao desenvolvimento do esmalte dos dentes decíduos (AU).

The consequences of microcephaly associated with Congenital Zika Virus Syndrome (CZS) and other congenital infections on the dental development of the affected child are not well known. The objectives of this study were to evaluate the frequency of dental alterations in children with microcephaly, to analyze if there is an association of dental alterations with CZS, and to verify if microcephaly is a risk factor for dental alterations. For this, two crosssectional observational studies and one case-control study were performed. A single calibrated examiner (Kappa > 0,8) evaluated the presence of dental alterations of number, shape and size, alterations in the chronology and sequence of tooth eruption, and alterations in the tooth enamel development in children with CZS and other congenital infections, as well as in normoreactive children. Information related to mothers' pregnancies and child births were collected and a socioeconomic questionnaire was applied. Data were descriptively evaluated and chi-square test and Fisher's exact test were used as association tests considering a significance level of 5% (studies 2 and 3). The first study sample consisted of 49 children between 7 and 35 months of age with CZS-associated microcephaly. The most prevalent alterations were related to the eruption chronology (93.9%), changes in the development of the enamel (76.1%) and the dental eruption sequence (71.7%). Next, 62 children aged 7 to 35 months with CZS-associated microcephaly and other congenital infections comprised the sample in the second study. There was no statistically significant association between CZS and the presence of changes in chronology (p = 1.00), sequence of tooth eruption (p = 0.16) and tooth enamel development (p = 1.00). In the case-control study, 81 normoreactive children and children with microcephaly between 30 and 35 months of age were part of the sample, which were then paired by gender and age at a 1:1 ratio after identifying the frequencies of each of the dental alterations, and then allocated to the case (presence of dental changes) or control (absence of dental changes) groups. The presence of microcephaly was statistically associated with delayed tooth eruption (p<0.001), the presence of changes in tooth eruption sequence (p<0.001) and dental enamel defects (p<0.001). It was concluded that children with CZS-associated microcephaly had delayed dental eruption, alterations in the eruptive sequence and hypomineralization of primary tooth enamel; however, a Zika virus infection was not associated with these dental changes. A microcephaly, regardless of its etiology, is a risk factor for changes related to the tooth eruption process and the development of primary tooth enamel. It is concluded that microcephaly associated with CZS and other congenital infections is a risk factor for delayed tooth eruption, alterations in the eruptive sequence and defects in dental enamel development occurring (AU).

Odontoma Compuesto: Diagnóstico y Tratamiente Reporte de Casos & Revisión de la Literatura / Compound Odontoma: Diagnosis and Treatment Therapeutic Measures. Case Reports & Literature Review

RESUMEN: Los odontomas son los tumores odontogénicos benignos más prevalentes. Estos están conformados por tejido dentario, epitelio odontogénico y tejido mesenquimático. Se dividen en dos subtipos: odontomas compuestos, que presentan tejidos dentarios normales, pero con una alteración en su conformación y tamaño, y odontomas complejos, que presentan tejidos dentarios bien formados, pero rodeados de tejido desorganizado. Se presentan tres casos de pacientes sexo femenino, quienes acuden para evaluación y tratamiento debido al hallazgo radiográfico de odontoma compuesto. En el caso nº 1 se realizó la desinclusión del diente impactado, la exodoncia del diente remanente, la exéresis del odontoma compuesto, la instalación de un implante dental en el alveolo de la zona intervenida y, finalmente, el posicionamiento de injerto óseo. En el caso nº 2 se realizó la exodoncia del diente temporal remanente y la exéreis del odontoma compuesto. Ambos casos evolucionaron de manera favorable sin complicaciones post operatorias. En el caso nº 3 se realizó la fenestración del diente 18 y la exéreis del odontoma compuesto que retenía su erupción. Todos los casos evolucionaron de manera favorable sin complicaciones post operatorias. Dada la prevalencia de esta patología, es necesario un adecuado conocimiento sobre ella y sus características, para realizar un adecuado diagnóstico y tratamiento. Existen distintas medidas terapéuticas respecto a la rehabilitación de una zona edéntula como resultado de la exéresis de estos tumores. Esto depende principalmente de la edad del paciente. Al ser requeridas medidas rehabilitadoras, un punto importante a considerar es la posibilidad de realizar todos los procedimientos quirúrgicos en un solo tiempo operatorio.

ABSTRACT: Odontomas are the most common benign odontogenic tumors and are composed of dental tissue, odontogenic epithelium and mesenchymal tissue. They are divided into two subtypes: Compound odontomas, which present normal tooth tissue, but an alteration in their conformation and size, and complex odontomas, which present well-formed tooth tissue, but are surrounded by disorganized tissue. A bibliographic review was performed by one operator in Pubmed and Epistemonikos. After filtering by title and abstract, only one systematic review was selected. We present two clinical cases of compound odontoma in female patients at the Military Hospital in Santiago. Patients were referred for evaluation and treatment due to the radiographic finding of compound odontoma. In case # 1, the impacted tooth was disincluded, the remaining temporal tooth was extracted, and excision of the compound odontoma carried out; subsequently a bone graft implant was placed in the remaining socket. In case # 2, the remaining temporal tooth was extracted and compound odontoma excised. After that, natural eruption of the corresponding permanent tooth is expected. In case # 3, tooth 1.8 fenestration and excision of compound odontoma was performed. In this case also, natural eruption of the corresponding permanent tooth is expected. Given the prevalence of this pathology, adequate knowledge of compound odontomas and their characteristics is necessary for proper diagnosis and treatment. There are different therapeutic measures for the rehabilitation of an edentulous area following excision of compound odontoma. This depends mainly on the age of the patient. When rehabilitation measures are required, an important aspect to consider for the patient, is the possibility of a single surgical event, as was done in case # 1.

Fragile X syndrome: panoramic radiographic evaluation of dental anomalies, dental mineralization stage, and mandibular angle

ABSTRACT Fragile X syndrome (FXS) is a disorder linked to the chromosome X long arm (Xq27.3), which is identified by a constriction named fragile site. It determines various changes, such as behavioral or emotional problems, learning difficulties, and intellectual disabilities. Craniofacial abnormalities such as elongated and narrow face, prominent forehead, broad nose, large and prominent ear pavilions, strabismus, and myopia are frequent characteristics. Regarding the oral aspects, deep and high-arched palate, mandibular prognathism, and malocclusion are also observed. Objective: The purpose of this study was to evaluate the dental radiographic characteristics as described in 40 records of patients with panoramic radiography. Material and Methods: The patients were in the range of 6–17 years old, and were divided into two groups (20 subjects who were compatible with the normality standard and 20 individuals diagnosed with the FXS), which were matched for gender and age. Analysis of the panoramic radiographic examination involved the evaluation of dental mineralization stage, mandibular angle size, and presence of dental anomalies in both deciduous and permanent dentitions. Results: The results of radiographic evaluation demonstrated that the chronology of tooth eruption of all third and second lower molars is anticipated in individuals with FXS (p<0.05). In this group, supernumerary deciduous teeth (2.83%), giroversion of permanent teeth (2.31%), and partial anodontia (1.82%) were the most frequent dental anomalies. In addition, an increase was observed in the mandibular angle size in the FXS group (p<0.05). Conclusion: We conclude that knowledge of dental radiographic changes is of great importance for dental surgeons to plan the treatment of these individuals.

Family polythelia associated with dental anomalies: a case report / Politelia familiar asociada con anomalías dentarias: un caso clínico

Abstract Polythelia is defined as the presence of supernumerary nipples without the presence of additional mammary gland, within the milk line extending from the axilla to the pubic region. Though the presence of dental anomalies can create a simple esthetic problem with specific clinical considerations, the association with familial polythelia has rarely been reported. A report of association of dental anomalies and polythelia in an Argentine family is presented and the considerations about the dental practice suggesting a careful anamnesis and referral to a medical consultation with regard to possible pathologic conditions or potentially malignant transformation of accessory breasts are discussed.

Resumen La politelia ha sido definida como la presencia de pezones supernumerarios sin tejido accesorio glandular; generalmente, éstos siguen las líneas mamarias que discurren imaginariamente desde las axilas hasta la región inguinal. Si bien la presencia de anomalías dentarias puede originar un simple problema estético con específicas consideraciones clínicas, la asociación con politeliafamiliar ha sido escasamente informada. Se presenta un caso de politelia asociada con anomalías dentarias en una familia argentina, y se discuten sugerencias para una cuidadosa anamnesis odontológica y derivación a la consulta médica para prevenir eventuales condiciones patológicas o una potencial transformación maligna de los tejidos mamarios.

Incontinentia pigmenti

Incontinentia pigmenti is a rare genodermatosis in which the skin involvement occurs in all patients. Additionally, other ectodermal tissues may be affected, such as the central nervous system, eyes, hair, nails and teeth. The disease has a X-linked dominant inheritance pattern and is usually lethal to male fetuses. The dermatological findings occur in four successive phases, following the lines of Blaschko: First phase - vesicles on an erythematous base; second phase - verrucous hyperkeratotic lesions; third phase - hyperchromic spots and fourth phase - hypochromic atrophic lesions.

Orodental findings in Hallermann-Streiff syndrome.

Hallermann-Streiff syndrome-also called occulomandibulofacial syndrome, Francois syndrome, oculomandibulodyscephaly with hypotrichosis, Aubry syndrome I, and Ullrich-Fremery-Dohna syndrome-is a rare genetic disorder, which comprisesmultiple congenital abnormalities affecting chiefly the head and face. It is characterized by bird-like facies, dental abnormalities, hypotrichosis, atrophy of skin, congenital cataracts, bilateral microphthalmia, and proportionate nanism. An interesting case of Hallermann-Streiff syndrome in a 23-year-old female patient is reported here, with the emphasis on the orodental findings.

Minichromosome maintenance 2 and 5 expressions are increased in the epithelium of hereditary gingival fibromatosis associated with dental abnormalities

INTRODUCTION: Gingiva fibromatosis is a relatively rare condition characterized by diffuse enlargement of the gingiva, which is caused by expansion and accumulation of the connective tissue. OBJECTIVE: The aim of the present study was to investigate proliferative and apoptotic biomarker expression in normal gingiva and two forms of gingival fibromatosis. METHODS: Archived tissue specimens of hereditary gingival fibromatosis, gingival fibromatosis and dental abnormality syndrome and normal gingiva were subject to morphological analysis and immunohistochemical staining. The results were analyzed statistically. RESULTS: Proteins associated with proliferation were found in the nuclei of epithelial cells from the basal and suprabasal layers, whereas apoptotic proteins were detected in the cytoplasm of the upper layers of the epithelium. Increased expressions of minichromosome maintenance proteins 2 and 5 were observed in the gingival fibromatosis and dental abnormality syndrome samples. In contrast, geminin expression was higher in normal gingiva samples. No difference in the expression of apoptotic proteins was observed among the groups. CONCLUSION: Our findings support a role for augmented proliferation of epithelial cells within the overgrown tissues associated with gingival fibromatosis or dental abnormality syndrome. However, our data suggest that different biological mechanisms may account for the pathogenesis of different types of gingival fibromatosis.

Síndrome lacrimo-auriculo-dento-digital (LADD): relato de caso e revisão da literatura / The lacrimo-auriculo-dento-digital syndrome (LADD): case report and literature review

A síndrome de Levy-Hollister ou lacrimo-auriculo-dento-digital (LADD) é uma síndrome rara, de herança autossômica dominante, podendo ocorrer de forma isolada ou em várias gerações de uma mesma família. O diagnóstico é feito por meio da identificação de anormalidades do sistema lacrimal, como redução ou ausência de produção de lágrimas, alterações ósseas, dentárias, de glândulas salivares e orelha externa. Trata-se de uma criança, de 13 anos, em acompanhamento no Hospital do Servidor Público Estadual de São Paulo desde os cinco meses de idade, com quadro clínico compatível com a síndrome, apresentando quadro de olho seco. Devido ao fato das manifestações oculares ocorrerem precocemente, sendo o oftalmologista um dos primeiros profissionais a ser procurado, o mesmo deve ter conhecimento da síndrome, a fim de diagnosticar e acompanhar adequadamente o indivíduo acometido.

Levy-Hollister or lacrimo-auriculo-dento-digital (LADD) syndrome is a rare entity with autossomic dominant inheritance occuring as an isolated form or affecting many family generations. Diagnosis is based on the identification of the lacrimal drainage system abnormalities with reduction or absence of tear production and bone, teeth, salivar glands and outer ear abnormalities. A 13 year-old male patient has been followed at the Hospital Servidor Público Estadual in São Paulo due to dry eye since his first year of life. Due to the occurrence of early ocular manifestations in patients with Levy-Hollister or lacrimo-auriculo-dento-digital syndrome, ophthalmologists must be aware to recognize and control this syndrome.

An unusual case of non-syndromic occurrence of multiple dental anomalies.

Dental anomalies have been known to occur in humans due to a variety of genetic and environmental factors. Combinations of dental anomalies are known to be associated with specific syndromes. A few cases of multiple dental anomalies have been reported in patients with no generalized abnormalities. This case report describes an unusual occurrence of a combination of dental anomalies in an apparently normal healthy 12-year-old female patient. The dental anomalies in this patient were multiple dens invaginatus, generalized enamel hypoplasia, generalized microdontia, root resorption and multiple periapical lesions, shovel shaped incisors, cup shaped premolars, taurodontism, hypodontia and supernumerary teeth.

Multiple dental anomalies and aggressive periodontitis: A coincidence or an association.

Aggressive periodontitis (AgP) comprises a group of rare, often severe, rapidly progressive forms of periodontitis mostly characterized by an early age of clinical manifestation and a distinctive tendency for cases to aggregate in families. Abnormal dental morphology and position have been associated with severe periodontal diseases. The purpose of this paper is to report a case of multiple dental anomalies associated with AgP. This paper reports a case of unusual association of multiple dental anomalies to AgP. Clinical findings and history led to the diagnosis of localized AgP, and radiologically. It was associated with multiple dental anomalies, especially supernumerary roots. Thus, the present case represents a very interesting demonstration of AgP association with supernumerary roots and the nature of this association merits further investigations.

Goldenhar syndrome with unusual features.

We report here the case of a 17 year-old girl with the classic signs of Goldenhar syndrome in the form of multiple accessory tragi, bilateral ocular dermoids, mandibular hypoplasia (micrognathia) and cervical lordosis. She also had a high arched palate, gingival hypertrophy and malaligned teeth, features which are as yet unreported.

Witkop's tooth and nail syndrome: a multifaceted approach to dental management.

Witkop's tooth and nail syndrome is a rare autosomal dominant disorder of ectodermal dysplasia characterized by hypodontia and nail dysplasia. Mutations in MSX-1 have been shown to be associated with this syndrome. There is failure of development and eruption of the dentition. Tooth shape may vary; the most common forms are conical and narrow crowns. The nails may be spoon shaped and slow growing and affect both finger and toe nails. The nail involvement is more severe in childhood. The present case describes a 14-year-old boy who showed the characteristic features of Witkop's syndrome. A multifaceted approach to the dental management of the patient is discussed.

Kabuki make-up (Niikawa-Kuroki) syndrome: dental and craniofacial findings in a Brazilian child

This article reports the case of a Brazilian child diagnosed with Kabuki make-up syndrome (KMS), addressing the clinical features observed, with emphasis on the disease-specific oral and craniofacial manifestations. The patient had the distinctive KMS craniofacial appearance, mild delayed mental development, fingers with prominent fingertip pads and visual deficit. The dental findings included fusion of the left mandibular incisors (central and lateral), gemination of the right mandibular central incisor and congenital agenesis of the right mandibular lateral incisor, in the primary dentition, as well as absence of both permanent mandibular lateral incisors. Fusion and gemination have not been previously referred to as typical dental features in KMS. The detection of unique dental findings, such as missing teeth and dental anomalies of form in the primary dentition by means of clinical and radiographic examinations, might consist of a helpful diagnosis parameter in identifying children who may have milder forms of Kabuki syndrome.

Este artigo relata o caso de uma criança portadora da Síndrome de Kabuqui e descreve as características clínicas observadas, com ênfase nas manifestações craniofaciais e intrabucais. O paciente apresentava características craniofaciais clássicas da síndrome, deficiência mental leve, déficit visual e atraso no desenvolvimento dental. Os achados intrabucais incluíam a fusão dos incisivos inferiores do lado esquerdo (central e lateral), geminação do incisivo central inferior direito e agenesia do incisivo lateral inferior direito na dentição decídua bem como ausência dos incisivos laterais inferiores permanentes. Fusão e geminação ainda não foram descritas em pacientes com a síndrome. Assim, a detecção de achados dentais como agenesias e anomalias de forma na dentição decídua, por meio de exames clínicos e radiográficos, são importantes para auxíliar no diagnóstico de crianças com uma forma moderada da Síndrome de Kabuqui.

Displasia ectodérmica hipohidrótica / Hypohidrotic ectodermal dysplasia

A displasia ectodérmica (DE) é uma rara doença congênita que afeta várias estruturas provenientes do ectoderma, principalmente cabelos, unhas, dentes, pele, glândulas sudoríparas e sebáceas. A displasia ectodérmica hipohidrótica ligada ao cromossomo X é a forma mais conhecida e uma das quais o cuidado odontológico é a etapa mais importante do tratamento. Este artigo ressalta a importância do diagnóstico e intervenção precoce da síndrome e relata a abordagem odontológica integral da paciente atendida. Durante uma visita domiciliar, foi constatado a DE e a paciente foi encaminhada para o ambulatório do curso de odontologia da universidade, em que por meio da anamnese, exame clínico e radiográfico foi elaborado o plano de tratamento. Foram realizados procedimentos educativos e preventivos, como instrução de higiene bucal e da dieta alimentar, controle de placa bacteriana e remoção de hábitos bucais ou deletérios, juntamente com procedimentos de reabilitação oral. Conclui-se que o diagnóstico e o tratamento precoce são fatores importantes para melhorar as condições estéticas, funcionais e psicológicas das crianças afetadas

Lobster-claw syndrome.

Lobster-Claw syndrome is a rare autosomal dominant, hand-foot malformation with Oro-dental features. This is a rare condition and from the available reports so far, cases being reported are less than 1%. Most reports have focused on the hand-foot deformity of this syndrome. This paper highlights the typical Oro-dental features associated with this syndrome such as retained deciduous teeth, hypodontia and variation in crown size, arch length and arch width.