Abnormal dental phenotypes in GAPO syndrome: A descriptive study with a new ANTXR1 variant & insights on teeth eruption.

Objective: GAPO syndrome is usually diagnosed clinically owing to its characteristic features of growth retardation, alopecia, pseudoanodontia, and ophthalmic anomalies. Pseudoanodontia describes the failure of eruption of the two sets of teeth in these patients. Thus, the abnormal dental phenotype is the emergence of a set or part of a set of dentitions. Purpose: This study reports the physical, oro-dental, and molecular findings of two new sibs with GAPO syndrome and provides a description of the dental phenotype of one of the patients reported before. Materials & Methods: The patients were subjected to full medical history taking and three generations-pedigree construction. They were phenotyped according to the elements of morphology: Standard terminology series. After parental consents were acquired, molecular analysis was carried out for the two sibs (Patient 1 & 2). Results: These included a new gene variant associated with erupted teeth in GAPO syndrome and new clinical features. A new classification for the terminologies of eruption disturbances was suggested. Conclusion: The study asserts the importance of oro-dental examination and follow-ups as dental updates may occur in these cases.

Endodontic Management of a Mandibular Second Premolar with Three Root Canals and Taurodontism: A Case Report.

A complete understanding of canal morphology is essential to achieve successful endodontic treatment. Although mandibular second premolars are reported to have usually only one root canal at the apex, in this case we reported a mandibular second premolar with three root canals and taurodont morphology. A patient was referred to the endodontic department at Mashhad Faculty of Dentistry with a chief complaint of pain in her mandibular right second premolar tooth. The diagnosis of pulpal necrosis and chronic apical periodontitis was established. The initial radiograph showed a taurodont premolar with two roots. However, with cone-beam computed tomography assistance, three root canals were confirmed. The root canal treatment process was successfully managed with a dental operating microscope. Using cone-beam computed tomography and a dental operating microscope greatly facilitated the determination of tooth morphology and successful following nonsurgical endodontic treatment.

Endodontic management of taurodontism in a patient with Morquio syndrome: Case report of a 16-year-old girl.

BACKGROUND AND AIM: Mucopolysaccharidosis (MPS) type IV, referred to as Morquio A syndrome (MAS), is a rare genetic disorder characterized by an insufficient level in the storage of glycosaminoglycans within lysosomes. Diagnosis generally depends on clinical examination, skeletal radiographs, and histochemical tests. The condition is characterized by prominent skeletal deformities, limited joint mobility, significant growth impairment, abnormalities in tooth alignment, and defects in tooth enamel. The present clinical case report aims to provide details of a MAS case with peculiar dental findings, including multiple taurodonts where emergency dental treatment was rendered. CASE REPRESENTATION: A 16-year-old girl with short stature and prominent facial characteristics reported severe pain in the right maxillary back region. Clinical examination revealed multiple areas of alveolar bone loss, decayed #36, and temporary restoration on #16. Radiographic examination indicated multiple posterior teeth with taurodontism. Past medical history was confirmative of Morquio syndrome. Treating taurodonts endodontically is challenging due to the enlarged pulp chamber, shortened roots, and constricted root canal anatomy. Motorized canal preparation and the use of bioceramic sealer with single cone obturation were done to achieve the best results. CONCLUSION: The present case report encountered difficulty locating and negotiating the root canals and establishing the glide path. Using a dental operating microscope and stiff hand files for glide path and short-length Niti rotary files helped achieve the desired results. Although MPS IV is not frequently seen in dental practice, persons with this syndrome can continue to have good oral and overall health if their dental and medical condition if appropriately managed.

Generalized Non-syndromic Hypertaurodontism With Delayed Eruption: A Case Report.

Taurodontism is a rare dental anomaly defined by a change in tooth shape due to Hertwig's epithelial sheath not folding inward at the right horizontal level. It has a larger pulp chamber and a pulpal floor that is shifted apically, and the cementoenamel junction (CEJ) is not constricted. This condition is more frequently observed in permanent teeth than in primary teeth and can occur in a bilateral or unilateral manner, affecting any quadrant or group of teeth. This brief case report discusses a 14-year-old female patient who presented with complaints of decayed teeth in the lower right and left posterior regions of the jaw. Radiographic examination revealed the presence of non-syndromic taurodontism in both the deciduous teeth and their permanent successors. Dental management included oral prophylaxis, application of pit and fissure sealants, indirect pulp capping, and restoration with glass ionomer cement for the affected teeth.

Endodontic management of taurodontic teeth: A case series.

This paper illustrated successful endodontic non-surgical (re)treatments of molars and premolar with different taurodontic classifications and accompanied anomalies like C-shape and extra root canals in otherwise healthy patients. Magnification with illumination besides active irrigation with NaOCl were the most helpful items in managing these cases.

Genetic Variants in KCTD1 Are Associated with Isolated Dental Anomalies.

KCTD1 plays crucial roles in regulating both the SHH and WNT/ß-catenin signaling pathways, which are essential for tooth development. The objective of this study was to investigate if genetic variants in KCTD1 might also be associated with isolated dental anomalies. We clinically and radiographically investigated 362 patients affected with isolated dental anomalies. Whole exome sequencing identified two unrelated families with rare (p.Arg241Gln) or novel (p.Pro243Ser) variants in KCTD1. The variants segregated with the dental anomalies in all nine patients from the two families. Clinical findings of the patients included taurodontism, unseparated roots, long roots, tooth agenesis, a supernumerary tooth, torus palatinus, and torus mandibularis. The role of Kctd1 in root development is supported by our immunohistochemical study showing high expression of Kctd1 in Hertwig epithelial root sheath. The KCTD1 variants in our patients are the first variants found to be located in the C-terminal domain, which might disrupt protein-protein interactions and/or SUMOylation and subsequently result in aberrant WNT-SHH-BMP signaling and isolated dental anomalies. Functional studies on the p.Arg241Gln variant are consistent with an impact on ß-catenin levels and canonical WNT signaling. This is the first report of the association of KCTD1 variants and isolated dental anomalies.

The relevance of taurodontism in forensic dental age estimation.

Taurodontism is a dental morphological anomaly characterized by enlarged pulp cavities repositioned towards the apical region of the tooth, coupled with shortened root structures. Molars are commonly affected by this alteration. Certain populations exhibit up to 48% prevalences for this dental alteration, underscoring its significance in dental age estimation (DAE). In the field of DAE, an individual's chronological age is inferred from specific dental features, frequently employed within the forensic context. The effect of taurodontism on the features of DAE is an unanswered issue. The influence of taurodontism on eruption, mineralization, radiographic visibility of root canals, and radiographic visibility of the periodontal ligament space in mandibular third molars- some of the established criteria for DAE as examples-is currently not systematically examined. Some common staging scales for the dental features of DAE cannot technically be applied to taurodontic teeth. Additionally, given the association of taurodontism with syndromes affecting tooth development, caution is warranted in age assessment procedures. Notably, taurodontic teeth may serve as indicators of syndromes influencing skeletal development, further emphasizing the relevance of taurodontism in forensic age assessment. Presumably taurodontic teeth were included in reference data to some extent due to their partially high prevalence in the past, whereby the influence of taurodontism has been statistically absorbed within the overall spread of the features. Future studies should compare the temporal course of these tooth characteristics in affected and unaffected teeth. Subsequent initiatives should focus on raising awareness among forensic dentists regarding taurodontism, necessitating in-depth exploration of the subject.

Dental Anomalies in Primary Dentition among Arabian Children: A Hospital-Based Study.

An observational study was carried out in a teaching hospital in Saudi Arabia to determine the occurrence of dental anomalies among Arabian children. The study included children of Saudi nationality with primary teeth. The study assessed the prevalence of dental anomalies in their primary dentition. The assessment and data collection were conducted by a single examiner, utilizing clinical examination and intra-oral radiographs. A comparative analysis was conducted to examine dental anomalies in relation to gender (boys and girls) and arch type (maxillary and mandibular). In addition, the study explored the occurrence of gender-specific dental anomalies depending on arch type. The data analysis was conducted using IBM Statistics (version 21.0) with a significance level of p < 0.05. In total, there were 245 children included in the final analysis. The study population consisted of boys (66%) and girls (34%), with an average age of 4.87 ± 0.9 years. Taurodontism was the most prevalent dental abnormality, occurring in 2.8% of the individuals in the study sample. The study sample exhibited hypodontia in 2%, supernumerary teeth in 2.4%, double teeth in 2%, and microdontia in 1.2%. Talon cusp and macrodontia have a relatively low incidence of 0.4%. Boys exhibit supernumerary teeth, microdontia, macrodontia, talon cusp, and taurodontism, whereas hypodontia and double teeth were more frequent in girls.

Prevalence and Distribution of Dental Anomalies in Schoolchildren in Kuwait.

OBJECTIVE: Dental anomalies (DA) can affect paediatric patients' aesthetics, function, and psychological well-being. There is a lack of data about the prevalence of DA in children in Kuwait. This study aimed to investigate the prevalence and distribution of DA amongst schoolchildren aged 8 to 12 years. METHODS: A retrospective study was conducted using panoramic digital radiographs of children who attended a single dental center. All radiographs were evaluated by 2 calibrated and trained examiners. RESULTS: DA were present in 110 (20.1%) out of the 546 panoramic radiographs examined: 53.6% in females and 46.4% in males. The mean age of children with DA (9.83 ± 1.29) was similar to that of children with no anomalies (9.96 ± 1.46). The most prevalent anomaly was dental agenesis (9.3%), followed by taurodontism (6.6%) and ectopic eruption (EE, 2%). DA were more common in the maxilla (58.2%) compared to the mandible (41.8%, P = .042). Congenitally missing teeth were significantly more frequent in the mandible (56.9%) than in the maxilla (43.1%, P = .003). EE was significantly more common in the maxilla (90.9%) than in the mandible (9.1%, P = .024). Microdontia and root dilacerations were only present in males, whilst supernumerary teeth, transposition, and impacted teeth were noted in females only. CONCLUSIONS: The prevalence of DA amongst schoolchildren in Kuwait was considered to be relatively high. Certain DA were associated with gender. The significant prevalence of DA highlights the need for early diagnosis using panoramic radiographs, particularly during the ages of 9 and 10, in order to ensure effective patient management.

LRP4 mutations, dental anomalies, and oral exostoses.

BACKGROUND: In order to generate a normal set of teeth, fine-tuning of Wnt/ß-catenin signaling is required, in which WNT ligands bind to their inhibitors or WNT inhibitors bind to their co-receptors. Lrp4 regulates the number of teeth and their morphology by modulating Wnt/ß-catenin signaling as a Wnt/ß-catenin activator or inhibitor, depending on its interactions with the partner proteins, such as Sostdc1 and Dkk1. AIM: To investigate genetic etiologies of dental anomalies involving LRP4 in a Thai cohort of 250 children and adults with dental anomalies. DESIGN: Oral and radiographic examinations and whole exome sequencing were performed for every patient. RESULTS: Two novel (p.Leu1356Arg and p.Ala1702Gly) and three recurrent (p.Arg263His, p.Gly1314Ser, and p.Asn1385Ser) rare variants in low-density lipoprotein receptor-related protein 4 (LRP4: MIM 604270) were identified in 11 patients. Oral exostoses were observed in five patients. CONCLUSION: Antagonism of Bmp signaling by Sostdc1 requires the presence of Lrp4. Mice lacking Lrp4 have been demonstrated to have alteration of Wnt-Bmp-Shh signaling and an abnormal number of incisors. Therefore, the LRP4 mutations found in our patients may disrupt Wnt-Bmp-Shh signaling, thereby resulting in dental anomalies and oral exostoses. Root maldevelopment in the patients suggests an important role of LRP4 in root morphogenesis.

Prevalence of Taurodontism in Contemporary and Historical Populations from Radom: A Biometric Analysis of Radiological Data.

Taurodontism is a morphological anomaly of multirooted molars characterized by apical displacement of the pulp chamber, shortened roots, and the absence of constriction at the dentoenamel junction. It can negatively impact the outcome of dental treatment plans. This study aimed to compare the prevalence of taurodontism among contemporary and historical populations from Radom, Poland. Five hundred eighty-two panoramic radiographs of contemporary patients and 600 radiographs of historical individuals were analyzed using the Shifman and Chanannel index. Group differences were determined with Pearson's chi-square tests according to sex, site, tooth group, and historical period. The study also evaluated the degree of severity of taurodontism in relation to dental groups, gender, and the periods from which contemporary patients as well as historical individuals originated. In the contemporary population, taurodontism was observed in 34% of individuals. In the historical data, the highest prevalence of taurodontism (31%) was observed among individuals from the 18th and 19th centuries, while earlier periods exhibited considerably lower prevalence rates. Across contemporary and historical populations, the maxillary molars were the most commonly affected teeth. Hypotaurodontism was the most prevalent form of taurodontism. The prevalence of taurodontism has gradually increased from the 11th century to the current day. The results of the research are of great importance for the clinician in terms of planning comprehensive dental treatment.

Multiple Developmental Dental Anomalies in a Nonsyndromic Patient: A Rare Case Report.

The present case report illustrates a rare occurrence of multiple dental anomalies in a healthy 12-year-old patient including anomalies of shape and number of teeth. The developmental dental anomalies noticed in the present case scenario are dens invaginatus, taurodontism, radix entomolaris, supernumerary teeth, and pulp stones seen in multiple teeth. It is not rare to notice these anomalies individually, but their combination is rare in a single, non-syndromic patient. This report also signifies the importance of thorough extra-intra-oral and radiographic examinations of patients with dental anomalies.

Phenotypic characteristics of taurodontism and a novel WNT10A variant in non-syndromic oligodontia family.

OBJECTIVE: Variants in wingless-type MMTV integration site family member 10A (WNT10A) have been proposed to be the most common cause of non-syndromic oligodontia (NSO). The goal of the present study was to identify the novel WNT10A variants in Chinese families with NSO. DESIGN: Clinical data were collected from 39 families with oligodontia admitted to the Hospital of Stomatology Hebei Medical University (China) from 2016 to 2022. Whole-exome sequencing (WES) and Sanger sequencing were performed to identify WNT10A variants in three families with non-syndromic oligodontia. Amino acid conservation analysis and protein conformational analysis were conducted for the WNT10A variant. Genotype-phenotype analysis was performed on the previously reported WNT10A variants related to NSO. RESULTS: We found a novel heterozygous WNT10A variant c.1127 G>A (p.Cys376Tyr) and two reported heterozygous variants c.460 C>A (p.Leu154Met) and c.511 C>T (p.Arg171Cys). Structural modeling showed that the novel WNT10A variant was located in a highly conserved domain, which led to structural damage of WNT10A protein. In addition, we found that the phenotype of the WNT10A variants affected the maxillary second premolars, followed by the mandibular second premolars, and rarely affected the maxillary central incisor. Herein, it is the first time to report that NSO patients with WNT10A monoallele mutation carry taurodontism phenotype and 6.1% prevalence of taurodontism in WNT10A-related NSO patients. CONCLUSIONS: Our results demonstrated that the novel variant c.1127 G>A (p.Cys376Tyr) of WNT10A causes NSO. The present study expanded the known variation spectrum of WNT10A and provided valuable information for genetic counseling of families.

Challenges and Treatment Considerations with Comprehensive Rehabilitation of a Case of Taurodontism in Primary Dentition: A Case Report and Review.

Background: Taurodontism is an anomalous developmental condition where the coronal portion of the tooth is enlarged while the radicular portion is compromised. This may have important clinical implications while considering endodontic or exodontic procedures which may affect the technique of the treatment or its prognosis. Case Report: This article describes the case of a 4-year-old female girl with molars exhibiting taurodontism with its clinical management and a review of this unique clinical condition. Conclusion: This case report and review summarizes the diagnosis, classification, clinical management challenges and comprehensive rehabilitation of taurodontism in primary teeth. How to cite this article: Gupte MP, Mistry LN, Jawdekar A. Challenges and Treatment Considerations with Comprehensive Rehabilitation of a Case of Taurodontism in Primary Dentition: A Case Report and Review. Int J Clin Pediatr Dent 2023;16(3):510-514.

Solitary median maxillary central incisor in association with taurodontism and oligodontia: A case report.

In this case report, we have highlighted the probability of coinciding multiple dental anomalies, such as SMMCI, taurodontism, and oligodontia. Thus, further research is necessary to establish a correlation between oligodontia, taurodontism, and SMMCI.

An International Investigation of Molar Incisor Hypomineralisation (iMIH) and Its Association with Dental Anomalies: Development of a Protocol.

BACKGROUND: Molar incisor hypomineralisation (MIH) is a common disorder of tooth development, which has recently been found to be associated with a higher prevalence of hypodontia. The aim of this international multicentre study is to determine the association between MIH and other developmental anomalies in different populations. METHODS: Investigators were trained and calibrated for the assessment of MIH and dental anomalies and ethical approvals obtained in each participating country. The study aimed to recruit 584 children with MIH and 584 children without MIH. Patients aged 7-16 years who attend specialist clinics will be invited to participate. Children will undergo a clinical examination to determine the presence and severity of MIH, using an established index. The presence of any other anomalies, affecting tooth number, morphology, or position, will be documented. Panoramic radiographs will be assessed for dental anomalies and the presence of third permanent molars. Statistical analysis, using a chi squared test and regression analysis, will be performed to determine any differences in dental anomaly prevalence between the MIH and non-MIH group and to determine any association between dental anomalies and patient characteristics. CONCLUSION: This large-scale study has the potential to improve understanding about MIH with benefits for patient management.

Taurodontism in simultaneous existence with multiple supernumerary teeth: A case report.

Supernumerary teeth and taurodontism are both common dental anomalies when found as isolated entities. However, simultaneous presence in the same individual without a syndrome is rarely reported in the literature. This case report describes a 20-year-old non-syndromic male with multiple supernumerary teeth in association with multiple molar and premolar taurodonts and no family history of genetic predisposing factors. Taurodontism was measured and confirmed through cone-beam computed tomography for accuracy of the classification. Early diagnosis with the aid of radiographic imaging and persistent follow-up are crucial to treatment.

Prevalence of Dental Anomalies and Its Role in Sex Estimation among Children of Jazan Region, Saudi Arabia.

BACKGROUND: This study aimed to ascertain the prevalence of dental anomalies and their ability to estimate sex status. MATERIAL AND METHODS: This cross-sectional radiographic study was based on the evaluation of dental anomalies of Saudi children aged between 5 and 17 years. A total of 1940 orthopantomograms (OPG) were screened, of which 1442 were included. All the OPGs were digitally evaluated with ImageJ software. The demographic variables and dental anomaly findings were subjected to descriptive and comparative statistical analysis. Discriminant function analysis was conducted for sex estimation. p value < 0.05 was considered as significant. RESULTS: The mean age of the children in this study was 11.35 ± 0.28 years. At least one dental anomaly was detected in 161 children (11.17%), including 71 males and 90 females. Only 13 children (8.07%) presented with more than one anomaly. The most common dental anomaly detected was root dilaceration (47.83%) followed by hypodontia (31.68%). The least common dental anomaly was infraocclusion (1.86%). The sex prediction accuracy using discriminant function analysis was 62.9% (p < 0.01). CONCLUSION: The prevalence of dental anomalies was 11.17% with root dilaceration and hypodontia being the most common. The role of dental anomalies in sex estimation was found to be ineffective.

Prevalence of taurodontism in individuals in Northwest China determined by cone-beam computed tomography images.

Objective: The aim of this retrospective study was to evaluate the prevalence of taurodontism in a group of adult dental patients in Northwest China with the aid of cone-beam computed tomography (CBCT). Methods: This study used Shifman and Chanannel's criteria to statistically analyze the prevalence of taurodontism in the premolars and molars of the Chinese population. CBCT images of 5488 teeth from 580 subjects of Chinese origin were evaluated. The measured data were statistically analyzed and the chi-square test was also used to compare the prevalence of taurodontism between male and female subjects and between the upper and lower jaws (P < 0.05). Results: Taurodontism was detected in 169 patients, with a prevalence of 29.14%, of which 27.24% were males and 30.65% were females. The chi-square test showed that there was no significant difference between males and females (P > 0.05). Taurodontism was found in 7.45% of all teeth examined. Taurodonts were significantly more common in the maxilla (9.06%) than in the mandible (5.15%) (P < 0.001), and the maxillary second molar (25.18%) was the most common tooth affected. According to morphology, hypotaurodonts were the most common (60.39%) among taurodontic teeth. Conclusions: Taurodontism was relatively common in the Chinese population and was almost equally distributed between males and females. The maxillary second molar was the most common tooth of all taurodonts measured, and taurodonts were significantly more common in the maxilla than in the mandible. Hypotaurodontism was the most common form of taurodontism. Our study provides a reference for dental deformities in the Chinese population and the diagnosis and treatment of taurodontism.