Prosthetic rehabilitation with fixed prosthesis of a 5-year-old child with Hypohidrotic Ectodermal Dysplasia and Oligodontia: a case report.

BACKGROUND: Ectodermal dysplasia is a rare genetic disorder that affects ectodermally derived structures, including teeth, nails, hair, and sweat glands. Hypohidrotic ectodermal dysplasia is the most common type, with oligodontia being the most striking dental feature. Prosthetic rehabilitation in children with ectodermal dysplasia is an important step toward improving their overall quality of life. The fixed prosthesis has the advantages of being more stable in the mouth with good child compliance and a good aesthetic outcome. CASE PRESENTATION: Our patient was a 5-year-old Middle Eastern boy with oligodontia caused by ectodermal dysplasia. He was managed by fabrication of an upper functional space maintainer and a lower fixed partial denture to restore occlusion, masticatory function, aesthetics, and overall quality of life. CONCLUSIONS: The use of the fixed prosthesis in children is a new and evolving treatment modality that resolves many of the issues caused by removable prostheses. It accommodates jaw growth in the mandible, reduces the need to remake the prosthesis, and has an overall better aesthetic outcome.

Experiences of daily life and oral rehabilitation in oligodontia - a qualitative study.

OBJECTIVE: Quantitative research indicate increased anxiety and poorer mental health related quality of life (QoL) in individuals with oligodontia (congenital absence of six or more teeth). The aim of this qualitative study was to complement and explore the individuals' experiences of life and oral rehabilitation, hopefully improving the care for these patients. MATERIAL AND METHODS: Twelve participants (6 females, 6 males, aged 21-48) with oligodontia and experiences of comprehensive dental treatments, consented to participate in a semi-structured interview. The questions in the interview guide were based on previous research and clinical experience and included both open (i.e. how is your life?) and specific questions (i.e. do health care personnel know enough about your condition?). The interview transcripts were coded and analysed using a phenomenological method of analysis. The Regional Ethics Committee approved the study. RESULTS: The following themes grew out of the data; 'feeling of being different', 'the burden of treatment', 'shared decision-making', 'treatment increases self-esteem' and 'use of coping strategies'. Psychological distress and reduced QoL seemed to be related to negative aspects of the themes, which covered unacceptable aesthetics, reduced orofacial function, the long-term process of oral rehabilitation, and negative experiences with healthcare services. The informants used problem focused and emotionally focused strategies to meet these challenges. Finalizing oral rehabilitation and shared decision making were positive aspects of the themes. CONCLUSION: All themes expressed by the participants were of importance for experienced QoL and psychological distress, and should be acknowledged by health care personnel when planning and performing treatment.

Rehabilitation of ectodermal dysplasia patients presenting with hypodontia: outcomes of implant rehabilitation part 1.

PURPOSE: This study has evaluated the pre and post perceptions of patients with ectodermal dysplasia (ED) who have been referred to Westmead Centre for Oral Health for treatment with dental implants. METHODS: Six patients with ED and hypodontia were treated with new maxillary complete dental prostheses or fixed implant prostheses, and a mandibular fixed dental prosthesis with implants. Patient perceptions were recorded with a 10cm visual analogue scale (VAS). All patients were asked to draw a line on the VAS which best described their feelings regarding aesthetics, chewing, diet and speech. RESULTS: Improvement in aesthetics was reported for all patients with change scores ranging from +9 to +4 points. Similarly, all patients reported an improvement in chewing ability with +6 point changes in 2 patients, and +8, +3, +7 and +1 in the other 4 patients. Five out of 6 patients reported less dietary restriction with 3 patients each indicating a change of +6 points, +4 points, +3 points, and 2 patients reporting +1 point change. Speech improved by +1 point for 3 patients, whilst 1 patient improved by +6 points, a further 2 patients scored no change. CONCLUSIONS: Patients with ED treated with dental implants reported encouraging outcomes post treatment after prostheses were fitted. Follow-up from 1.6 to 6.8 years has confirmed these improvements.

Does dental agenesis have an impact on OHRQoL of children, adolescents and young adults? A systematic review.

Dental agenesis (DA) is defined as the congenital absence of teeth and is considered as the most common dental anomaly. It may cause speech and masticatory dysfunctions as well as esthetic problems. Its impact on oral health-related quality of life (OHRQoL) is not fully understood. The aim of the study was to assess whether DA affects OHRQoL of children, adolescents and young adults. A broad search was done on databases (Pubmed, Scopus, Web of Science and Virtual Health Library) using Medical Subject Headings (MeSH) and free terms. Eligibility criteria for article selection were predetermined and were classified according to quality assessment and risk of bias. The electronic search produced 178 titles and abstracts. After excluding duplicate abstracts and applying the eligibility criteria, three articles were assessed for the final qualitative synthesis. The three articles were classified as moderate quality and present risk of bias. No articles were found that had evaluated children and young adults. From the three articles that were selected, only one was found to have a greater impact in the adolescent agenesis group with statistical differences in all domains. There is insufficient evidence available to conclude if DA affects OHRQoL of children, adolescents and young adults.

Associations between ectodermal dysplasia, psychological distress and quality of life in a group of adults with oligodontia.

OBJECTIVE: The aim of this study was to assess demographics, self-reported signs of ectodermal dysplasia (problems with hair, nails, skin and sweat glands), present teeth, previous dental treatment, psychological distress and QoL in individuals with oligodontia, and to explore the associations between these factors. We also aimed to compare the level of psychological distress and QoL between the study group and normative samples. METHODS: Forty-seven individuals with oligodontia registered at a resource centre in Norway were included in the study. The participants completed self-administered questionnaires on demographics, ED signs, dental treatments, psychological distress and QoL (overall, health-related- and oral health-related QoL). Relevant statistics (independent t-test, correlation analysis and hierarchical multiple regressions) were used. RESULTS: Thirty-five participants reported ED signs. Forty-one participants had tooth replacements (nine had removable dentures). Sixteen had ≤10 present teeth, 13 perceived dry mouth and seven were unemployed. Persons with ≤10 present teeth had higher anxiety- and depression-scores than those having >10 present teeth. Unemployment, dry mouth and removable dentures indicated poor health-related- and oral health related QoL. Compared to a normative sample; the study group had significantly poorer mental health (MH)-related QoL, mean (SD); (51.1(8.2) versus 46.8(9.3), p < .001) and more anxiety, mean (SD); (4.5(3.1) versus 6.7(3.6), p < .001). ED signs and treatments were most important for psychological distress and MH related QoL (MCS), whereas demographic parameters were most important for the other QoL measures. CONCLUSIONS: The psychological burden of oligodontia is significant, emphasizing the importance of a holistic approach by caregivers.

Effect of malocclusion severity on oral health-related quality of life and food intake ability in a Korean population.

INTRODUCTION: The aim of this study was to evaluate the effect of malocclusion severity on oral health-related quality of life and food intake ability in adult patients, controlling for sex, age, and the type of dental clinic visited. METHODS: The sample consisted of 472 Korean patients (156 male, 316 female) with a mean age of 21.1 (SD, 8.6) years in a dental hospital and a private clinic. The correlations between the Korean version of the Oral Health Impact Profile-14 (OHIP-14K), subjective food intake ability (FIA) for 5 key foods, and Index of Orthodontic Treatment Need-Dental Health Component (IOTN-DHC) were investigated. RESULTS: The mean IOTN-DHC and OHIP-14K scores were significantly higher for the dental hospital patients than for the private clinic patients (IOTN-DHC, P <0.001; OHIP-14K, P <0.05). Malocclusion severity was significantly higher in male than in female subjects (P <0.001). Older patients perceived their oral health-related quality of life more negatively than did the teens (P <0.001). As the severity of the malocclusion increased, oral health-related quality of life and masticatory function worsened (OHIP-14K, P <0.001; FIA, P <0.05). CONCLUSIONS: As the severity of the malocclusion and the age of the patients increased, oral health-related quality of life and masticatory function relatively deteriorated. This finding provides evidence that severe malocclusions are associated with lower quality of life and less masticatory efficiency in older patients.

Limitations of a method used for adolescent assessment of smile aesthetics.

AIMS: To establish whether adolescent orthodontic patients with hypodontia have a preference between the aesthetic outcomes of two treatment strategies for lateral incisor agenesis. MATERIALS/METHODS: Standardized photographs of pre-orthodontic patients with missing lateral incisors were manipulated to produce images that represented space opening and tooth replacement in the lateral incisor space and space closure with canine substitution into the lateral incisor space. Adolescent orthodontic patients with hypodontia were recruited to assess the aesthetics of the images. A control group of subjects without tooth agenesis was recruited. Each examiner undertook two tests to assess the smile aesthetics of the images: (1) rating attractiveness using visual analogue scale (VAS) and (2) choice of preference between pairs of images. RESULTS: Difficulties experienced with image manipulation and poor intra-examiner reliability of the VAS make interpretation of the results challenging. Care should be taken if findings are used to aid clinical decisions, as the validity of the main findings is questionable. Results suggest that although adolescents perceive a difference in the aesthetic result of space opening and space closure for missing lateral incisors, the impact on the smile attractiveness is not clinically significant. When forced to choose between the aesthetics of space opening or space closure, the majority of examiners chose space opening with tooth replacement. No difference was found in smile ratings or preferences between adolescents with hypodontia and those with no missing teeth. CLINICAL IMPLICATIONS: The methods used in this study may not be reliable for adolescent assessment of aesthetics.

Discriminative ability of the generic and condition specific Oral Impact on Daily Performance (OIDP) among adolescents with and without hypodontia.

BACKGROUND: The aims of this study were to (1) investigate to what extent the generic and condition specific (CS) forms of the oral impact of daily performance (OIDP) inventory discriminate between a group of patients with hypodontia and a group of patients having malocclusion, (2) assess the association of the generic and CS OIDP with severity and localisation of hypodontia, whilst adjusting for patients' age and sex. METHODS: A total of 163 patients aged 10-17 years were included in a cross-sectional study. Two groups were investigated: 62 patients with non-syndromic hypodontia and 101 non-hypodontia patients. Both groups had a malocclusion of similar treatment need. All patients underwent a clinical and radiographic examination and completed a Norwegian version of the generic and the CS OIDP inventory. CS scores were established for impacts attributed to hypodontia. RESULTS: The mean number of missing teeth in the hypodontia group was 6.2. The prevalence of generic and CS oral impacts in the hypodontia group were 64% and 30%, and the corresponding rates in the non-hypodontia group were 62% and 10%. The generic OIDP did not discriminate between the two groups with respect to overall scores. The CS OIDP discriminated strongly between patients with and without hypodontia regarding problems with emotional status, showing teeth, social contact, speaking and carrying out work. Compared to the non-hypodontia group, patients with hypodontia, with severe hypodontia (≥ 6 missing teeth) and upper anterior hypodontia were respectively 3.4, 2.5 and 7.0 times more likely to report any oral impact attributed to small teeth, gaps between teeth and missing teeth. CONCLUSIONS: Hypodontia and malocclusion patients report a considerable burden of oral impacts. The CS-OIDP measure discriminated most effectively between patients with and without hypodontia and was related to severity and upper anterior localisation of hypodontia.

Smile attractiveness of patients treated for congenitally missing maxillary lateral incisors as rated by dentists, laypersons, and the patients themselves.

STATEMENT OF PROBLEM: Esthetic judgments can help dental professionals better understand how attentive people are to their own smiles and those of others. PURPOSE: The purpose of this study was to evaluate the smile attractiveness of patients treated for maxillary lateral incisor agenesis compared with that of individuals with a complete dentition. MATERIAL AND METHODS: Photographs were made of the smiles of patients with maxillary lateral incisor agenesis who were treated with space closure and teeth recontouring (n=26) or space opening and implants (n=20) and of a control group with a complete dentition (n=22). Both laypersons and dentists assessed smile attractiveness by using a visual analog scale. Patients and controls also assessed the level of satisfaction with their own smile. Assessments were performed twice, and the reliability of the method was determined with Cronbach α and intraclass correlation. Multifactorial and 1-way ANOVA were used to analyze smile attractiveness and participant satisfaction, followed by the Bonferroni post hoc test (α=.05). RESULTS: Attractiveness ratings by dentists and laypersons did not differ significantly among the study groups (P=.64). The ratings of male dentists and female laypersons differed significantly from those of other evaluators (P=.01). Patients with space closure and teeth recontouring were significantly more satisfied than controls (P=.002). No significant differences were found between the patients with implants and controls or between the patients with implants and those with space closure and teeth recontouring. CONCLUSIONS: The smiles of patients with maxillary lateral incisor agenesis were judged to be as attractive as those of the controls. Male dentists were the most critical raters, closely followed by female laypersons. All participants had high levels of satisfaction with their own smile; patients treated with space closure and teeth recontouring were the most satisfied.

Perceptions of dental professionals and laypeople to altered dental esthetics in cases with congenitally missing maxillary lateral incisors.

BACKGROUND: The smile perception of patients is not strictly related to standardized protocols and technical implications which certainly affect clinicians' decisions. The absence of maxillary lateral incisors could affect smile esthetics either with treatment or not. The aim of the present study was to investigate if different perceptions on altered smiles due to missing maxillary lateral incisors, with or without treatment, exist among different groups of people (laypersons, adult orthodontic patients, general dentists, and orthodontists). METHODS: An ideal smile model was selected and altered simulating different malocclusions and treatment options. Twelve simulations were submitted to four categories of respondents: laypeople, adult orthodontic patients, general dentists and orthodontists. They were asked to express smile perception for each simulation by ranking and rating simulations using a 0 to 100 visual analog scale. Analysis of variance was used to determine if there were statistically significant differences in values assigned among the four categories of respondents for each simulation. RESULTS: Significant differences in smile perceptions were found between professionals (dentists and orthodontists) and laypeople. Presence of dental tipping and marked diastema in the arch were disharmonious aspects less tolerated in a smile by all categories of evaluators. Simulations associated with space closure orthodontic treatment were ranked as the most attractive smile and significantly ranked higher by dental professionals than patients and laypeople. CONCLUSIONS: Treatment, absence of diastema, and symmetry were the most accepted characteristics by all categories of respondents. Ideal orthodontic treatment options might be overestimated by clinicians when compared to laypeople's smile perception.

The impact of tooth agenesis on oral health-related quality of life in children.

OBJECTIVE: The purpose of this cross-sectional survey was to evaluate the psychosocial impact of tooth agenesis in children and to investigate the potential influence of gender, socioeconomic status, severity of tooth absence, and the number of retained primary teeth on their quality of life. MATERIALS AND METHODS: A total of 86 children (36 male, 50 female) with tooth agenesis, aged 11-14 years were recruited from the Birmingham Dental Hospital, United Kingdom. Thirty subjects with a complete dentition and having a low treatment need acted as controls. Children completed the validated Child Perceptions Questionnaire (CPQ) and their parents completed the Parental-Caregiver Perceptions Questionnaire (P-CPQ). RESULTS: The median number of missing teeth in the sample population was 6. There were significant differences in the oral symptoms, functional limitations and the social and emotional well-being reported between the agenesis and control groups. The overall CPQ scores were significantly higher in children with tooth agenesis (P<0·001). No significant correlation was detected between the number of missing teeth and the quality of life score. There was no influence found on the CPQ score from gender, socioeconomic status, the site of agenesis or the presence of retained primary teeth. There was moderate correlation between parental and child reported quality of life. CONCLUSIONS: Tooth agenesis can have a significant impact on the quality of life of children, resulting in oral symptoms, functional limitation and also affecting emotional and social well-being. This does not appear to be related to the number of missing teeth. This study has implications for our understanding of the effect of tooth absence on the quality of life of children and their parents and addressing these reported impacts may help to improve patient satisfaction.

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.

The use of the Oral Health Impact Profile to measure the impact of mild, moderate and severe hypodontia on oral health-related quality of life in young adults.

There is limited understanding of the subjective impact of congenital absence of teeth in patients with hypodontia. This study aimed to investigate the impact of mild, moderate and severe hypodontia on oral health-related quality of life (OHRQoL) and its relationship to age, gender and extent of hypodontia prior to treatment. 82 patients (43 females and 39 males, age range from 16 to 34 years) with a confirmed diagnosis of non-syndromic hypodontia were recruited for this study prior to treatment. Demographic details were recorded and a clinical examination documented the number and location of missing teeth. Participants were also asked to complete an oral health-related quality of life measure, the OHIP-49. The impact was of hypodontia was significant, with appearance concerns being the most prevalent impacts on oral health-related quality of life. Gender was a significant predictor of the overall OHIP-49 score, with females having higher level of impacts. The number and location of missing permanent teeth was not a good predictor of quality of life. However, location of missing teeth was a predictor of the psychological discomfort subscale score. There was a positive correlation between age and the functional limitation and physical disability subscale scores. This study shows that the impact of hypodontia on oral health-related quality of life is substantial.

Impact of resin bonded bridgework on quality of life of patients with hypodontia.

OBJECTIVES: To determine the impact of hypodontia on the quality of life of adolescent and young adult patients, and, to assess the impact of restoring tooth spaces with resin bonded bridgework on quality of life of patients with hypodontia. METHODS: In a prospective study, 82 patients with a confirmed diagnosis of hypodontia participated. The primary outcome was oral health related quality of life (OHRQoL) and this was measured using the OHIP-49 prior to treatment. The pre-treatment sample was then divided into two groups: the test group (n=40 patients) who had completed orthodontic treatment and had tooth spaces restored with resin bonded bridgework, and, a control group (n=42 patients) who were still in the process of orthodontic treatment. All patients completed a follow-up OHIP-49, and between and within group comparisons made. RESULTS: The pre-treatment sample included 43 females and 39 males, age ranged from 16 to 34 years (median age 19). Forty-three patients had more than 4 congenitally missing teeth and thirty-nine had ≤ 4 congenitally missing teeth. There were no differences between the groups prior to treatment. For the test group, there was a significant improvement in median OHIP summary scores (p<0.001) after treatment. OHIP scores deteriorated to a significant degree for control subjects (p=0.002). The effect sizes for the pre-post treatment change in both groups were moderate to large. CONCLUSIONS: Hypodontia has a significant impact on oral health related quality of life. Provision of resin bonded bridges has a positive impact on oral health related quality of life of patients with hypodontia.

Assessment of a condition-specific quality-of-life measure for patients with developmentally absent teeth: validity and reliability testing.

OBJECTIVES: This article describes the process of validity and reliability testing of a condition-specific quality-of-life measure for patients with hypodontia presenting for orthodontic treatment. The development of the instrument is described in a previous article. SETTING AND SAMPLE POPULATION: Royal Devon and Exeter NHS Foundation Trust & Musgrove Park Hospital, Taunton. MATERIALS AND METHODS: The child perception questionnaire was used as a standard against which to test criterion validity. The Bland and Altman method was used to check agreement between the two questionnaires. Construct validity was tested using principal component analysis on the four sections of the questionnaire. Test-retest reliability was tested using intraclass correlation coefficient and Bland and Altman method. Cronbach's alpha was used to test internal consistency reliability. RESULTS: Overall the questionnaire showed good reliability, criterion and construct validity. This together with previous evidence of good face and content validity suggests that the instrument may prove useful in clinical practice and further research. CONCLUSIONS: This study has demonstrated that the newly developed condition-specific quality-of-life questionnaire is both valid and reliable for use in young patients with hypodontia.

Smile esthetics: the influence of posterior maxillary gingival margin position.

PURPOSE: Successful replacement of posterior teeth using contemporary prosthodontic techniques in esthetically demanding cases relies upon visual replication of the natural posterior dentition and surrounding gingival architecture. There is currently little in the way of guidance for creating ideal or acceptable gingival relationships for posterior teeth. MATERIALS AND METHODS: A cross-sectional study was conducted comparing perceptions of four groups of individuals to six digitally manipulated images with various posterior teeth gingival margin position configurations. A total of 120 volunteers aged 12 years to 80 years, comprising 30 patients diagnosed with hypodontia, 30 patients diagnosed with periodontal disease, 30 patients without either condition, and 30 qualified dentists were recruited from the Eastman Dental Institute & Hospital, London. A ranked order of preference for each set was obtained, and this was repeated after a minimum time interval of 10 minutes. RESULTS: Posterior gingival margin configurations from 0 mm to 2 mm (measured at the first premolar) were deemed most esthetic by the majority of the patient groups; dentists had a strong preference for the 1 mm configuration. Dentists appeared to be more perceptive to the alterations in gingival positions. CONCLUSIONS: Posterior gingival margin configurations where the first premolar margins were 1 mm lower than the canine margins were deemed the most esthetically pleasing; however, it is likely that a range of acceptability of 1 mm deviations from this ideal exists.

The impact of hypodontia: a qualitative study on the experiences of patients.

Congenital absence of teeth affects 2-6 per cent of the population, but its impact on quality of life (QoL) is not fully understood. The symptoms of hypodontia can vary and therefore also the treatment and management of this condition. Determining and understanding the possible impact of hypodontia on patients could inform and improve the management of such patients. A purposive sample of 10 participants aged 16-25 years (five males and five females) with mild, moderate, or severe hypodontia at various stages of treatment was recruited. The participants had previously completed a 49-item Oral Health Impact Profile (OHIP) questionnaire with summary scores ranging from 24 to 143. All subjects participated in semi-structured interviews, and these were transcribed and analysed using NVivo software. The results show that the role of hypodontia patients in the treatment decision-making process changes significantly as they move from childhood to adulthood. Participants indicated dissatisfaction with the lack of communication with dental services throughout early adolescence as they became more cognizant of their condition which in turn led to concerns with regard to appearance which impacted on their psychosocial well-being. Hypodontia patients expect improved communication with dental practitioners and services as they become more cognizant of their condition and wish to become more actively involved in the decision-making process regarding their current and future treatment.

Ectodermal dysplasia: dental management and benefits, a case report.

AIM: This case report describes a method of restoring function and aesthetics in a 9-year-old girl with ectodermal dysplasia with 15 years follow-up. Ectodermal dysplasia is both physically and emotionally devastating to patients. It is important that they are treated at an early age to help their social interaction. With proper dental intervention, the quality of life can be improved for patients with ectodermal dysplasia.