Ectodermal dysplasia is a group of hereditary diseases characterized by developmental defects of ectodermal structures. Its oral manifestations mainly center on congenital missing teeth, abnormal tooth morphology, and maxillofacial bone developmental disorders, which seriously affect the masticatory function, maxillofacial development, and mental health of affected children. In this article, the multidimensional diagnostic strategy system for children with ectodermal dysplasia and the related progress of early oral prosthodontic treatment methods were systematically reviewed to provide references for clinicians in the diagnosis and treatment of children with ectodermal dysplasia.
Child ; Humans ; Anodontia ; Ectodermal Dysplasia/diagnosis* ; Prosthodontics ; Tooth Abnormalities/therapy*

OBJECTIVES: This study aimed to investigate the clinical characteristics of congenital deciduous teeth absence and its permanent teeth performance type by using panoramic radiographs. METHODS: A total of 15 749 panora-mic radiographs of 3-6-year-old children with deciduous dentition were collected from January 2020 to December 2021. The incidence of congenital deciduous teeth absence was observed, and the abnormality of permanent teeth was recor-ded. SPSS 24.0 software was used for statistical analysis. RESULTS: The incidence of congenital deciduous teeth absence was 2.54% (400/15 749), which was found in 217 girls and 183 boys, and the difference between the genders was statistically significant (P=0.003). The absence of one and two deciduous teeth accounted for 99.75% (399/400) of the subjects. In addition, 92.63% (490/529) of mandibular deciduous lateral incisor was congenitally absent, 44.80% (237/529) of deciduous teeth was absent in the left jaw, and less than 55.20% (292/529) was absent in the right; the difference between them was statistically significant (P=0.017). The absence of 96.41% (510/529) deciduous teeth in the mandibular was significantly more than that of 3.59% (19/529) in the maxillary, and the difference between was statistically significant (P=0.000). Furthermore, 68.00% (272/400) and 32.00% (128/400) of deciduous teeth were absent in unilateral and bilateral, respectively, and the difference was statistically significant (P=0.000). Four types of congenital deciduous teeth absence with permanent teeth were observed as follows: 1) 73.91% (391/529) of permanent teeth was absent; 2) 20.60% (109/529) of permanent teeth was not absent; 3) the number of fused permanent teeth accounted for 4.91% (26/529); 4) the number of supernumerary teeth was 0.57% (3/529). CONCLUSIONS Although the absence of congenital deciduous teeth is less common than that of permanent teeth, it affects deciduous and permanent teeth to some extent. Dentists should pay attention to trace and observe whether abnormalities are present in the permanent teeth and take timely measures to maintain children's oral health.
Male ; Female ; Humans ; Tooth, Deciduous ; Dentition, Permanent ; Tooth, Supernumerary/epidemiology* ; Anodontia/etiology* ; Tooth Abnormalities/complications* ; Fused Teeth/epidemiology*

Fused teeth were a phenomena of teeth anomalies in shape, which can affect the dental teeth both in primary and permanent dentition. Fused teeth do not only cause problems on crowding of dentition, abnormal occlusion and aesthetic, but also increase risks of dental caries, endodontics diseases, periapical diseases and periodontal diseases. Fusion of deciduous teeth may lead to abnormality of subsequent permanent teeth. Treatment of fused teeth may require multidisciplinary approach in endodontics, periodontics, oral and maxillofacial surgery, prosthodontics and orthodontics. The aim of the present article is to review the etiology, classification, clinical manifestations and treatment of fused teeth in order to provide dental clinicians with a reference of clinical management for fused teeth.
Humans ; Fused Teeth/therapy* ; Anodontia ; Tooth, Deciduous ; Dental Caries/therapy* ; Esthetics, Dental

Humans ; Anodontia/genetics* ; Tooth Abnormalities/genetics* ; Mutation ; Malocclusion/genetics* ; Wnt Proteins/genetics*

Long-term missing teeth can lead to alveolar bone loss in the edentulous area. Guided bone regeneration (GBR) is a bone augmentation method. It is widely used in clinical practice and broadens the indications of orthodontic treatment to a certain extent. This article reports a case of an adult patient with edentulous space in the maxillary central incisor, which was successfully closed through orthodontic treatment combined with GBR. This study will provide a re-ference for future clinical work.
Adult ; Alveolar Bone Loss ; Anodontia ; Bone Regeneration ; Humans ; Incisor ; Maxilla ; Orthodontic Space Closure

Tooth agenesis is the most common form of congenital craniofacial dysplasia seen in stomatology clinics, which may be caused by genetic and/or environmental factors. Tooth development is regulated by a series of signaling pathways, and variants in any of these strictly balanced signaling cascades can result in tooth agenesis and/or other oral defects. Notably, variants of genes of the Wnt/beta-catenin signaling pathway are important cause for both non-syndromic and syndromic tooth agenesis. This article has provided a review for the molecular genetics of tooth agenesis associated with Wnt/beta-catenin signaling pathway, which may shed lights on the etiology and molecular mechanism of this disease.
Anodontia/genetics* ; Genetic Research ; Humans ; Tooth ; Wnt Proteins/genetics* ; Wnt Signaling Pathway/genetics*

The goal of this study was to identify MSX1 gene variants in multiple Chinese families with nonsyndromic oligodontia and analyse the functional influence of these variants. Whole-exome sequencing (WES) and Sanger sequencing were performed to identify the causal gene variants in five families with nonsyndromic oligodontia, and a series of bioinformatics databases were used for variant confirmation and functional prediction. Phenotypic characterization of the members of these families was described, and an in vitro analysis was performed for functional evaluation. Five novel MSX1 heterozygous variants were identified: three missense variants [c.662A>C (p.Q221P), c.670C>T (p.R224C), and c.809C>T (p.S270L)], one nonsense variant [c.364G>T (p.G122*)], and one frameshift variant [c.277delG (p.A93Rfs*67)]. Preliminary in vitro studies demonstrated that the subcellular localization of MSX1 was abnormal with the p.Q221P, p.R224C, p.G122*, and p.A93Rfs*67 variants compared to the wild type. Three variants (p.Q221P, p.G122*, and p.A93Rfs*67) were classified as pathogenic or likely pathogenic, while p.S270L and p.R224C were of uncertain significance in the current data. Moreover, we summarized and analysed the MSX1-related tooth agenesis positions and found that the type and variant locus were not related to the severity of tooth loss. Our results expand the variant spectrum of nonsyndromic oligodontia and provide valuable information for genetic counselling.
Anodontia/genetics* ; Humans ; MSX1 Transcription Factor/genetics* ; Pedigree ; Whole Exome Sequencing

A 19-year-old female patient came with the chief complaint that the upper anterior teeth looked small and with many spaces. The patient claimed that the tooth had never been replaced since birth. The patient wanted to improve the aesthetics of the tooth. Treatment, in this case, was made using a fixed prosthetic to aesthetically rehabilitate through prosthodontic treatment in the anterior teeth agenesis followed by attrition. It can be concluded that fixed prosthodontic treatment using a splint bridge is one treatment option that can be used in multiple anterior teeth agenesis followed by tooth wear. An appropriate material selection increases the aesthetics and functional aspects of the final treatment.
Denture, Partial, Fixed ; Anodontia ; Dental Occlusion ; Humans ; Health

ABSTRACT Globally, non-syndromic tooth agenesis is commonly seen in clinical practice. However, its management is often complex and requires a multidisciplinary team approach for the maximal outcome. While various treatment options are possible, considerations for the treatment are not only based on the dentofacial conditions but also cultural and social background and personal preference of the patient. Thus, patientcentred care approach should always be practised for an optimal outcome. In the present case, a patient with established craniofacial growth presenting with bilateral agenesis of maxillary lateral incisors and over-retained deciduous maxillary left canine sought for aesthetic improvements. The patient did not prefer any orthodontic treatment citing a prolonged treatment duration and sub-optimal motivation as a hindrance. Thus, a prosthodontic only approach was taken by providing a conventional cantilever bridge and ceramic veneers to achieve the aims of treatment. This article discusses the possible limitation of such prosthodontic only solution in managing tooth agenesis.
Prosthodontics ; Anodontia

Oligodontia is a condition in which the patient has more than six ageneses, excluding the third molars. Whereas the absence of one tooth is quite common, oligodontia is a rare congenital condition that can happen with or without the syndrome. The condition happens due to disturbances during odontogenesis process. Management of oligodontia is a long-term process, and it involves prosthetic, restorative, and orthodontic treatment to support the oral function and esthetic of the patient. This article aims to report a rare case of agenesis of 13 permanent teeth (excluding third molars) in an 11-year-old girl and her treatment plan.
Anodontia