Diagnosis and initial management of children presenting with premature loss of primary teeth associated with a systemic condition: A scoping review and development of clinical aid.

BACKGROUND: Premature loss of primary teeth (PLPT) can be a rare presentation of systemic medical conditions. Premature loss of primary teeth may present a diagnostic dilemma to paediatric dentists. AIMS: To identify systemic conditions associated with PLPT and develop a clinical aid. DESIGN: OVID Medline, Embase and Web of Science were searched up to March 2023. Citation searching of review publications occurred. Exclusion occurred for conference abstracts, absence of PLPT and absence of English-language full text. RESULTS: Seven hundred and ninety-one publications were identified via databases and 476 by citation searching of review articles. Removal of 390 duplicates occurred. Following the exclusion of 466 records on abstract review, 411 publications were sought for retrieval, of which 142 met inclusion criteria. Thirty-one systemic conditions were identified. For 19 conditions, only one publication was identified. The majority of publications, 91% (n = 129), were case reports or series. Most publications, 44% (n = 62), were related to hypophosphatasia, and 25% (n = 35) were related to Papillon-Lefèvre. Diagnostic features were synthesised, and a clinical aid was produced by an iterative consensus approach. CONCLUSIONS: A diverse range of systemic diseases are associated with PLPT. Evidence quality, however, is low, with most diseases having a low number of supporting cases. This clinical aid supports paediatric dentists in differential diagnosis and onward referral.

British Society of Paediatric Dentistry: A policy document on dental neglect in children.

The British Society of Paediatric Dentistry's (BSPD) first policy document on dental neglect was published online in 2009. It proposed a new original definition of dental neglect, discussed the identification of dental neglect and recommended adopting a tiered response, with three stages of intervention according to level of concern. Furthermore, it detailed how the dental team should both contribute to the child protection process and implement wider measures to safeguard and promote children's welfare. Since 2009, these concepts have been widely adopted in the UK and beyond. Furthermore, there have been significant advances in both research and practice. Policy documents produced by the BSPD represent a majority view, based on the consideration of currently available evidence, and are tailored to a UK working environment. Although this updated document's recommendations remain broadly unchanged, this version reflects the professions' progress in understanding dental neglect and minor updates to terminology and, following a consultation process, has been amended to address the needs of two main audiences-dental professionals and nondental health and social care professionals-in order to enhance interdisciplinary working.

Heterozygous COL17A1 variants are a frequent cause of amelogenesis imperfecta.

BACKGROUND: Collagen XVII is most typically associated with human disease when biallelic COL17A1 variants (>230) cause junctional epidermolysis bullosa (JEB), a rare, genetically heterogeneous, mucocutaneous blistering disease with amelogenesis imperfecta (AI), a developmental enamel defect. Despite recognition that heterozygous carriers in JEB families can have AI, and that heterozygous COL17A1 variants also cause dominant corneal epithelial recurrent erosion dystrophy (ERED), the importance of heterozygous COL17A1 variants causing dominant non-syndromic AI is not widely recognised. METHODS: Probands from an AI cohort were screened by single molecule molecular inversion probes or targeted hybridisation capture (both a custom panel and whole exome sequencing) for COL17A1 variants. Patient phenotypes were assessed by clinical examination and analyses of affected teeth. RESULTS: Nineteen unrelated probands with isolated AI (no co-segregating features) had 17 heterozygous, potentially pathogenic COL17A1 variants, including missense, premature termination codons, frameshift and splice site variants in both the endo-domains and the ecto-domains of the protein. The AI phenotype was consistent with enamel of near normal thickness and variable focal hypoplasia with surface irregularities including pitting. CONCLUSION: These results indicate that COL17A1 variants are a frequent cause of dominantly inherited non-syndromic AI. Comparison of variants implicated in AI and JEB identifies similarities in type and distribution, with five identified in both conditions, one of which may also cause ERED. Increased availability of genetic testing means that more individuals will receive reports of heterozygous COL17A1 variants. We propose that patients with isolated AI or ERED, due to COL17A1 variants, should be considered as potential carriers for JEB and counselled accordingly, reflecting the importance of multidisciplinary care.

Flexibility and access to dental postgraduate speciality training.

Increasingly, there is a clear emphasis on the need for improved access to flexible training. This reflects changing societal attitudes and expectations towards work-life balance and increasing demand for flexible working. The recently published Advancing dental care review: final report (2021) reflects these same demands within dental postgraduate training. Increasing flexible training options has the potential for far-reaching beneficial effects for dental speciality training. There are barriers to accessing speciality training for specific applicant groups and this risks potentially excluding excellent-quality candidates from pursuing speciality training. There is opportunity in utilising and developing flexible training options to support widening access to dental speciality training. Recruitment and working pattern arrangements should reflect these aspirations, ensuring that the best trainees are successful irrespective of their personal circumstances.

Looked after children: an overview for the dental team.

Looked after children (LAC) are children and young people (CYP) who have been under the continuous care of the local authority for more than 24 hours. Reasons for becoming looked after include concerns about abuse or neglect, breakdown in family functioning, or absence of a suitable caregiver (for example bereavement, parental illness, or separation, such as for unaccompanied asylum-seeking children). Many LAC live with foster families, extended family, or in residential care homes, but a CYP can be legally 'looked after' and still be living with their original or birth family, or living independently with support. Regardless of the circumstances, the local authority has responsibility for meeting the needs of the CYP, including dental care, usually via a named social worker.The evidence available suggests that LAC are at higher risk of dental caries and pain. In 2021, Public Health England reported on inequalities in oral health in England and although evidence was limited, found LAC to have poorer oral health and access to care. It is important that LAC are considered for enhanced prevention and reviewed regularly to enable appropriate provision of dental care. Understanding who can consent for dental treatment is essential.

Public and dental professionals' use of social media to discuss amelogenesis imperfecta.

BACKGROUND: Amelogenesis imperfecta (AI) is an inherited disorder of enamel development that is challenging to treat and often associated with negative patient and parental outcomes. Social media provides a valuable perspective on patients' and dental professionals' experience of AI and dental care. AIM: To explore how the public and dental professionals use social media to discuss AI. DESIGN: A cross-sectional study involving a systemic search of eight social media platforms using the search term 'amelogenesis imperfecta'. Relevant posts were selected using predefined eligibility criteria. Word content of eligible posts was qualitatively analysed using a thematic framework approach. RESULTS: A total of 555 posts were identified, of which 144 were eligible for analysis. For dental professionals, the posts included case reports and seeking and sharing of information. For the public, the posts were related to individuals' experience of AI, dental treatment and outcome of treatment. CONCLUSIONS: Posts from individuals affected by AI suggest a need for better distribution of reliable information and greater support. Case reports indicate that dental professionals find it challenging to recognise AI and determine appropriate treatment options. Social media could potentially be used to inform and support people with AI and allow dental professionals to share information and learning with peers.

The dental management of children with congenital heart disease following the publication of Paediatric Congenital Heart Disease Standards and Specifications.

The Paediatric Congenital Heart Disease Standards and Specifications (PCHDSS) were published in May 2016 by NHS England. The standards describe in detail the cardiac care patients should expect in England. They are also the first cardiology standards to include an oral health section. The dental standards outline what oral health care patients should receive from both cardiology and dental healthcare professionals, with immediate effect. Children with congenital heart disease (CHD) are at increased risk of infective endocarditis and often have poorer oral health compared to healthy children. Children with cardiac disease can be complex to manage appropriately due to their increased dental anxiety and reduced access to dental care. The PCHDSS dental section highlights the importance of collaborative working between cardiology, primary care and paediatric dentistry. This should ensure preventive advice is delivered regularly, oral disease diagnosed early and patients managed or referred appropriately. This article will summarise CHD, the PCHDSS, its implications and discuss the oral health of children with a cardiac defect. The importance of treatment planning and dental management for this high risk group, in addition to informing readers when to refer to specialist care will also be described.

The dental health of children subject to a child protection plan.

BACKGROUND: In the United Kingdom, child maltreatment is an area of increased awareness and concern. AIM: To compare the dental health of children subject to child protection plans with controls. DESIGN: Children had to be aged between two and 11 years, medically healthy, and subject either to a child protection plan or attending the paediatric outpatient orthopaedic or general surgery clinics (control group). All children had a standardized oral examination. RESULTS: Seventy-nine children were examined in each group. Children with child protection plans had statistically higher levels of primary tooth decay than controls (mean dmft 3.82 and 2.03, Mann-Whitney U test P = 0.002). After adjusting for socioeconomic status, the incidence rate ratios for the occurrence of dental caries in the primary dentition in children with a child protection plan was 1.76 (95% CI: 1.44-2.15) relative to the controls. There was no statistical difference in the levels of permanent tooth decay between the study and control groups (mean DMFT 0.71 and 0.30, respectively). The care index was significantly lower (P = 0.008, Mann-Whitney U test) in the study group (1.69%) compared to the control group (6.02%). CONCLUSIONS: Children subject to child protection plans had significantly higher levels of dental caries in the primary dentition.

An investigation into dental anxiety amongst paediatric cardiology patients.

BACKGROUND: There is evidence that children with cardiac conditions have high levels of untreated dental disease. One possible explanation is that they are more dentally anxious as a result of increased exposure to medical interventions. Therefore, the primary aim of this study was to compare the level of dental anxiety between paediatric cardiology patients and healthy children. METHODS: The study group comprised 54 children (mean age 12.2 years) who attended the outpatient paediatric cardiology clinic in tertiary care. The control group (n = 53, mean age 12.38 years) was recruited from consultant-led new-patient orthodontic clinics. Child dental anxiety was measured using the Modified Child Dental Anxiety Scale (faces version). The parents completed the Modified Dental Anxiety Scale along with a questionnaire regarding their child's medical and dental histories. RESULTS: The mean level of dental anxiety was significantly higher in the study group (P < 0.05). Analysis of covariance indicated that overnight hospital admission history may have influenced the strength of this relationship. CONCLUSIONS: Paediatric cardiology patients had significantly increased levels of dental anxiety. It is likely that aspects of their medical history, notably overnight hospital admissions, are contributory factors.

Current concepts in hypophosphatasia: case report and literature review.

BACKGROUND. Hypophosphatasia (HP) is characterized by defective mineralization of bone and teeth because of deficient alkaline phosphatase activity. There are generally six recognized clinical forms, of which the most severe is often lethal prenatally or early in life. In milder forms, such as odontohypophosphatasia (OHP), premature exfoliation of primary teeth may be the only clinical manifestation. CASE REPORT. A 20-month-old girl was referred to the Specialist Paediatric Salaried Dental Service within the Harrogate and District NHS Foundation Trust with mobility of tooth numbers 71 and 81. Clinical examination revealed grade III mobile 71 and 81, with minimal gingival inflammation and plaque deposits. There were no other dental findings and no significant medical history. Tooth numbers 71 and 81 exfoliated prematurely with no evidence of root resorption, shortly after presentation. Haematological and urinary investigations showed no abnormalities. Histological examination showed a complete absence of cementum. A diagnosis of OHP was made. After 10 months of dental follow-up, no further teeth have increased mobility. CONCLUSION. Odontohypophosphatasia should be included as a differential diagnosis in children presenting with early loss of primary teeth. The dentist may be the first health care professional to whom the patient presents.

The prevalence of molar incisor hypomineralisation in Northern England and its relationship to socioeconomic status and water fluoridation.

BACKGROUND: Molar incisor hypomineralisation (MIH) is a condition which has significant implications for patients and service provision. AIMS: The aim of this survey was to determine the prevalence of MIH in 12-year olds in Northern England and to consider the relationship with socioeconomic status and background water fluoridation. DESIGN: Twelve-year-old children were examined for the presence of MIH. Participating dentists were trained and calibrated in the use of the modified Developmental Defects of Enamel index. Children were examined at school under direct vision with the aid of a dental mirror. A diagnosis of MIH was attributed to a child if they had a demarcated defect in one or more of their first permanent molars. RESULTS: Of 4795 children that were selected, 3233 (67.4%) were examined. Overall prevalence of MIH was 15.9% (14.5-17.1%). There was an association between prevalence of MIH and deprivation quintiles with a positive correlation in the first 4 quintiles (P < 0.05). There was no difference in prevalence between fluoridated Newcastle and other areas. CONCLUSION: Prevalence of MIH is equivalent to other European populations. Prevalence was related to socioeconomic status but not to background water fluoridation.

Understanding child neglect. Current perspectives in dentistry.

The aim of this paper is to update the reader on the subject of dental neglect in children. Recent national guidelines produced by the National Institute for Health and Clinical Excellence and the British Society of Paediatric Dentistry have raised the profile of this condition by providing specific, evidence-based recommendations for recognition and response to dental neglect. Dental neglect may be a sign of general neglect or may be considered maltreatment in itself. The specific dental and non-dental features are described and actions secondary to a diagnosis of dental neglect are discussed. Three types of intervention are described: preventive dental team management, preventive multi-agency management, and referral to child protection services. With increasing awareness of this condition and through access to the expertise of other specialist agencies in this field, the primary care practitioner can play a key role in safeguarding the welfare of children.

The oral health of children considered very high risk for infective endocarditis.

BACKGROUND: Children with previous experience of infective endocarditis or with prosthetic heart valve are considered at very high risk for infective endocarditis. AIM: The aim of this study was to compare the dental health of a group of these children with a group of healthy controls and to determine parental awareness of the importance of good oral health. DESIGN: Oral examination was carried out in 28 children with previous infective endocarditis or a prosthetic heart valve to assess oral health. Findings were compared to a healthy control group of 28. Questionnaires were distributed to the parents to assess awareness of oral health. RESULTS: There was no significant difference in DMFT scores of study and control group (2.43 +/- 3.72 and 1.36 +/- 2.5 respectively) or in DMFT scores of study and control group (1.5 +/- 1.73 and 1.15 +/- 1.42 respectively), 36% of the study group had untreated caries. Parental knowledge of the link between oral health and infective endocarditis was excellent. CONCLUSIONS: There were no significant differences between the oral health of cardiac children and healthy children although the dmft and DMFT scores of the study group were high. Of concern was the proportion of children with untreated caries in spite of good dental awareness and attendance.

British Society of Paediatric Dentistry: a policy document on dental neglect in children.

International Journal of Paediatric Dentistry 2009This policy document was prepared by J.C. Harris, R.C. Balmer, and P.D. Sidebotham on behalf of the British Society of Paediatric Dentistry (BSPD). Policy documents produced by the BSPD represent a majority view, based on consideration of currently available evidence. They are produced to provide guidance with the clear intention that the policy be regularly reviewed and updated to take account of changing views and developments.

Dental health and treatment in a group of children with congenital heart disease.

PURPOSES: The purposes of this study were to: (1) determine the prevalence of dental caries and developmental enamel defects in children with congenital heart (CHD) disease; and (2) evaluate previous dental treatment. METHODS: One hundred and seventy-six 2- to 16-year-old children were examined during their outpatient cardiology appointment. The study group consisted of 86 CHD children. Sixty healthy children formed the control group. RESULTS: Mean dmft and DMFT scores were 1.57 (+/- 3.01 SD) and 0.77 (+/- 1.42) for the study group and 1.81 (+/- 3.64) and 0.38 (+/- 1.16) for the control group, respectively. Eight out of 86 CHD children and 5 out of 60 healthy children had enamel defects on their permanent teeth. No significant differences were demonstrated between the 2 groups. The care index for primary teeth was 10% for the study group and 3% for the control group. In permanent dentition, the care index was 30% and 16%, respectively CONCLUSIONS: Children with and without congenital heart disease had similar levels of dental disease. The care index for primary teeth was higher in CHD children, although the overall level was very low.

Enamel defects and ectopic eruption in a child with Usher syndrome and a cochlear implant.

BACKGROUND: Usher syndrome is a genetic disorder consisting of progressive loss of vision and hearing. CASE REPORT: The paper describes an 8-year-old girl with Usher syndrome type I who presented with generalized defects of the permanent dentition and ectopic eruption of the right maxillary first permanent molar. A cochlear implant had been fitted for her hearing loss, and the report reviews the implications of this device for dental treatment. The impacted first permanent molar was encouraged to erupt into the correct position by shaving the distal surface of the second primary molar. CONCLUSION: This is the first report to describe in detail an association between Usher syndrome and enamel defects.

The role for 'reminders' in dental traumatology: 4. The use of a computer database for recording dento-alveolar trauma in comparison to unstructured and structured paper-based methods.

The aims of this study were to investigate the effectiveness of a computer database (CD) developed for this study, a plain paper unstructured history (USH) and structured histories (SH) for the recording of important prognostic factors for simulated dento-alveolar trauma. Twelve vocational trainees, seven postgraduates in paediatric dentistry and 24 general dental practioners were randomly assigned to using USH, SH or CD. Each dentist visited a series of simulated trauma cases (with models, photos, radiographs and actors) and was asked to record important prognostic factors for each injury and make a diagnosis. There were a total of 243 dentist contacts with the trauma stations. The average percentage of important prognostic factors recorded per station was: USH 53%, SH 75.3% and CD 58.6%. SH was significantly better than the other two methods (P < 0.001, anova). Interestingly, those general dental practitioners (GDPs) who qualified prior to 1990 were significantly poorer at recording important prognostic information using CD. This effect was not obvious when using USH and SH. It was also seen that USH and SH were significantly better at helping clinicians reach a correct diagnosis as compared with CD (P < 0.001, chi-squared). A paper-based SH was the most effective method for collecting essential prognostic information for simulated trauma cases used in this study. At present, the introduction of our CD for recording of trauma is not justified without significant modification.

Enamel defects and Lyonization in focal dermal hypoplasia.

We report a pattern of enamel hypoplasia in focal dermal hypoplasia similar to that found in females with X-linked amelogenesis imperfecta. Three cases of focal dermal hypoplasia are described, with specific focus on the oral and dental features. In these cases the teeth all had vertical grooving with notching of the incisal or cuspal tips. Also recorded were blunt roots of taurodont form with open apices and missing teeth in 1 case. Oral papillomas were present in 2 cases. The pattern of enamel defects is attributed to Lyonization, which is consistent with the pattern of skin and bone lesions typically seen in focal dermal hypoplasia. This supports the proposal that focal dermal hypoplasia is X-linked. The authors conclude that the pattern of dental defects in focal dermal hypoplasia is consistent with Lyonization.

The experiences with oral health and dental prevention of children with congenital heart disease.

OBJECTIVE: To examine the degree to which children, considered to be at risk from infective endocarditis, had received professional education and preventive procedures in regard to dental health, and to evaluate the knowledge of their parents of the link between oral health and infective endocarditis. MATERIALS AND METHODS: Questionnaires were distributed to the families of 38 children under the care of paediatric cardiology. A short dental examination was carried out. Parents were asked if they knew why oral health was of particular importance in their child. RESULTS: Of the children, 58% demonstrated evidence of previous or current dental disease, with 24% having had at least one filling, 13% with one or more teeth showing deficiency of enamel, and 39% with untreated dental caries. Only 79% of the children were registered with a dentist. According to Chi squared test, there was no difference in the dental health of registered and non registered children. Of the study group, 29% had received instruction in oral hygiene, 42% had received dietary advice, 13% had received advice regarding fluoride supplementation or had had fluoride professionally applied, and 8% had had fissure sealants. These percentages remained relatively low even if only registered children, or only registered children with previous or current dental disease, were considered. Only 64% of parents were aware of the link between the oral health of their children and infective endocarditis. Parents of children who were registered were more likely to be aware of this link than parents of children who were not registered. CONCLUSIONS: In spite of being registered with general dental practitioners, few children with congenital heart disease had received basic education in dental hygiene. Even children known to have had dental disease and, therefore, considered to be more vulnerable, were overlooked.