Generalized Dentin Dysplasia in a Four-Year-old dog

Dentin dysplasia is an autosomal-dominant genetic abnormality that occurs in humans and results in diffuse radiographic dental abnormalities and variable tooth discoloration due to an underlying defect in secondary dentinogenesis. This case report presents distinctive radiographic and histopathologic dental abnormalities in a dog that are consistent with generalized dentin dysplasia. These findings are similar to but not completely analogous to any specific clinical type of dentin dysplasia in humans. Grossly, the majority of the teeth in this case were discolored and most were determined to be vital. Dentin dysplasia should be included in the list of differential diagnoses of discolored teeth and notably this form of discoloration does not necessarily indicate loss of vitality.

200 A Scoring System for Equine Dental Health

Scoring systems have been implemented in veterinary practice to indicate the severity of pathologies, such as lameness and gastric ulceration. The need for a scoring system of equine dentition in relation to digestive health has been identified. A scoring system would allow veterinarians, owners, and researchers to more accurately assess dental health and the resulting impact it may have on chewing ability. A proposed system, the Equine Dental Scoring System (EDSS), was developed via collaboration of a team of veterinarians and equine nutritionists familiar with equine dental abnormalities. The EDSS was designed to assign higher scores corresponding to increasing severity of dental abnormalities that would impede proper chewing. The proposed scoring system ranges from 0 to 5 as follows: 0) no sharp enamel points, soft tissue damage, or malocclusion, (1) sharp enamel points, but no soft tissue damage or malocclusion, (2) sharp enamel points and soft tissue damage, but no malocclusion, (3) mild malocclusion with all aligned teeth meeting level (ex. ramps, hooks), (4) moderate malocclusion with all teeth meeting but not level (ex. wave, smile, diagonal, frown), (5) major malocclusion with one or more teeth not meeting or inhibited temporomandibular joint movement (ex. step, shear, retained cap), and/or infection, and/or pain while chewing. The EDSS was validated by assessing agreement via the Cohen’s kappa statistic between four trained professionals scoring ten images of horse dentition. Both the weighted (к = 0.62) and unweighted (к = 0.73) kappa statistics indicated substantial agreement between scorers, signifying reliable repeatability of the EDSS. Presenting dental health in the form of a score would indicate severity of dental pathologies and allow for quantitative and statistical evaluation of dental health in nutrition research and veterinary medicine.

Sella Turcica Abnormalities, Dental Age and Dental Abnormalities in Polish Children

The frequency of some sella turcica abnormalities on cephalometric radiographs is age related. Chronological age might not overlap with growth; however, no studies could be found on the association between sellar morphology and dental age. Although an association exists between sella turcica bridging and dental abnormalities, no studies have been found correlating sellar abnormalities other than bridging with dental abnormalities. The aim of this study was to find any correlations between sella turcica abnormalities and dental age or dental abnormalities. Methods: Lateral cephalograms and panoramic radiographs of 206 children aged 6–15 years were analyzed for sela turcica abnormalities, Demirijan dental age, and dental abnormalities. Results: The prevalence of dental abnormalities in patients with sela turcica abnormalities was 16.98%, while in those with normal sella, it was 3%. The differences between dental and chronological age were higher in patients with sella turcica abnormalities (p = 0.002). Dental abnormalities were more prevalent (p = 0.001) in patients with sellar abnormalities other than sellar bridging than in those with sellar bridging or normal sella. Conclusions: Sella turcica abnormalities are correlated with delayed dental age. Dental abnormalities are more frequent in patients with sellar abnormalities. Dental abnormalities are less frequent in subjects with sellar bridges compared to those with other sellar abnormalities.

Norwegian Orthodontists’ Experience and Challenges With Treatment of Patients With Cleft Lip and Palate

Background: Patients born with cleft lip and/or palate (CL/P) have orthodontic treatment challenges due to maxilla deficiency, malocclusions, and dental abnormalities. In Norway, orthodontic treatment is done by centralized CL/P teams. Due to traveling restrictions, this treatment might be done locally in the future. The experience of Norwegian community orthodontists in managing such patients has not been investigated previously. Objective: To assess Norwegian orthodontists’ management of patients with CL/P and need for further education. Material and Methods: All orthodontists in Norway were sent a questionnaire about their experience, challenges, and knowledge and asked about their need of further theoretical education and clinical training in the management of patients with CL/P. Results: Norwegian orthodontists’ standard of knowledge of CL/P treatment is adequate. However, few respondents have treated a high number of cleft patients. Eighty-six percent of the participants believed that treating CL/P patients involves challenges, such as time-consuming treatment and technical difficulties. Increased perceived need for more education was revealed among participants stated unpreparedness during education (4 folds), encountered challenges, and lack of knowledge (almost 3 folds). Conclusions: The study revealed that community orthodontists in Norway lack experience and acknowledged the challenges in treating patients with CL/P. Most of the respondents perceived a need for additional education and clinical training to treat CL/P patients competently. The findings suggested more focus on patients with CL/P management in the curricula and more collaboration between centralized CL/P teams and community orthodontists.

Pediatric Mandibular Distraction Osteogenesis and Long-Term Dental Morbidity: A Clinical Evaluation and Review of the Literature

Objectives: Pediatric mandibular distraction osteogenesis (MDO) is an increasingly common surgical procedure used to lengthen the mandible. Little is known about the long-term effects of MDO on dental development. This retrospective study sought to identify dental abnormalities at mixed dentition and early permanent dentition stages in patients who had undergone MDO. Methods: All pediatric patients who underwent MDO with imaging and clinical exams at mixed dentition and early permanent dentition stages were evaluated identify pathology. Data included demographic information, medical and surgical history, complications, and dental exam information. Descriptive statistics were reported. Results: Twenty-two hemimandibles at mixed dentition stage and 24 at early permanent stage were included. Mean age at initial MDO was 7.95 years with a mean follow-up time of 15.1 years. Nine patients required more than 1 distraction. At the mixed dentition stage, all patients showed abnormalities. The most common abnormalities were absent, dysplastic, or ectopic molars. At early permanent dentition stage, 87.5% of patients had dental abnormalities. The most common abnormality was missing molars (third, second, and first), but dysplastic, impacted, and ectopic teeth were also noted. At both mixed and early permanent dentition stages, some patients were noted to have abnormal V-shaped sigmoid notching of the mandibular ramus of undetermined clinical significance. Buccal mucosal scarring that interfered with orthodontic treatment was observed in a subset of patients. Conclusions: This study provides long-term dental analyses of patients undergoing MDO for mandibular hypoplasia. Dental abnormalities were seen at both mixed and early permanent dentition stages. Molars were the most commonly affected with absence the most commonly seen abnormality. Dysplastic, ectopic, and impacted teeth were also seen in posterior and anterior dentition, and abnormalities in buccal soft tissue and the mandibular ramus were also seen. MDO remains a crucial tool for functional and aesthetic improvement in children with hypoplastic mandibles. Dental abnormalities as a consequence of MDO should be considered as part of surgical planning process.

POS1126 PREVALENCE OF HYPOPHOSPHATASIA IN ADULT RHEUMATOLOGY PATIENTS SCREENED BY LOW LEVELS OF ALKALINE PHOSPHATASE

Background:Hypophosphatasia (HPP) is a genetic disease caused by one or more mutations in the alkaline phosphatase (ALP) gene, which encodes tissue-specific ALP and affects the mineralization process. Accordingly, arthralgia, fractures, and dental abnormalities have been reported in adults, and fatal courses in children. This metabolic disorder is commonly misdiagnosed with other more prevalent bone diseases due to its low prevalence and lack of recognition (i.e. chrondrocalcinosis). However, no epidemiological studies on the prevalence of HPP in the rheumatological patient population have been available to date.Objectives:To identify the prevalence of HPP in rheumatological patients screened for persistent low levels of ALP and association with mutations in the ALP gene.Methods:All adult rheumatology patients were screened for pathological low levels of ALP (< 35 IU/L) between January 1, 2017 and June 30, 2019 at the Department of Rheumatology, Clinic of Internal Medicine III, University Hospital Bonn, Germany. Medical files of patients with pathological low ALP levels were then reviewed for clinical signs and symptoms as well as results of genetic testing for HPP (full sequencing using Next Generation Sequencing).Results:In total, 2,289 rheumatology patients were screened for low ALP levels. In 60 patients (2.62 %), pathological low ALP levels were identified, while in 30 of these (1.31 %), persistent low ALP levels were detected. In 19 of the 30 patients, genetic tests for ALPL gene mutations were done. Seven out of 19 patients (36.84 %) had HPP-related symptoms (fracture, dental abnormalities) with normal bone densitometry, while four of these patients (21.05 %) had a history of fracture and three patients (15.78 %) showed dental abnormalities. In addition to the typical HPP signs and symptoms, 13 patients (68.42%) showed mutations in the ALPL gene. One of the ALP mutations was found to be a novel genetic variant, classified as pathological. Interestingly, no association with chondrocalcinosis was detected.Conclusion:In summary, it can be concluded that HPP is an under-diagnosed condition with a higher proportion of affected rheumatologic patients than previously thought (at least 0.56 percent of rheumatologic patients vs. 0,01 percent in a spanish healthy population). If we replicate these numbers for the german population (83 million, 5 percent of whom suffer from rheumatic conditions) the yield is approximately 4.15 million. This possibly indicates that 23,240 potential cases of HPP are currently not diagnosed. Therefore, implementation of a protocol in clinical practice to prevent underdiagnosis of HPP and to treat this disease appropriately is essential.References:[1]García-Fontana C, Villa-Suárez JM, Andújar-Vera F et al (2019) Epidemiological, Clinical and Genetic Study of Hypophosphatasia in A Spanish Population: Identification of Two Novel Mutations in The Alpl Gene. Sci Rep. https://doi.org/10.1038/s41598-019-46004-2[2]Mornet E, Yvard A, Taillandier A et al (2011) A molecular-based estimation of the prevalence of hypophosphatasia in the European population. Ann Hum Genet 75:439–445. https://doi.org/10.1111/j.1469-1809.2011.00642.xFigure 1.Flow chart of identification of potential hypophosphatasia (HPP) patients from an adult rheumatology population. Threshold alkaline phosphatase < 35 IU/L at 37`C. ALP: alkaline phosphataseDisclosure of Interests:Pantelis Karakostas: None declared, Ramona Dolscheid-Pommerich: None declared, Moritz Hass: None declared, Peter Brossart: None declared, Valentin Schäfer Speakers bureau: AbbVie, Novartis, BMS, Chugai, Celgene, Medac, Sanofi, Lilly, Hexal, Pfizer, Janssen, Roche, Schire, Onkowissen, Royal College London, Consultant of: Novartis, Chugai, AbbVie, Celgene, Sanofi, Lilly, Hexal, Pfizer, Amgen, BMS, Roche, Gilead, Medac, Grant/research support from: Novartis, Hexal, Lilly, Roche, Celgene, Universität Bonn.

Sella Turcica Morphology on Cephalometric Radiographs and Dental Abnormalities—Is There Any Association?—Systematic Review

Background: The sella turcica is a saddle-like structure in the middle cranial fossa on the intracranial surface of the sphenoid bone, visible on lateral cephalograms routinely conducted for orthodontic diagnosis. The development of facial structures follows similar traits to the sella turcica: glandular anomalies may be associated with functional disorders, e.g., altered hormonal levels, thus influencing dental development. The aim of this study is to find out if there is any association between the morphology of the sella turcica on cephalometric radiographs and the presence of dental abnormalities. (2) Methods: The search was conducted on 27 January 2021 in four search engines: Medline (PubMed Central), Scopus, Web of Science, Embase. The keywords used in the search strategy were as follows: “sella turcica” AND (“dental abnormalities” OR “dental anomalies” OR “malocclusion”). Since all the studies finally included were retrospective case–control studies, the Newcastle–Ottawa Quality Assessment Form for Case–Control Studies was applied. (3) Results: The search strategy identified 465 articles: 289 from PubMed, 121 from Scopus, 32 from Web of Science and 23 from Embase. Finally, 10 full-text papers were included into qualitative analysis. (4) Conclusions: Sella turcica bridging is very frequent among orthodontic patients. A clear association exists between dental abnormalities and sella turcica bridging.