Natal Teeth Research Articles

Molecular epidemiology of pachyonychia congenita in the Israeli population.

Pachyonychia congenita (PC) is a rare autosomal dominant disorder featuring palmoplantar keratoderma, nail dystrophy, oral leucokeratosis, pilosebaceous cysts and natal teeth. PC results from dominant mutations in one of five genes (KRT6A, KRT6B, KRT6C, KRT16, KRT17) encoding keratin proteins. To delineate the clinical and genetic features of PC in a series of Israeli patients. We used direct sequencing of genomic DNA, and also used cDNA sequencing where applicable. We collected clinical information and molecular data in a cohort of Israeli families diagnosed with PC (n=16). Most of the patients were Ashkenazi Jews and had a family history of PC. The most common clinical findings were painful focal plantar keratoderma (94%) accompanied by nail dystrophy (81%), pilosebaceous cysts (31%) and prenatal/natal teeth (13%). In contrast to the high prevalence of KRT6A mutations in other populations, we found that KRT16 mutations were the most common type among Israeli patients with PC (56%). Most (77%) of the Israeli patients with PC with KRT16 mutation carried the same variant (c.380G>A; p.R127H) and shared the same haplotype around the KRT16 locus, suggestive of a founder effect. The data gleaned from this study emphasizes the importance of population-specific tailored diagnostic strategies.

Downs syndrome associated with dentitia praecox in maxillary posterior region: A case report

Down syndrome or trisomy 21 is a chromosomal abnormality. Presence of neonatal or pre-deciduous teeth is commonly seen in downs syndrome. According to published literature maxillary and mandibular anterior teeth are commonly seen. Presence of Rege-Fida disease in infants with Down syndrome due to mandibular anterior neonatal teeth and secondary ulceration is well known, however presence of posterior neonatal teeth is rare. Here by we present a case report of a 48 day old child with Down’s syndrome with pre-deciduous dentition in the maxillary posterior region. Keywords: Downs Syndrome, posterior Natal tooth, Neonatal tooth

Paquioniquia congénita tipo I o síndrome de Jadassohn-Lewandowsky: un caso de presentación familiar

La paquioniquia congénita es una rara genodermatosis de herencia autosómica dominante, que produce alteración de la queratinización a nivel de piel, uñas y mucosas. El primer caso fue descrito por Jadassohn y Lewandowsky en 1906, y actualmente afecta a miles de personas en el mundo. El Registro Internacional de Investigación de Paquioniquia Congénita (IPCRR, del inglés, International PC Research Registry) ha identificado a nivel mundial hasta enero de 2020, 977 individuos en 517 familias con paquioniquia congénita, confirmadas genéticamente. Esta condición es considerada en nuestro país como una enfermedad huérfana, y previamente solo hay un reporte en la literatura de un caso en 2009. Se presenta el caso clínico de un paciente masculino de 23 años de edad procedente del área rural del municipio de Timbío, Colombia, con historia clínica de distrofia ungueal hipertrófica en todas las uñas de manos y pies, queratodermia palmoplantar y dolor plantar moderado a severo. Además, presentaba leucoqueratosis oral, hiperqueratosis folicular en muslos e hiperhidrosis palmoplantar. No presentaba quistes foliculares ni antecedente de dientes natales. Sus manifestaciones clínicas se catalogaron como propias de la paquioniquia congénita tipo I. Sumado a lo anterior, se identificaron características clínicas similares en otros miembros de la familia en tres generaciones, incluidos un hermano, la madre y la abuela materna, lo cual evidenció un patrón de herencia propio de esta enfermedad.

The Wiedemann-Rautenstrauch or Neonatal Progeroid Syndrome: Report of a Patient with Gingival Hyperplasia and Severe Anterior Open Bite

Introduction: The Wiedemann-Rautenstrauch syndrome (WRS) is a rare progeroid syndrome with an autosomal recessive pattern of inheritance. The main clinical features include severe intrauterine and postnatal growth failure, distinctive facial appearance, hydrocephaly, prominent scalp veins, absence of subcutaneous fat, sparse hair of the scalp, eyebrows, and eyelashes, generalized lipoatrophy, psychomotor delay, progressive neurological deterioration, and short life expectancy. Natal teeth and micrognathia are reported as the oral manifestations of this syndrome. Case Presentation: Here is the report of a 6.5-year-old female patient with clinical signs of WRS referred to the Department of Periodontology of Shiraz School of Dentistry with the chief complaint of toothache and gingival hyperplasia. Carious teeth were extracted, and excess gingival tissue was removed through a surgical procedure under general anesthesia. The patient was the first case with a concomitant severe anterior open bite. Conclusions: Therapies for WRS are symptomatic, requiring the coordinated efforts of a team of specialists. Dentists and oral surgeons can be recruited by pediatricians and family physicians to improve the quality of life of such patients.

HIGH-POWER LASER TREATMENT FOR PERIPHERAL OSSIFYING FIBROMA IN A NEWBORN

A 2-month-old girl presented with her parents with a nodular lesion on the anterior lower alveolar ridge, which has lasted for 45 days. The girl had a natal tooth (71), which was removed when she was 15 days old. Moreover, they reported current impairment in sucking, swallowing, and breathing. Physical examination revealed a nodular, firm, and pediculate lesion, measuring 10 × 5 mm, covered by a pink, flat, and smooth mucosa. Radiograph showed the absence of tooth 71 and tooth 81 erupting under the lesion. An excisional biopsy was performed under local anesthesia using a diode laser. Microscopic examination revealed a lesion covered by squamous epithelium, presenting a proliferation of round-shaped mesenchymal cells in the lamina propria, associated with deposits of mineralized material. The final diagnosis was peripheral ossifying fibroma, and the patient is free of recurrence after 9 months. Support: CAPES.

A survey of the oral lesions in newborns and infants: A two-year cross-sectional study

Introduction. Oral lesions in newborns and infants represent a wide range of diseases often creating apprehension and anxiety among parents. Aim. We aimed to assess the type, prevalence and associated factors of oral lesions in newborns and infants. Material and methods. Participants were recruited during a two-year cross-sectional study among children who had their first teething assessed. The mothers answered medical history questions and the children underwent a clinical examination by paediatric dentists after training, calibration, and pilot study. Results. Two hundred and forty-eight children aged up to 12 months took part in the study. The mean number of teeth (SD) was 1.96 (2.55). 1.25% of children had natal teeth. Dental caries was observed in 3.2% children; traumatic dental injuries caused by falling in 7%; enamel hypoplasia in 1.9%, and mucosal lesions in 19.8% (Bohn’s nodules, mucocele, candidiasis). Conclusions. Oral lesions in infants may involve teething, teeth and oral mucosa. One child out of five children has mucosal lesions, most often Bohn’s nodules or candidiasis. There is a significant correlation between the prevalence of natal/neonatal teeth and of Bohn’s nodules.

Prematurely erupted teeth in neonates: A report of three cases

The presence of teeth in a neonate is a rare occurrence. The aetiology of these natal or neonatal teeth is still not established. Their prevalence is considered to be low, more frequently seen in females. They routinely occur in the same position as that of the deciduous dentition and more commonly in the incisor and molar regions of the mandibular arch. It requires an assiduous clinical and radiographic evaluation for diagnosis and pertinent treatment. The dentists and the child’s paediatricians should be well informed about possible clinical complexities such as Riga -Fede disease linked to natal or neonatal teeth. The clinician should consider the overall wellbeing of the infant, before extracting natal/neonatal teeth to prevent the risk associated with hypoprothrombinaemia, Periodic follow-ups should be scheduled to evaluate its recurrences or residual tooth formation. Keywords: Natal tooth, Neonatal tooth, Prematurely erupted teeth, Riga Fede disease, Residual tooth.

Fibroma osificante periférico en reborde gingival mandibular de lactante. Reporte de caso

El fibroma osificante periférico (FOP) es una lesión exclusivamente de la encía, considerada de carácter reactivo, que se presenta en forma frecuente en la segunda década de vida, siendo rara en lactantes. Cuando se evidencia en este grupo de pacientes, se observa una asociación directa con un diente natal o neonatal. El objetivo de este reporte es dar a conocer el caso de un fibroma osificante periférico que se desarrolló en el reborde alveolar mandibular de incisivos, de un lactante con antecedente de extracción de un diente natal a los tres meses de edad.

Dentes natais inferiores detectados nas primeiras horas de vida: relato de caso

O objetivo desse artigo é relatar um caso clínico de paciente com dente natal apresentando as características clínicas, causas e tratamento. Relata-se o caso de uma paciente do sexo feminino, recém-nascido com 30 horas de vida, apresentando após o parto desconforto respiratório e hipotermia grave (31,3ºC) brevemente solucionados pela equipe médica responsável. Ainda nas primeiras horas de vida, foi detectado presença de dois dentes inferiores na arcada dentária e apontados por dificultarem o aleitamento materno, tendo sido encaminhado para o serviço de Odontologia, do Hospital Universitário Lauro Wanderley.Ao exame físico intraoral não foi visto lesões ou traumas em mucosa e comprovou-se a presença de dois dentes na região dos incisivos centrais inferiores que apresentavam mobilidade acentuada. Em virtude da dificuldade de aleitamento, mobilidade dentária e preocupação dos dentes serem aspirados, tornou-se necessário fazer a remoção dos elementos. O procedimento foi realizado sob anestesia local tópica com Lidocaína spray 5%, aplicada em haste de algodão. Em seguida, foi utilizado um descolador de Molt, remoção de fina película de mucosa que recobria os dentes e realizado a exérese dos dois elementos dentários. Não foi necessário realizar sutura e não houve intercorrências durante procedimento cirúrgico.Desse modo, conclui-se que esta condição rara necessita de um trabalho multidisciplinar entre a equipe responsável pela gestante e o recém-nascido que devem reconhecer esta anomalia o mais brevemente possível para poder encaminhar à um cirurgião-dentista para definir em conjunto qual a melhor conduta.

TRAUMATIC SUBLINGUAL ULCERATION IN A NEWBORN

Natal and neonatal teeth are those which are present in the oral cavity at the time of birth and within thirty days of birth. They are likely to be associated with traumatic sublingual ulceration caused to the suckling as well as movements of the tongue. The treatment of choice is mostly conservative whenever possible. The purpose of this report is to present a case of traumatic sublingual ulceration in a twenty days old baby. The lesion resolved soon after the offending tooth was removed.

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English

Genodermatoses with teeth abnormalities.

Many genodermatoses exhibit abnormal teeth findings. Studies examining these entities are scarce and narrow in their scope. This paper reviews the evolution, development, and structure of the tooth and provides a summary of genodermatoses with aberrant dental findings. The latter are classified according to the abnormal dental findings: periodontal disease, anodontia/oligodontia/hypodontia, polydontia, enamel hypoplasia, natal teeth, dental pits, and others. Finally, we provide an algorithm that dermatologists and dentists can follow to better recognize genodermatoses with dental involvement.

A Review of Benign Congenital Anomalies.

Benign congenital anomalies are common in neonates. Some of these common benign congenital anomalies include preauricular pits, sacral dimples, supernumerary digits, accessory nipple, and natal teeth. It is important for health care providers who take care of newborns to recognize these benign anomalies so that unnecessary evaluations and investigations are not performed. However, some of these anomalies may be associated with clinically significant conditions. In this article, we discuss when further evaluation should be performed in babies presenting with these common benign congenital anomalies. [Pediatr Ann. 2020;49(2):e66-e70.].

Revisiting pachyonychia congenita: a case-cohort study of 815 patients.

Pachyonychia congenita (PC) is a group of autosomal dominant disorders caused by mutations in one of five keratin genes (KRT6A, KRT6B, KRT6C, KRT16, KRT17). The establishment of an international registry containing clinical and molecular data led to the development of a disease classification based on the mutant gene and associated features. To harness the same resource to clarify the prevalence of PC-associated clinical features, delineate phenotype-genotype correlations and identify prognostic features for disease severity. In total, 815 individuals with confirmed keratin mutations registered in the International Pachyonychia Congenita Research Registry were surveyed for clinical findings associated with PC. Data were analysed using various statistical methods, including the Student's t-test, χ2 -test and anova tests for differences in means/proportions. Spearman correlation and logistic regression were used for phenotype-genotype correlations. KRT6A mutations were associated with oral leucokeratosis, hoarseness, youngest age or highest number of fingernails/toenails involved, and use of walking aids. KRT17 mutations were most commonly associated with cysts and natal teeth. Using logistic regression, we found that oral leucokeratosis was correlated with earlier toenail involvement, walking aids, nursing difficulties and hoarseness. Cysts were correlated with oral leucokeratosis, natal teeth and ear wax. Natal teeth predicted earlier toenail involvement, walking difficulties and cyst formation. Hoarseness was correlated with an increased number of involved fingernails. Here, we establish phenotype-genotype correlations in the largest cohort of patients with PC described to date and reveal novel and clinically useful predictors of disease course and manifestations. What's already known about this topic? Pachyonychia congenita (PC) is a group of autosomal dominant disorders caused by mutations in one of five keratin genes (KRT6A, KRT6B, KRT6C, KRT16, KRT17). The main clinical features are nail dystrophy, palmoplantar keratoderma, oral leucokeratosis and cysts. The establishment of an international registry containing the clinical and molecular data of patients with PC led to the development of a disease classification based on the mutant gene and associated features. What does this study add? Data were collected via an international registry to clarify the prevalence of PC-associated clinical features, delineate phenotype-genotype correlations and identify prognostic features for disease severity. This is the largest cohort of patients with PC described to date. The earliest clinical manifestations of PC are nail dystrophy and palmoplantar keratoderma. Diagnosis can be suspected and confirmed in preschool years. Painful plantar keratoderma has the most profound and debilitating effect on quality of life and daily function. Linked Editorial: Steele and O'Toole. Br J Dermatol 2020; 182:521-522. Linked Comment: Mordaunt. Br J Dermatol 2020; 182:537.

Effectiveness of an Oral Health Education Program to Improve Mothers’ Awareness of Natal Teeth: A Randomized Controlled Study

Objective: To evaluate the effectiveness of an oral health talk aided by a video on improving the awareness of mothers about natal teeth in two rural communities in southwest Nigeria. Material and Methods: A cluster-randomized controlled trial was conducted among 80 mothers in two rural communities in Nigeria, randomized into study and control groups. Baseline information was obtained with a pre-tested interviewer administered questionnaire. The mothers in the study group participated in an oral health education program comprising of a video show on natal teeth in addition to an oral health talk. The control group did not receive any intervention. Post-intervention data was obtained three weeks afterwards. Data collected was analyzed with SPSS using paired t-test, independent t-test and Chi-square test . Results: The sociodemographic characteristics of participants in both groups were similar. The mean knowledge, attitude and practice percentage scores for the study group were 38.9 ± 26.3% (pre-intervention) and 73.6 ± 26.2% (post-intervention); while for the control group, the values were 43.2 ± 31.4% (pre-intervention) and 43.9 ± 32.4% (post-intervention). The study group, had a 34% increase in knowledge, attitude and practice percentage score (CI=23.7-45.5; t=6.4; p<0.001), while the control group, had an increase of 0.7% (CI=-0.7-2.2; t=1.00; p=0.323) . Conclusion: An oral health education program comprising of oral health talk and a video about natal teeth improved the awareness of mothers about natal teeth as a normal phenomenon.

Assessment of Intraoral Findings of Neonates, Born in and around Meerut City.

ABSTRACTAimThe aim of this study is to evaluate the occlusal relationships between the gum pads of neonates and to record intraoral findings in neonates and compare with the existing findings.Materials and methodsThe oral cavities of 1,000 healthy neonates from birth to 1 month of age were examined to evaluate the occlusal relationships between the gum pads and to record other intraoral findings in neonates and compare with the existing findings. Oral findings recorded were oral mucosal cysts, Fordyce's spot, Epstein's pearls, Bohn's nodules, ankyloglossia, natal or neonatal teeth and attachment of the upper midline frenum. Relationship between the alveolar ridges was also recorded. Obtained data were evaluated and statistically analyzed using the Statistical Package for Social Sciences (SPSS) version 21.0 and Epi-info version 3.0.ResultsThe maxillo-mandibular relationship of gum pads in approximately 97% cases, the mandible was found distal and lingual to maxilla. The presence of anterior open bite was observed in 9.1% cases. The most common intraoral finding in examined neonates was an oral mucosal cyst, 61.2%. The presence of Epstein's pearls was the next most common finding 38.3%. Fordyce's spots were found only in 1.2% of cases, while the presence of natal/neonatal teeth was only in 0.6% of cases. Ankyloglossia was present in only 0.5% neonates. Maxillary frenum was present in 79.1% of cases and it was attached on the alveolar crest.ConclusionThe results of the present study were in accordance with the pre-existing data. However, anterior open-bite was seen in significantly less percentage of the population.How to cite this articleNiranjan MM, Srivastava N, Rana V, et al. Assessment of Intraoral Findings of Neonates, Born in and around Meerut City. Int J Clin Pediatr Dent 2020;13(1):48–52.

Natal teeth associated with Riga-Fede disease (Sublingual traumatic disease/traumatic lingual ulceration): A case report and review of literature

Natal teeth associated with Riga-Fede disease (Sublingual traumatic disease/traumatic lingual ulceration): A case report and review of literature

Concurrent presentation of dental lamina cysts of the new born and natal tooth in a child: a case report and literature review

Dental lamina cysts and natal/neonatal teeth are rare findings in a neonate with the dental lamina cysts presenting as white or pink small nodules, often multiple, approximately 1 to 3mm in diameter. They are usually located on the alveolar mucosa of both maxilla and mandible and also on the midpalatine raphe; the whitish colored lamina cysts found on the alveolar mucosa can be confused clinically with neonatal teeth. Neonatal teeth are teeth found in infants within the first 30 days of life, and they present a social stigma to the parent. They have been reported to be a component of the full complement of teeth in 90-95% of cases. Apart from the social stigmata, they can also induce trauma to surrounding soft tissues and / or to the mother’s breast (Riga-Fede phenomenon). While the dental lamina cysts usually resolve spontaneously within few weeks to months, there are indications that might preclude extraction of a natal/neonatal tooth.Keywords: Dental lamina cyst, Epstein’s pearls, Mucosal cyst, natal/neonatal tooth

Natal Tooth: A Histomorphologic Variant, a Rarity

AbstractPresence of natal or neonatal teeth in a newborn is rare, that is, 1 in 3,000 cases. Various etiological factors have been put forth explaining the presence of natal teeth but they are not very clear. Although some authors have suggested that these teeth may represent predeciduous supernumerary teeth, most of these teeth represent prematurely erupted portions of the deciduous dentition, not supernumerary teeth. They can be left untreated in some cases if they do not cause any difficulty to the mother while feeding or do not pose a risk of swallowing in the newborn. One such case of natal tooth with histological variation is presented here.

Literature review and micro-computed tomography analysis of natal teeth: A pilot study.

To outline the current literature surrounding natal teeth, and then, in a pilot study, to evaluate natal teeth using micro-computed tomography (micro-CT) to determine their anatomical profile, and compare and contrast different analytical methods to assess natal teeth. 2 extracted natal teeth (mandibular central incisors) and 1 exfoliated mandibular primary central incisor were subjected to micro-CT analysis. Within natal teeth, there were no statistical differences in tooth mineral density (TMD) of both enamel and dentine (P>.05), whereas mandibular primary central incisors had a significantly higher TMD of both enamel and dentine in comparison with both natal tooth 1 and natal tooth 2 (P<.05). Mandibular primary central incisors had a greater thickness and volume of both enamel and dentine, but exhibited lower pulpal space volume. Micro-CT is an alternative and non-invasive method to anatomically assess natal teeth. According to the pilot study, natal teeth exhibited lower TMD, decreased enamel and dentine thickness, and smaller pulpal space volume in comparison with mandibular primary incisor teeth. This pilot study creates a foundation to establish the collection and analysis of natal teeth on a larger scale over time using micro-CT.