Generalised hypomineralisation of enamel in oculodentodigital dysplasia: comprehensive dental management of a case

2021 ◽  
Vol 14 (1) ◽  
pp. e238079
Author(s):  
Emilija Daina Jensen

Oculodentodigital dysplasia (ODDD) is a rare congenital disorder characterised by developmental abnormalities of the eye, dentition and digits of the hands and feet, with neurological symptoms reported in 30% of individuals. Dental anomalies associated with ODDD include enamel hypoplasia and subsequent caries, microdontia, missing teeth, amelogenesis imperfecta, pulp stones and delayed tooth development. Here, we describe the comprehensive dental management of a 3-year-old girl who presented with rapid deterioration of the primary dentition due to generalised enamel hypomineralisation. Conservative, comprehensive restorative management was performed under general anaesthesia. Within 6 months, further breakdown of the remaining unrestored enamel was noted. This case documents the challenges of conservative management in dental anomalies that are not well documented due to the extreme rarity of the disorder.

2022 ◽  
Vol 2 ◽  
Author(s):  
Rasha N. Alotaibi ◽  
Brian J. Howe ◽  
Lina M. Moreno Uribe ◽  
Consuelo Valencia Ramirez ◽  
Claudia Restrepo ◽  
...  

Odontogenesis is a complex process, where disruption can result in dental anomalies and/or increase the risk of developing dental caries. Based on previous studies, certain dental anomalies tend to co-occur in patients, suggesting that these traits may share common genetic and etiological components. The main goal of this study was to implement a multivariate genome wide association study approach to identify genetic variants shared between correlated structural dental anomalies and dental caries. Our cohort (N = 3,579) was derived from the Pittsburgh Orofacial Clefts Study, where multiple dental traits were assessed in both the unaffected relatives of orofacial cleft (OFC) cases (n = 2,187) and unaffected controls (n = 1,392). We identified four multivariate patterns of correlated traits in this data: tooth agenesis, impaction, and rotation (AIR); enamel hypoplasia, displacement, and rotation (HDR); displacement, rotation, and mamelon (DRM); and dental caries, tooth agenesis and enamel hypoplasia (CAH). We analyzed each of these four models using genome-wide multivariate tests of association. No genome-wide statistically significant results were found, but we identified multiple suggestive association signals (P ≤ 10−5) near genes with known biological roles during tooth development, including ADAMTS9 and PRICKLE2 associated with AIR; GLIS3, WDR72, and ROR2 associated with HDR and DRM; ROBO2 associated with DRM; BMP7 associated with HDR; and ROBO1, SMAD2, and MSX2 associated with CAH. This is the first study to investigative genetic associations for multivariate patterns of correlated dental anomalies and dental caries. Further studies are needed to replicate these results in independent cohorts.


Author(s):  
Deepashri H Kambalimath

Congenital missing permanent second molar is an extremely rare condition. Non syndromic mandibular second molar agenesis associated with other anomalies has occasionally been reported in literature, but isolated sporadic cases are rarely observed. Number of interactions between genetic and environmental factors during the process of tooth development might be the causative etiology for agenesis. This report presents an isolated case of hypodontia with absence of bilateral mandibular second molar agenesis in a healthy 18 year old female patient is presented and literature review on prevalence of most missing teeth with incidence of missing second molar in various regions of the world and in various regions of Indian continent is presented. No such case has been reported in Indian literature so far.


2021 ◽  
Vol 7 (7) ◽  
pp. eabf1798
Author(s):  
A. Murashima-Suginami ◽  
H. Kiso ◽  
Y. Tokita ◽  
E. Mihara ◽  
Y. Nambu ◽  
...  

Uterine sensitization–associated gene-1 (USAG-1) deficiency leads to enhanced bone morphogenetic protein (BMP) signaling, leading to supernumerary teeth formation. Furthermore, antibodies interfering with binding of USAG-1 to BMP, but not lipoprotein receptor–related protein 5/6 (LRP5/6), accelerate tooth development. Since USAG-1 inhibits Wnt and BMP signals, the essential factors for tooth development, via direct binding to BMP and Wnt coreceptor LRP5/6, we hypothesized that USAG-1 plays key regulatory roles in suppressing tooth development. However, the involvement of USAG-1 in various types of congenital tooth agenesis remains unknown. Here, we show that blocking USAG-1 function through USAG-1 knockout or anti–USAG-1 antibody administration relieves congenital tooth agenesis caused by various genetic abnormalities in mice. Our results demonstrate that USAG-1 controls the number of teeth by inhibiting development of potential tooth germs in wild-type or mutant mice missing teeth. Anti–USAG-1 antibody administration is, therefore, a promising approach for tooth regeneration therapy.


2021 ◽  
Vol 15 (10) ◽  
pp. 2710-2711
Author(s):  
Saman Malik ◽  
Faiqa Hassan ◽  
Muhammad Farooq ◽  
Usman ul Haq ◽  
Amna Faisal ◽  
...  

Background: There are different types of teeth anomalies that effects the people of different regional populations. Aim: To determine the occurrence of dental anomalies in patients of Taxila that visit our college for routine dental procedures. Methods: The study was retrospective and was conducted on periapical intraoral radiographs of patients between the ages of 15 to 35 years, with no gender discrimination at Dental College HITEC-IMS. Results: We collected data from 450 periapical intraoral radiographs that were taken in last six months (i.e. 15th January 2021 till 15th July 2021) in dental radiology department. Conclusion: The dental anomalies that were found in the population of taxila were impacted teeth, missing teeth, rotated tooth, supernumerary teeth (mesiodens), root dilacerations, peg lateral, taurodontism and hypercementosis. Keywords: Root anomalies, dental anomalies, periapical radiograph


2021 ◽  
pp. 146531252110654
Author(s):  
Nusaybah Elsherif ◽  
Jose Rodriguez ◽  
Farooq Ahmed

Background: Hypodontia is one of the most common anomalies in dentistry. Hypodontia has a negative impact on oral health-related quality of life with patients best seen in a multidisciplinary clinic to improve treatment outcomes. Aim: To investigate the prevalence of hypodontia and its association with other dental anomalies, as well as malocclusion, and to investigate the treatment planned for patients attending the clinic and whether the type of missing teeth affected the proposed treatment. Materials and Methods: Analysis of consecutive patients attending the hypodontia clinic at a dental hospital between February and November 2020. A total of 100 patients who met the inclusion criteria were identified. Data collected included the following: age/sex; number and type of missing teeth; pre-treatment occlusion; presence of other dental anomalies; and planned treatment. Results: A total of 100 patients (55% female; age range = 7–41 years; mean age = 18 years) were included. Of the cohort, 47% had a class I skeletal relationship and participants were significantly less likely to have a class III skeletal or incisor relationship; 45% had another dental anomaly with the most common being microdontia. Space opening was the preferred treatment option for those managed by orthodontic treatment with resin-bonded bridges most likely to be used for restoration of spaces, 86%. Conclusion: Lower second premolars were the most commonly missing teeth. Participants were significantly less likely to have a class III incisor or skeletal relationship than class I or II. Space opening was the favoured approach for orthodontic treatment, particularly for maxillary lateral incisors.


1996 ◽  
Vol 33 (5) ◽  
pp. 436-439 ◽  
Author(s):  
Peter J. Anderson ◽  
Anthony L.H., Moss

The incidence of dental abnormalities in the cleft lip and palate population has been reported to be much higher than in the normal population. The role of genes in the production of a cleft lip and palate, and dental anomalies is thought to be complex, with autosomal dominant, recessive, and x-linked genes all playing a role. Noncleft parents can carry some of the cleft lip and palate genes, which produce clinically subtle manifestations in their facial skeleton. The purpose of this study was to look for evidence of increased dental anomalies in the non-cleft parents of cleft lip and palate children. The dentitions of the parents of 60 children with different types of cleft lip and palate were examined prospectively to see whether or not they exhibited features found more readily in the cleft lip and palate rather than did the normal population. Their dentitions were studied to record the following dental features: congenitally missing teeth, supernumerary teeth, or morphologic changes of the crowns of the permanent teeth. The number and position of any frenal attachments were also recorded. The results of this study did not show any differences in incidence of dental anomalies from the noncleft population. There was no evidence to support the hypothesis that congenital absence of lateral incisors is a microform of cleft lip and palate. Further, these results also failed to reveal any consistent pattern in the number and position of frenal attachments.


1970 ◽  
Vol 10 (1) ◽  
pp. 41-44 ◽  
Author(s):  
P Nayak ◽  
S Nayak

Objective: Developmental disturbances of teeth contribute to dental problems encountered in general practice. These are a group of disorders where prevention is not possible, with the exception of environmental enamel hypoplasia. Surveys done on various populations have found prevalence of dental anomalies to be 5.46%. Knowledge of common dental anomalies when available can be a useful tool for forensic dentistry. Since such epidemiological data is not available for the Jodhpur population in Rajasthan, this study was conducted to determine the prevalence of developmental dental anomalies in 500 school going children. Methodology & Result: A total of 500 school children (290 male & 210 female) of age ranging between 6 - 15 years were examined clinically for developmental anomalies of teeth. Prevalence of Peg lateral, supernumerary teeth & Talon’s cusp was 0.4%, 0.6% & 0.2% respectively while 18.8% showed fluorosis induced enamel hypoplasia. Key Words: Dental anomalies; Peg lateral; supernumerary teeth; Talon's cusp; Enamel hypoplasia. DOI: 10.3329/bjms.v10i1.7318 Bangladesh Journal of Medical Science Vol.10 No.1 Jan 2010 pp.41-44


PEDIATRICS ◽  
1969 ◽  
Vol 44 (4) ◽  
pp. 564-569
Author(s):  
Murray Feingold ◽  
Frederic Shiere ◽  
Helmi R. Fogels ◽  
David Donaldson

Three generations of a family with Rieger's syndrome are presented. Ocular and dental abnormalities constitute the major manifestations. Hypodontia, microdontia, enamel hypoplasia, missing teeth, peg-shaped teeth, and malocclusion are frequent oral findings. Hypoplasia of the maxilla causes the mandible to appear prominent, giving the patient a prognathic appearance. Aniridia, hypoplastic iris, and glaucoma are the major eye problems. The propositus also has short stature and decreased growth hormone levels which have not been reported before in this syndrome. The importance of early diagnosis is stressed in order to preserve the remaining dentition by proper dental care and to be aware of the possibility of the presence of glaucoma, which should be treated as soon as discovered.


2019 ◽  
Vol 20 (1) ◽  
Author(s):  
Morenike Oluwatoyin Folayan ◽  
Michael Alade ◽  
Abiola Adeniyi ◽  
Maha El Tantawi ◽  
Tracy L. Finlayson

Abstract Background To determine the association between developmental dental anomalies (DDA), early childhood caries (ECC) and oral hygiene status of 3–5-year-old children resident in Ile-Ife, Nigeria. Methods This was a cross-sectional study. We analyzed data for 3–5-year-olds extracted from the dataset of a household survey collected to determine the association between ECC and maternal psychosocial wellbeing in children 0–5-year-old. The outcome variables for the study were ECC and poor oral hygiene. The explanatory variable was the presence of developmental dental anomalies (supernumerary, supplemental, mesiodens, hypodontia, macrodontia, microdontia, peg-shaped lateral, dens evaginatus, dens invaginatus, talons cusp, fusion/germination, hypoplasia, hypomineralized second molar, fluorosis, amelogenesis imperfecta). The prevalence of each anomaly was determined. Poisson regression analysis was conducted to determine the association between presence of developmental dental anomalies, ECC and oral hygiene status. The model was adjusted for sex, age and socioeconomic status. Results Of the 918 children examined, 75 (8.2%) had developmental dental anomalies, 43 (4.7%) had ECC, and 38 (4.1%) had poor oral hygiene. The most prevalent developmental dental anomalies was enamel hypoplasia (3.9%). Of the 43 children with ECC, 6 (14.0%) had enamel hypoplasia and 3 (7.6%) had hypomineralized second primary molar. There was a significant association between ECC and enamel hypoplasia (p < 0.001) and a borderline association between ECC and hypomineralized second primary molars (p = 0.05). The proportion of children with poor oral hygiene (PR: 2.03; 95% CI: 0.91–4.56; p = 0.09) and ECC (PR: 2.02; 95% CI: 0.92–4.46; p = 0.08) who had developmental dental anomalies was twice that of children with good oral hygiene and without ECC respectively, although the differences did not reach statistical significance. Conclusions Enamel hypoplasia and hypomineralized second primary molars are developmental dental anomalies associated with ECC. developmental dental anomalies also increases the probability of having poor oral hygiene in the population studied.


2018 ◽  
Vol 66 (1) ◽  
pp. 106-110
Author(s):  
Stephanie Anagnostopoulos FRIEDRICH ◽  
Jonas RODRIGUES ◽  
Berenice Barbachan e SILVA

ABSTRACT Albright hereditary osteodystrophy is a disorder comprising phenotypic characteristics of genetic origin, such as short stature, obesity, and brachydactyly. It is a rare disorder and is related to pseudohypoparathyroidism. Within dentistry, it may be associated with enamel hypoplasia and late eruption. Furthermore, due to neurological problems, these patients may impose behavioural difficulties during dental appointments. The present study aims to describe the case of a patient with a possible diagnosis of Albright hereditary osteodystrophy, presenting symptoms and limitations to dental management.


Sign in / Sign up

Export Citation Format

Share Document