Oral health-related quality of life before and after crown therapy in young patients with amelogenesis imperfecta.

BACKGROUND: Amelogenesis imperfecta (AI) is a rare, genetically determined defect in enamel mineralization associated with poor esthetics and dental sensitivity. Because the condition is associated with negative social outcomes, this study evaluated oral health-related quality of life (OHRQoL), dental fear, and dental beliefs before and after early prosthetic crown therapy for AI during adolescence. METHODS: The study included 69 patients with AI, aged 6-25 yr: 33 males and 36 females (mean age 14.5 ± 4.3); healthy controls (n = 80), patients with cleft lip and palate (CLP; n = 30), and patients with molar incisor hypomineralization (MIH; n = 39). All matched in age and gender, and all but the CLP group insocioeconomic area. Patients completed three questionnaires measuring OHRQoL (OHIP-14), dental fear (CFSS-DS), and dental beliefs (DBS-R). Twenty-six patients with severe AI between ages 9 and 22 yr received crown therapy and completed the questionnaires twice: before and after therapy. RESULTS: OHIP-14 scores were significantly higher among patients with AI (7.0 ± 6.7), MIH (6.8 ± 7.6) and CLP (13.6 ± 12.1) than healthy controls (1.4 ± 2.4) (p < 0.001). After crown therapy, quality of life problems in the 26 patients with severe AI decreased significantly, from 7.8 ± 6.1 to 3.0 ± 4.8 (p < 0.001). Early prosthetic therapy did not increase dental fear or negative attitudes toward dental treatment. CONCLUSIONS: OHRQoL increased after early crown therapy in patients with severe AI. Therapy did not increase dental fear or negative attitudes toward dental treatment.

How do children with amelogenesis imperfecta feel about their teeth?

BACKGROUND: Amelogenesis imperfecta (AI) is an inherited dental condition affecting enamel, which can result in significant tooth discolouration and enamel breakdown, requiring lifelong dental care. The possible impact of this condition on children and adolescents from their perspectives is not fully understood. AIMS: The aim of the study was to explore the impact of AI on children and adolescents through in-depth interviewing. The information derived from this was then used to construct a questionnaire to distribute to a larger cohort of AI patients. DESIGN: This research involved semistructured in-depth interviews with seven AI patients, and common themes and concepts were then identified using framework analysis. A questionnaire was developed based on the themes and subthemes identified, and completed by 40 AI patients at various stages of treatment. RESULTS: Children and adolescents with AI exhibited concerns regarding aesthetics and function. Patients also expressed a high level of concern regarding comments by other people and self-consciousness associated with this. A small number of AI patients highlighted the effect of their dental treatment and health on their personal life. CONCLUSION: The results indicate that there are marked impacts on children and adolescents as a result of AI, including aesthetics, function, and psychosocial.

Impact of moderate and severe hypodontia and amelogenesis imperfecta on quality of life and self-esteem of adult patients.

OBJECTIVES: The objective of this study was to investigate the impact of moderate and severe hypodontia and amelogenesis imperfecta on the quality of life and self-esteem of affected adult patients. METHODS: Forty one adult patients (aged 18-45 years) with clinical and radiological diagnoses of moderate to severe hypodontia and twenty seven patients diagnosed with amelogenesis imperfecta were age and gender matched with a control group of patients attending for routine dental care. Subjects completed the Oral Health Impact Profile [OHIP-49] and Rosenberg Self Esteem Scale. A paired t-test was used to analyse data; the test alpha level was set at P ≤ 0.05. RESULTS: The results for hypodontia patients were significantly different from controls in six out of the seven OHIP-49 domains, the exception being the Handicap domain. Total scores were also significantly different between the two groups (P=0.003). Self-esteem was not significantly different between the two groups (P=0.98). For amelogenesis imperfecta patients the results were significantly different from control patients in four out of the seven domains of the OHIP-49 and also in the total scores (P=0.01). When self-esteem was investigated there was no significant differences between the two groups (P=0.92). CONCLUSIONS: Moderate to severe hypodontia and amelogenesis imperfecta have marked negative impacts on the Oral Health Related quality of life of this patient population relative to controls. However, self-esteem was not significantly affected.

Seeking children's perspectives in the management of visible enamel defects.

AIM: To undertake a child-centred evaluation of treatment provision for visible enamel defects. DESIGN: Postal questionnaires, developed with children, were sent to 88 patients, aged 7-16 years, with visible enamel defects of permanent incisors and who had received microabrasion, with/without additional composite restoration at Sheffield Dental Hospital, UK. The questionnaires sought children's perceptions about their teeth before and after the intervention, as well as their evaluation of how they had been treated. Anonymised responses were graded using a 10 cm visual analogue scale (VAS) where a score of 10 indicated the most negative response, and zero the most positive response. RESULTS: Sixty three questionnaires were returned (72% response). Prior to treatment, children reported high levels of worry (VAS=6.8), embarrassment (VAS=6.9) and a perception that their teeth looked yellow and discoloured (VAS=7.3). Following treatment, children thought their teeth looked much better (VAS=1.6), felt happier (VAS=2.2) and more confident (VAS=1.6). They also felt very positive about their clinical experiences, rating the staff as extremely friendly and kind (VAS=0.4) and reporting that procedures were clearly explained (VAS=0.6). CONCLUSIONS: Simple non-invasive dental treatment can have a positive effect on appearance-related satisfaction. The use of child-centred approaches offers an invaluable insight into patient perspectives.

Amelogenesis imperfecta - a systematic literature review of associated dental and oro-facial abnormalities and their impact on patients.

OBJECTIVE: Amelogenesis imperfecta (AI) is a disease primarily affecting amelogenesis, but other aberrations have been reported. The purposes of this review were: (1) to identify other anomalies associated with AI, and (2) to describe the impact of the disease and its associated conditions on the oral health-related quality of life of patients, and the economic consequences. MATERIAL AND METHODS: A literature search was conducted in the following databases: PubMed, EMBASE, Bibliotek.dk, The Cochrane Library, Web of Science, and OMIM, supplemented by a search for selected authors. Based on titles and abstracts, 137 papers were identified. RESULTS: Most articles were case reports or case series with few cases. Aberrations were reported in the eruption process, in the morphology of the crown, in the pulp-dentine organ, and in the number of teeth. Gingival conditions and oral hygiene were usually reported to be poor, and calculus was a common finding. Open bite was the most commonly reported malocclusion. A negative impact on patients' oral health-related quality of life was described, but information was scarce. No information was found on the economic impact. CONCLUSIONS: A number of aberrations associated with AI have been reported, but not sufficiently systematic to allow for a secondary analysis and synthesis of the findings. The impact on patients in terms of reduced quality of life and economic burden needs to be studied.

Amelogenesis imperfecta: report of a successful transitional treatment in the mixed dentition.

The term amelogenesis imperfecta is applied to a clinically heterogeneous group of hereditary disorders that interfere with the normal development of dental enamel. These disorders cause a deficiency in the enamel's quantity and/or the quality that may result in poor dental esthetics. The purpose of this paper was to describe a case of hypoplastic amelogenesis imperfecta in an 8-year-old girl whose dissatisfaction with the appearance of her teeth led to impaired social functioning. Since the patient was in the mixed dentition stage, a temporary treatment aiming to improve dental esthetics, preserve oral function, and allow for the recovery of the patient's self-confidence was performed by a multidisciplinary team.

Oral management of a child with mixed dentition affected by amelogenesis imperfecta.

Amelogenesis Imperfecta (AI) is a hereditary developmental disorder affecting deposition, calcification or maturation of dental enamel in both the primary and permanent dentitions. Patients usually present tooth sensitivity as well as problems in chewing function and esthetics. In addition, dissatisfaction with the teeth appearance is often found, affecting the patient's social life. Oral rehabilitation of children with primary or mixed dentition is complex, since no definitive treatment can be done during periods of growth and until the end of eruption of the permanent dentition is accomplished. This clinical report describes the oral management of a 7-year-old girl with mixed dentition affected by AI. The patient showed accentuated teeth wear and root resorption, decreased occlusal vertical dimension, open bite and alteration in the sequence of eruption of permanent teeth. Most of the teeth were restored with resin modified glass ionomer cement. This approach markedly decreased the patient's dental hypersensitivity and improved functional chewing and esthetics. Positive psychological influence of the treatment on this patient was also observed.

A retrospective study of the prosthodontic management of patients with amelogenesis imperfecta.

PURPOSE: This article reports on a retrospective study on patients with a diagnosis of amelogenesis imperfecta and on their prosthodontic management, oral health status, and attitudes toward their condition. MATERIALS AND METHODS: The study comprised 15 patients and consisted of a clinical evaluation and questionnaire. Clinical examination included records of types of restorations and cements used, rating of restoration quality, as well as complications and periodontal variables. RESULTS: The 15 patients had a total of 213 prosthetic restorations. The median age of the restorations was 60 months. Following the California Dental Association's system, all restorations were rated as acceptable to excellent, with one exception. During the follow-up period, four (2%) restorations had been recemented and 16 (8%) restorations had been redone, five (2%) because of porcelain fractures and 11 (5%) because of caries; two (1%) endodontic treatments were performed after prosthetic restoration. Plaque and Bleeding Indices were 28% and 21%, respectively, while pocket depths of more than 3 mm were found at 7% of all tooth surfaces. All patients judged their condition as having affected them negatively. However, after prosthodontic rehabilitation, patients experienced an improvement in self-esteem. CONCLUSION: Patients with severe clinical manifestations of amelogenesis imperfecta obtained extensive prosthodontic treatment at an early age. The restorations had in general performed well, and all patients were affected positively as a result.

The psychosocial impact of developmental dental defects in people with hereditary amelogenesis imperfecta.

BACKGROUND: Amelogenesis imperfecta (AI) is a hereditary dental condition with poor esthetics and dental sensitivity that frequently requires extensive dental treatment. The authors hypothesized that AI is associated with a negative psychosocial outcome. METHODS: Family members with and without AI completed a questionnaire including demographic and dental history questions, as well as a number of psychometric scales. The authors investigated the effects of AI status (with versus without), sex and age on each of the psychosocial outcomes using a generalized linear model. RESULTS: Subjects with AI (n = 30) had higher levels of social avoidance and distress, as well as higher levels of dysfunction, discomfort and disability attributable to their oral condition compared with subjects without AI (n = 29). The relationship of AI status to fear of negative evaluation, mastery and self-esteem was age-dependent. Younger subjects with AI tended to have higher fear of negative evaluation scores, while older subjects without AI tended to have higher fear of negative evaluation scores. Additionally, subjects without AI showed a definite decrease in mastery and self-esteem scores with age, while subjects with AI tended to show an increase in mastery and self-esteem scores with age. CONCLUSIONS: These results indicate that having AI has a marked impact on the psychosocial health of affected people comparable with the impact of systemic health conditions, especially at younger ages. CLINICAL IMPLICATIONS: Dental coverage for AI traditionally is excluded by third-party payers as being solely for esthetic reasons. The authors' study shows that AI has marked psychosocial effects, which suggests that dental treatment could be medically necessary and has far-reaching implications for the affected person's overall health.