Phenotypes, Genetics, and Estimated Prevalence of Focal Dermal Hypoplasia (Goltz Syndrome): A Single-Center Report.

BACKGROUND: Focal dermal hypoplasia (FDH), also known as Goltz syndrome, is a rare ectodermal dysplasia that primarily affects the skin, skeleton, and eyes. It is an X-linked dominant disorder, predominantly seen in females, caused by pathogenic variants in PORCN. METHODS: We characterized a case series of four genetically confirmed FDH patients (three females, one male) at Aarhus University Hospital, Denmark. We estimated the FDH prevalence from our local cohort and nationwide registry data. RESULTS: Three patients had characteristic dermatological findings suspicious for FDH and confirmed by targeted PORCN analysis. One patient had an atypical presentation with several malformations but only subtle skin changes and was diagnosed following trio exome-sequencing analysis. Skin atrophy with fat herniations and telangiectasias were typical cutaneous findings. Limb malformations included oligodactyly (cleft foot), syndactyly, and polydactyly. Eye abnormalities included coloboma and microphthalmos. Facial dysmorphology was defined by asymmetry, thin upper lip, and malformed ears. One patient developed a giant cell bone tumor, which is a rare feature of FDH. Dental findings included enamel hypoplasia with vertical grooving and irregular crowns. Four PORCN variants were identified, including three not previously reported in the literature.We estimated a regional point prevalence in Western Denmark of 1.6 cases per million population (95% confidence intervals (CI): 0.7-3.7 per million) and a nationwide registry-based point prevalence of 1.2 cases per million population (95% CI: 0.6-2.4 per million). CONCLUSIONS: FDH is an extremely rare and complex multisystem disorder of variable presentation, which requires close multidisciplinary collaboration for diagnosis and patient care.

EUROPEAN SOCIETY OF ENDODONTOLOGY Undergraduate Curriculum Guidelines for Endodontology.

Undergraduate education should accomplish graduates who are skilled to provide quality care for patients, who are aware of their scope of practice, competency level and limits and who are open to referring patients whose needs are beyond their own skills and experience. They should also become self-efficacious. Quality performance begins with good diagnosis and decision-making. Graduates should understand when to treat, why to treat, what to treat and how to treat. These guidelines include a list of capabilities that the graduating student will be expected to have achieved to provide a minimum level of competency in endodontics. Theoretical knowledge, practical skills, understanding and insight should be assessed, with both formative and summative assessment procedures, making use of reflection and feedback. Endodontic procedures should be undertaken within the context of comprehensive patient care and should be evidence based. Students should not perform treatments on patients until they have demonstrated in a pre-clinical setting that they possess the required skills. Only if it is not possible to simulate a specific procedure sufficiently in a pre-clinical setting should students learn this procedure by performing it clinically under close supervision. Clinical endodontics should ideally be supervised by endodontists or by staff with special knowledge, interest and self-efficacy in endodontics. It is advised to ensure that students apply their knowledge and practice their skills periodically throughout the continuum of endodontic education until graduation. A philosophy of lifelong learning and evidence-based practice should be instilled in all dental undergraduates.

PRIDASE 2024 guidelines for reporting diagnostic accuracy studies in endodontics: Explanation and elaboration.

The Preferred Reporting Items for Diagnostic Accuracy Studies in Endodontics (PRIDASE) 2024 guidelines are based on the Standards for Reporting of Diagnostic Accuracy Studies (STARD) 2015 guidelines and the Clinical and Laboratory Images in Publications (CLIP) principles, with the addition of items specifically related to endodontics. The use of the PRIDASE 2024 guidelines by authors and their application by journals during the peer review process will reduce the possibility of bias and enhance the quality of future diagnostic accuracy studies. The PRIDASE 2024 guidelines consist of a checklist containing 11 domains and 66 individual items. The purpose of the current document is to provide an explanation for each item on the PRIDASE 2024 checklist, along with examples from the literature to help readers understand their importance and offer advice to those developing manuscripts. A link to the PRIDASE 2024 explanation and elaboration document is available on the Preferred Reporting Items for study Designs in Endodontology (PRIDE) website (https://pride-endodonticguidelines.org/pridase/) and on the International Endodontic Journal website (https://onlinelibrary.wiley.com/page/journal/13652591/homepage/pride-guidelines.htm).

PRIDASE 2024 guidelines for reporting diagnostic accuracy studies in endodontics: A consensus-based development.

Studies investigating the accuracy of diagnostic tests should provide data on how effectively they identify or exclude disease in order to inform clinicians responsible for managing patients. This consensus-based project was undertaken to develop reporting guidelines for authors submitting manuscripts, which describe studies that have evaluated the accuracy of diagnostic tests in endodontics. These guidelines are known as the Preferred Reporting Items for Diagnostic Accuracy Studies in Endodontics (PRIDASE) 2024 guidelines. A nine-member steering committee created an initial checklist by integrating and modifying items from the Standards for Reporting of Diagnostic Accuracy (STARD) 2015 checklist and the Clinical and Laboratory Images in Publications (CLIP) principles, as well as adding a number of new items specific to the specialty of endodontics. Thereafter, the steering committee formed the PRIDASE Delphi Group (PDG) and the PRIDASE Online Meeting Group (POMG) in order to collect expert feedback on the preliminary draft checklist. Members of the Delphi group engaged in an online Delphi process to reach consensus on the clarity and suitability of the items in the checklist. The online meeting group then held an in-depth discussion on the online Delphi-generated items via the Zoom platform on 20 October 2023. According to the feedback obtained, the steering committee revised the PRIDASE checklist, which was then piloted by several authors when preparing manuscripts describing diagnostic accuracy studies in endodontics. Feedback from this process resulted in the final version of the PRIDASE 2024 checklist, which has 11 sections and 66 items. Authors are encouraged to use the PRIDASE 2024 guidelines when developing manuscripts on diagnostic accuracy in endodontics in order to improve the quality of reporting in this area. Editors of relevant journals will be invited to include these guidelines in their instructions to authors.

An international consensus study to identify "what" outcomes should be included in a core outcome set for endodontic treatments (COSET) for utilization in clinical practice and research.

BACKGROUND: Development of a standardized set of topic-specific outcomes known as a Core Outcome Set (COS) is important to address issues of heterogeneity in reporting research findings in order to streamline evidence synthesis and clinical decision making. AIM: The aim of the current international consensus study is to identify "what" outcomes to include in the Core Outcome Set for Endodontic Treatments (COSET). Outcomes of various endodontic treatments (non-surgical root canal treatment, surgical endodontics, vital pulp treatment and revitalization procedures) performed on permanent teeth were considered. METHODS: A standard validated methodology for COS development and reporting was adopted. The process involved identification of existing outcomes through four published scoping reviews. This enabled creation of a list of outcomes to be prioritized via semi-structured patient interviews, e-Delphi process and a consensus meeting with a range of relevant global stakeholders. Outcomes were prioritized using a 1-9 Likert scale, with outcomes rated 7-9 considered critical, 4-6 are important and 1-3 are less important. Outcomes rated 7-9 by ≥70% and 1-3 by <15% of participants were considered to achieve consensus for inclusion in the COS. The outcomes that did not achieve consensus in the first round were considered for further prioritization in the second Delphi round and consensus meeting. Final decisions about the outcomes to include in COSET were made by voting during the consensus panel meeting using the Zoom Poll function. RESULTS: A total of 95 participants including patients contributed to the COS development process. The consensus panel recommended, with strong consensus, eight outcomes shared across all treatment modalities for inclusion in COSET: pain; signs of infection (swelling, sinus tract); further intervention/exacerbation; tenderness to percussion/palpation; radiographic evidence of disease progression/healing; function; tooth survival; and patient satisfaction. Additional treatment specific outcomes were also recommended. DISCUSSION: Many of the outcomes included in COSET are patient reported. All should be included in future outcomes studies. CONCLUSION: COSET identified outcomes that are important for patients and clinicians and validated these using a rigorous methodology. Further work is ongoing to determine "how" and "when" these outcomes should be measured.

Outcomes reporting in systematic reviews on non-surgical root canal treatment: A scoping review for the development of a core outcome set.

BACKGROUND: Studies related to non-surgical root canal treatment are amongst the most frequently performed clinical studies in endodontics. However, heterogeneity in reporting outcomes and lack of standardization is a significant challenge to evidence synthesis and guideline development. OBJECTIVES: The aims of the present scoping review were to (a) identify outcomes reported in systematic reviews evaluating non-surgical root canal treatment; (b) identify how and when the reported outcomes were measured; (c) assess possible selective reporting bias in the included studies. The information obtained in this study should inform the development of a core outcome set (COS) for non-surgical root canal treatment. METHODOLOGY: Structured literature searches were performed to identify systematic reviews on non-surgical root canal treatments published in English between January 1990 and December 2020. Two reviewers undertook study selection and data extraction. Outcomes were categorized according to a healthcare taxonomy into five core areas (survival, clinical/physiological changes, life impact, resource use, and adverse events). The outcome measurement tools and length of follow-up were recorded. RESULTS: Seventy-five systematic reviews were included, of which 40 included meta-analyses. Most reviews reported on physiological and clinical outcomes, primarily pain and/or radiographic assessment of periapical status, and a variety of measurement tools and scales were used. Few reviews focused on tooth survival, life impact, resources, and adverse events. The heterogeneity amongst the reviews was large on all parameters. Less than 40% of the reviews assessed the risk of selective reporting. DISCUSSION: Overall aims of the included reviews were highly heterogenic; thus, outcomes and how they were measured also varied considerably. Patient-centred outcomes and the use of resources were rarely reported on. CONCLUSIONS: Most studies reported on physiological and clinical outcomes, in particular pain and/or radiographic healing. Measurement tools, scales, thresholds, and follow-up periods varied greatly within each outcome, making comparison across studies complicated. Less than 40% of the reviews assessed risk of selective reporting; thus, selective bias could not be ruled out. The presented information on reported outcomes, measurement tools and scales, and length of follow-up may guide the planning of future research and inform the development of a COS for non-surgical root canal treatment.

Effect of a formalin-based fixation method on bone mineral content in human ex-vivo specimens.

OBJECTIVE: Histopathology of formalin-fixated human ex-vivo specimens may be used as reference standard for evaluation of diagnostic index tests like CBCT or MRI. The aim was to estimate changes in bone mineral content (BMC) over time in human ex-vivo bone specimens fixated in a formalin-based solution for 24 h followed by storage in an alcohol-based medium for six months, assessed by dual-energy X-ray absorptiometry (DXA). METHODOLOGY: Bone specimens (n = 19) from human ex-vivo mandibles donated for science were included. BMC was measured by DXA before fixation (D0), after 24 h of immersion fixation in a formalin-based solution (D1), and hereafter every 30 days (M1-M6) during storage in a 30% ethanol-based storage medium for 6 months. Changes in BMC from D0 to D1 and from D0 to M6 were calculated and mean change in BMC estimated. RESULTS: Mean change in BMC from D0 to D1 was -0.73% (95% CI -1.75%; 0.29%), and from D0 to M6 -1.19% (95% CI -2.14%; -0.23%). CONCLUSIONS: No changes in BMC of ex-vivo human bone specimens were found after 24 h formalin-based immersion fixation. After six months storage in an ethanol-based medium, BMC mean loss of 1% was detected. In this range, changes in BMC are not clinically relevant.

Quality aspects of ex vivo root canal treatments done by undergraduate dental students using four different endodontic treatment systems.

OBJECTIVE: To evaluate factors associated with treatment quality of ex vivo root canal treatments performed by undergraduate dental students using different endodontic treatment systems. MATERIAL AND METHODS: Four students performed root canal treatment on 80 extracted human teeth using four endodontic treatment systems in designated treatment order following a Latin square design. Lateral seal and length of root canal fillings was radiographically assessed; for lateral seal, a graded visual scale was used. Treatment time was measured separately for access preparation, biomechanical root canal preparation, obturation and for the total procedure. Mishaps were registered. An ANOVA mirroring the Latin square design was performed. RESULTS: Use of machine-driven nickel-titanium systems resulted in overall better quality scores for lateral seal than use of the manual stainless-steel system. Among systems with machine-driven files, scores did not significantly differ. Use of machine-driven instruments resulted in shorter treatment time than manual instrumentation. Machine-driven systems with few files achieved shorter treatment times. With increasing number of treatments, root canal-filling quality increased, treatment time decreased; a learning curve was plotted. No root canal shaping file separated. CONCLUSIONS: The use of endodontic treatment systems with machine-driven files led to higher quality lateral seal compared to the manual system. The three contemporary machine-driven systems delivered comparable results regarding quality of root canal fillings; they were safe to use and provided a more efficient workflow than the manual technique. Increasing experience had a positive impact on the quality of root canal fillings while treatment time decreased.

Hypohidrotic ectodermal dysplasia caused by an intragenic duplication in EDAR.

Hypohidrotic Ectodermal Dysplasia is a syndrome with hypotrichosis, hypohidrosis, and hypodontia as the main symptoms. The prevalence is estimated to one in 5000-10,000 persons. In 10-15% the disease is caused by pathogenic variants in EDAR, and most of the known causal variants to date are missense or nonsense variants. We present a patient with classic Hypohidrotic Ectodermal Dysplasia and mammary gland aplasia with a duplication within EDAR as the likely cause. The duplication is de novo in the patient, and genome sequencing of DNA extracted from blood has revealed that the duplication is in tandem conformation, most likely entailing an altered EDAR protein with a dominant negative effect. This is to our knowledge the first report of an intragenic duplication in EDAR as causal for Hypohidrotic Ectodermal Dysplasia.

A comparison of video-based and slide-based teaching before hands-on rubber dam application: A quantitative and qualitative study.

PURPOSE/OBJECTIVES: Instructional videos may demonstrate the execution of complex clinical procedures and the cooperation between members of the dental team better than traditional slide-based teaching materials. The aim of the present study was to compare the effect of a procedural video on student ratings to a traditional still-image-based presentation in a course on rubber dam application. METHODS: In a randomized, double-blind, parallel arm design, participants (46 dental students) completed a seven-item, five-step Likert-scale questionnaire at baseline (t1), after a video-based or slide-based demonstration of rubber dam application (t2) and after hands-on training (t3). The students' judgement on the benefits of rubber dam (items 1-3), their motivation to use rubber dam (item 4), their self-efficacy (items 5-6) and their expected use of the teaching material (item 7) were assessed. Changes in the students' individual answers were analyzed for each item and comparison between intervention groups made. Moreover, the impact of the teaching format on in-class discussions was analyzed qualitatively using a thematic approach RESULTS: Both interventions arose comparable significant improvement in the students' Likert-scale ratings from t1 to t2, and again from t2 to t3. No significant differences between intervention groups were found in the students' ratings or in the qualitative analysis. CONCLUSIONS: Procedural videos have proven to be a valuable learning aid in a variety of teaching formats, but in the context of a live lecture, they may not constitute an improvement over traditional text- and still-image-based presentations.

Hands-on live demonstration vs. video-supported demonstration of an aesthetic composite restoration in undergraduate dental teaching.

PURPOSE/OBJECTIVES: Live hands-on demonstration of dental procedures is a central format in undergraduate dental teaching. It captures the immediacy of the clinical situation and allows for direct communication between instructor and students, but it also requires an experienced instructor who is able to handle both the performed treatment and its visualization alongside the actual teaching. The aim of the present work is to compare the hands-on demonstration of a class IV composite restoration to a teaching format where the instructor guided the students through a prerecorded procedural video of the same treatment. METHODS: The effect of both interventions on the students' self-perceived learning outcomes was analyzed by questionnaires (response rate 100%) in a randomized controlled double-blind (participants, outcome assessor) parallel group design (September 10 to October 3, 2019). In-class discussions were explored qualitatively by thematic analysis. RESULTS: Both teaching formats increased the students' self-reported motivation, self-efficacy, and patient-centeredness in a similar way, with no significant differences between interventions. During in-class discussions, both the instructor and the students were more active in the video group. In contrast to the hands-on group, discussions in the video group also involved patient-related topics, such as aesthetics and general health. The video-supported teaching format considerably reduced the amount of time spent on optimizing the visualization of the performed treatment. CONCLUSION: Video-supported instructor-guided demonstrations may represent a promising teaching format as an alternative to live hands-on demonstrations of dental procedures in undergraduate dental education.

Diagnostic validity of periapical radiography and CBCT for assessing periapical lesions that persist after endodontic surgery.

OBJECTIVES: Traditionally, healing after surgical endodontic retreatment (SER); i.e. apicectomy with or without a retrograde filling, is assessed in periapical radiographs (PR). Recently, the use of cone beam CT (CBCT) has increased within endodontics. Generally, CBCT detects more periapical lesions than PR, but basic research on the true nature of these lesions is missing. The objective was to assess the diagnostic validity of PR and CBCT for determining inflammation in SER cases that were re-operated (SER-R) due to unsuccessful healing, using histology of the periapical lesion as reference for inflammation. METHODS: Records from 149 patients, receiving SER 2004-10, were screened. In total 108 patients (119 teeth) were recalled for clinical follow-up examination, PR and CBCT, of which 74 patients (83 teeth) participated. Three observers assessed PR and CBCT as "successful healing" or "unsuccessful healing" using Rud and Molven's criteria. SER-R was offered to all non-healed teeth with expected favourable prognosis for subsequent functional retention. During SER-R, biopsy was performed and histopathology verified whether or not inflammation was present. RESULTS: All re-operated cases were assessed non-healed in CBCT while 11 of these were assessed successfully healed in PR. Nineteen biopsies were examined. Histopathologic diagnosis revealed 42% (teeth = 8) without periapical inflammation, 16% (teeth = 3) with mild inflammation and 42% (teeth = 8) with moderate to intense inflammation. A correct diagnosis was obtained in 58% with CBCT (true positives) and 63% with PR (true positives+true negatives). CONCLUSIONS: Of the re-operated teeth, 42% had no periapical inflammatory lesion, and hence no benefit from SER-R. Not all lesions observed in CBCT represented periapical inflammatory lesions.

Periapical Bone Healing after Apicectomy with and without Retrograde Root Filling with Mineral Trioxide Aggregate: A 6-year Follow-up of a Randomized Controlled Trial.

INTRODUCTION: In cases of post-treatment periapical disease, retreatment may be necessary. To choose the most appropriate retreatment method, knowledge of the long-term prognosis is important. Surgical endodontic retreatment (SER) is a relevant treatment method. This study assessed changes in outcome from 1 to 6 years after surgery. METHODS: SER was performed on teeth randomly allocated to have a MTA root-end filling (MTA group) or smoothing of the orthograde gutta-percha filling after apicectomy (GP group). Patients participating in the 1-year follow-up were reinvited for a 6-year clinical and radiographic examination. Three observers assessed treatment outcome both clinically and radiographically from the 1-year and 6-year follow-up examination. RESULTS: At the 6-year follow-up, 39 of 52 teeth were available and examined (75% participation rate). In the MTA group, 16 of 19 teeth (86%) and in the GP group 11 of 20 teeth (55%) were assessed as successful (P = .04). In the MTA group and the GP group, 80% and 90%, respectively, of teeth assessed as successful at the 1-year follow-up remained successful. All unsuccessful teeth in the MTA group (3 teeth) were lost because of vertical root fracture. CONCLUSIONS: The proportion of healed cases was larger in the MTA group than in the GP group at both the 1-year and 6-year follow-up. Findings indicate that a 1-year follow-up may not be sufficient in assessing the long-term outcome of surgical endodontic retreatment. With a longer follow-up, other factors not directly related to the endodontic treatment may be relevant for a successful outcome. This needs further investigation in larger patient samples.