Background: The object of this paper was to provide an overview of rare diseases (RDs) with periodontal manifestations and allocate them to relevant categories. Methods: In ROMSE, a database for “Rare Diseases with Orofacial Involvement”, all 541 entities were analyzed with respect to manifestations of periodontal relevance. Inclusion criteria were periodontally relevant changes to the oral cavity, in accordance with the 2018 version of the Classification of Periodontal and Peri-Implant Diseases and Conditions. Rare diseases were recorded, using the methodology described, and subsequently compared with the Orphanet Classification of Rare Diseases. Results: A total of 76 RDs with periodontal involvement were recorded and allocated in accordance with the Classification of Periodontal and Peri-Implant Diseases and Conditions. Of the 541 RDs analyzed as having known orofacial manifestations, almost 14 percent indicated a periodontally compromised dentition. Conclusions: Around 14 percent of RDs with an orofacial involvement showed periodontally relevant manifestations, which present not only as a result of gingivitis and periodontitis, but also gingival hyperplasia in connection with an underlying disease. Thus, dentists play an important role in therapy and early diagnoses of underlying diseases based on periodontally relevant manifestations.

Background: Systematic scleroderma is a rare chronic autoimmune disease of unknown aetiology. The aim of this study was to identify the prevalence of orofacial pathognomonic conditions in patients with systemic scleroderma using only randomised prospective studies that investigated the treatment of oral and maxillofacial changes, highlighted associations between the disease and Sjogren’s syndrome, and/or analysed the effect of oral hygiene. Methods: The literature was systematically reviewed based on Cochrane Library, EMBASE, PubMed, Scopus, and Web of Science articles published up to March 2020. The primary endpoint of this analysis was defined as an estimation of the prevalence of oral mucosal changes in different areas of the oral cavity (oral mucosa, tongue, lip, periodontal status, bones, and other regions) in patients suffering from scleroderma. Therefore, a systematic literature search (Cochrane Library, EMBASE, PubMed, Scopus, and Web of Science) was conducted and limited by the publication date (1950-03/2020) and the publication language (English). Extracted frequencies were pooled using methods for meta-analysis. In order to obtain the highest level of evidence, only prospective study reports were considered to be eligible. Results: After full-text screening, 14 (766 patients) out of 193 publications were eligible for the final analysis. Twelve studies produced reliable results in the final data sets. Calculation of the pooled effect estimate (random effects model) revealed a prevalence of 57.6% (95% CI: 40.8–72.9%) for the main area “lip”. For the area “oral mucosa”, a prevalence of 35.5% (95% CI: 15.7–62.0%) was calculated. The prevalence for “other regions” was only based on studies with salivary changes and was calculated to be 25.4% (95% CI: 14.2–41.3%). Conclusion: The most pathognomonic conditions in the orofacial region in patients with systemic scleroderma affect the lips, oral mucosa, and salivary glands.

Background and Objectives: Preliminary studies emphasize the similar performance of autogenous bone blocks (AUBBs) and allogeneic bone blocks (ALBBs) in pre-implant surgery; however, most of these studies include limited subjects or hold a low level of evidence. The purpose of this review is to test the hypothesis of indifferent implant survival rates (ISRs) in AUBB and ALBB and determine the impact of various material-, surgery- and patient-related confounders and predictors. Materials and Methods: The national library of medicine (MEDLINE), Excerpta Medica database (EMBASE) and Cochrane Central Register of Controlled Trials (CENTRAL) were screened for studies reporting the ISRs of implants placed in AUBB and ALBB with ≥10 participants followed for ≥12 months from January 1995 to November 2021. The review was conducted in accordance with Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. The risk of bias was assessed via several scoring tools, dependent on the study design. Means of sub-entities were presented as violin plots. Results: An electronic data search resulted in the identification of 9233 articles, of which 100 were included in the quantitative analysis. No significant difference (p = 0.54) was found between the ISR of AUBB (96.23 ± 5.27%; range: 75% to 100%; 2195 subjects, 6861 implants) and that of ALBB (97.66 ± 2.68%; range: 90.1% to 100%; 1202 subjects, 3434 implants). The ISR in AUBB was increased in blocks from intraoral as compared to extraoral donor sites (p = 0.0003), partially edentulous as compared to totally edentulous (p = 0.0002), as well as in patients younger than 45 as compared to those older (p = 0.044), cortical as compared to cortico-cancellous blocks (p = 0.005) and in delayed implantations within three months as compared to immediate implantations (p = 0.018). The ISR of ALBB was significantly increased in processed as compared to fresh-frozen ALBB (p = 0.004), but also in horizontal as compared to vertical augmentations (p = 0.009). Conclusions: The present findings widely emphasize the feasibility of achieving similar ISRs with AUBB and ALBB applied for pre-implant bone grafting. ISRs were negatively affected in sub-entities linked to more extensive augmentation procedures such as bone donor site and dentition status. The inclusion and pooling of literature with a low level of evidence, the absence of randomized controlled clinical trials (RCTs) comparing AUBB and ALBB and the limited count of comparative studies with short follow-ups increases the risk of bias and complicates data interpretation. Consequently, further long-term comparative studies are needed.

Fifteen percent of the 5000 to 8000 rare diseases (RDs) can manifest in the oral and maxillofacial region. Little attention has been paid to the care situation of people with RDs in dentistry. Hence, the aim of this study was to assess the level of knowledge about RDs among dentists at a university hospital (DUs) compared to dentists with different professional backgrounds and among general dentists, specialist dentists and DUs in the chamber district of Westfalen-Lippe. Moreover, self-assessment of the level of knowledge was evaluated. A questionnaire was designed, which was made available digitally via a link. A random sample of 1500 dentists, specialist dentists, and oral- and craniomaxillofacial surgeons from the membership of the Dental Association of Westfalen-Lippe, and all dentists, specialist dentists, and oral- and craniomaxillofacial surgeons working at the University Dental Hospitals Münster and Witten/Herdecke, were invited to participate to our study. Differences in the level of knowledge between DUs and non-DUs and differences between DUs, general dentists, and specialist dentists were tested via two-sided Fischer’s exact tests. Differences between the three groups of self-assessment of the level of knowledge and the self-assessment of how sufficient their own knowledge about RDs is were tested via two-sided Kruskal–Wallis tests. The global level of significance was controlled by the Bonferroni method. A total of 267 questionnaires were completed, of which 64.0% were answered by general dentists, 25.5% by specialist dentists and 10.5% by DUs. DUs had a significant higher level of knowledge about RDs (adjusted p = 0.012) compared to non-DUs and achieved higher scores (median = 16.5 points) than general (median = 13 points) and specialist dentists (median = 13 points) (p = 0.001). In the self-assessments, the differences were not significant (p > 0.05). In conclusion, most participants showed no or little knowledge about RDs, and DUs had a significant higher level of knowledge than non-university dentists.

Background and objectives: Rare diseases affect an estimated four million patients in Germany. Approximately 15% of the approximately 6000 to 8000 rare diseases known globally show manifestations in the dental, oral and maxillofacial regions. The present survey evaluated the knowledge and management of rare diseases and their orofacial alterations by dentists, dental specialists and oral and maxillofacial surgeons and dentists working at university hospitals for dentistry and/or oral and maxillofacial surgery. Materials and Methods: The study was designed as an anonymous cross-sectional study. Two anonymous online surveys were performed in all dentists in Germany using the open-source survey software limesurvey. The study cohorts were divided into dentists, dental specialists and oral and maxillofacial surgeons in practice, and dentists who worked in university dental and oral and maxillofacial surgery centers. The survey was performed between 1 October 2020 and 31 March 2021. Results: A total of 309 dentists and oral and maxillofacial surgeons in private practice and 18 dentists or oral and maxillofacial surgeons working at universities participated. A total of 209 (86.7%) study participants working in private practice indicated that the topic of rare diseases should be considered clinically relevant. University participants indicated that there was a lecture on rare diseases in only 7 (63.6%) cases. Only 2 (13.3%) participants reported active research on the topic in their department. Conclusions: The current knowledge on rare diseases is inadequate in suitable screening and therapy. Most of the participants believed that knowledge of rare diseases was very important for daily dental practice. The self-estimations showed that all of the participants estimated their knowledge as very good or inadequate, with a tendency in the direction of inadequate knowledge.

Only a few case reports and case series dealing with oral and dental health care are available in literature until now. The aim of the present pilot study was to determine the status of dental health in comparison to matched controls and to heighten the neurologists’ and dentists’ awareness of the oral aspects of the disease.