The Quebec Dental Anomalies Registry: Identifying genes for rare disorders.

There are more than 900 genetic syndromes associated with oral manifestations. These syndromes can have serious health implications, and left undiagnosed, can hamper treatment and prognosis later in life. About 6.67% of the population will develop a rare disease during their lifetime, some of which are difficult to diagnose. The establishment of a data and tissue bank of rare diseases with oral manifestations in Quebec will help medical professionals identify the genes involved, will improve knowledge on the rare genetic diseases, and will also lead to improved patient management. It will also allow samples and information sharing with other clinicians and investigators. As an example of a condition requiring additional research, dental ankylosis is a condition in which the tooth's cementum fuses to the surrounding alveolar bone. This can be secondary to traumatic injury but is often idiopathic, and the genes involved in the idiopathic cases, if any, are poorly known. To date, patients with both identified and unidentified genetic etiology for their dental anomalies were recruited through dental and genetics clinics for the study. They underwent sequencing of selected genes or exome sequencing depending on the manifestation. We recruited 37 patients and we identified pathogenic or likely pathogenic variants in WNT10A, EDAR, AMBN, PLOD1, TSPEAR, PRKAR1A, FAM83H, PRKACB, DLX3, DSPP, BMP2, TGDS. Our project led to the establishment of the Quebec Dental Anomalies Registry, which will help researchers, medical and dental practitioners alike understand the genetics of dental anomalies and facilitate research collaborations into improved standards of care for patients with rare dental anomalies and any accompanying genetic diseases.

Clinical Management of Interproximal and Occlusal Caries in Children and Adolescents by Canadian Dentists: A Survey.

PURPOSE: Early restorative interventions may have important implications in young patients, and the International Caries Classification and Management System strongly recommends non-surgical strategies in the management of dental caries. We aimed to assess management of interproximal and occlusal caries in children and adolescents (≤18 years of age) by Canadian dentists. METHODS: An electronic survey was created and sent to members of Canadian provincial regulatory dental bodies. The survey included 11 questions on demographic factors and 3 clinical situations on dental caries management. RESULTS: The response rate was 4.6% (n = 702). To treat interproximal carious lesions limited to enamel, 442 dentists (63.0%) reported using surgical caries removal on a permanent molar and 502 dentists (71.5%) did the same for a primary tooth. For occlusal carious lesions, the corresponding numbers were 300 dentists (42.7%) for a permanent molar and 269 (38.3%) for a primary molar. Age, year of graduation and province of practice appear to have a significant impact on the restorative threshold. CONCLUSIONS: According to the latest evidence-based recommendations for caries management, the presence of cavitated enamel should be the main indication to restore, and non-surgical interventions for non-cavitated lesions confined to enamel should be prioritized. Results show that a good proportion of respondents have a lower restorative threshold, particularly for interproximal lesions.

Biodentine Pulpotomies on Permanent Traumatized Teeth with Complicated Crown Fractures.

INTRODUCTION: The aim of this multicentric prospective study was to evaluate the clinical and radiographic outcomes of Biodentine (BD) pulpotomies on permanent teeth with complicated crown fractures. METHODS: This study was carried out in 2 hospital-based dental departments in Quebec, Canada. Children seeking emergency care after trauma to anterior permanent teeth were invited to participate in this study. The treatment outcome was assessed clinically and radiographically at 1 week and 1, 3, 6, 12, 18, and 24 months after treatment. RESULTS: Forty-five patients between the ages of 8 and 16 years (mean, 10.8 ± 2.4) were included in this study. Fifty-one teeth were treated with a BD pulpotomy. The study showed a survival rate of 100% and a success rate of 91%. Four failures were noted at 1-, 6-, and 15-month follow-up, and the teeth were subsequently treated with either conventional root canal therapy or regenerative endodontic procedure. Dental sensitivity to cold reported by patients reduced over time, and more teeth responded normally to pulp vitality tests during the course of this study. Slight discoloration was noted on 8 teeth (17%). Radiographic outcomes showed dentinal bridge formation in 91% of cases, and all immature teeth showed continued root formation. CONCLUSIONS: BD is a suitable material for pulpotomies on anterior permanent teeth with complicated crown fractures. It may be a good alternative to mineral trioxide aggregate pulpotomies because it does not cause significant discoloration, and it allows root maturation.

Evaluation of the severity of malocclusions in children affected by osteogenesis imperfecta with the peer assessment rating and discrepancy indexes.

INTRODUCTION: Osteogenesis imperfecta is a heritable disorder affecting bone and tooth development. Malocclusion is frequent in those affected by osteogenesis imperfecta, but this has not been studied in detail. The purpose of this study was to describe and quantify the severity of malocclusions in patients with osteogenesis imperfecta. METHODS: Articulated dental casts were obtained from 49 patients diagnosed with osteogenesis imperfecta (ages 5-19 years; 28 female) and 49 age- and sex-matched control subjects who did not have osteogenesis imperfecta. Both groups were seeking orthodontic treatment. Malocclusions were scored by using the peer assessment rating (PAR) and the discrepancy index (DI). RESULTS: The average United Kingdom weighted PAR scores were 31.1 (SD, 14.5) for the osteogenesis imperfecta group and 22.7 (SD, 10.7) for the control group (P <0.05). The mean United States weighted PAR scores were 32.2 (SD, 15.0) for patients with osteogenesis imperfecta and 21.6 (SD, 9.6) for the controls (P <0.05). The average modified DI scores were 29.8 (SD, 20.2) for the osteogenesis imperfecta group and 12.4 (SD, 6.8) for the control group (P <0.05). Group differences were greatest for lateral open bite (osteogenesis imperfecta group, 7.1; control group, 0.3) for the DI parameters and anterior crossbite (osteogenesis imperfecta group, 13.0; control group, 3.8 [United Kingdom]) for the PAR. CONCLUSIONS: Both the PAR and the DI showed that malocclusions were significantly more severe in patients with osteogenesis imperfecta than in the control group. There was a higher incidence of Class III malocclusion associated with anterior and lateral open bites in patients affected by osteogenesis imperfecta.

Clinical complications in the revascularization of immature necrotic permanent teeth.

The purpose of this case series was to report on the use of a technique of revascularization for necrotic immature permanent teeth, several problems encountered, and solutions to those problems. Eighteen pulp revascularizations were performed in 2009 using the original protocol of revascularization (adapted from the AAE/AAPD joint meeting in 2007 in Chicago). The protocol consisted of opening the canal and disinfecting it with sodium hypochlorite, sealing in a triple antibiotic paste for 2-6 weeks, re-opening, re-irrigating, creating a blood clot in the canal, and sealing with an MTA barrier over the clot. Three problems were encountered during the treatment: (1) bluish discoloration of the crown; (2) failure to produce bleeding; and (3) collapse of the mineral trioxide aggregate (MTA) material into the canal. Modifications to solve these problems included: changing one of the antibiotics, using a local anesthesia without epinephrine, and adding collagen matrix to the blood clot.

Bisphosphonates, osteonecrosis, osteogenesis imperfecta and dental extractions: a case series.

Over the past 4 years, numerous cases of osteonecrosis of the jaw in patients treated with bisphosphonates have been reported. Since 1998, children and adolescents with osteogenesis imperfecta have received bisphosphonates to increase their bone density and reduce the incidence of bone fractures. The results have been convincing, but recent reports of osteonecrosis of the jaw have caused great concern when these patients require dental extractions. The dental records of 15 children and adolescents with osteogenesis imperfecta, involving 60 dental extractions, mostly of primary teeth, done between 2001 and 2006, were reviewed. All patients but one had had or were having bisphosphonate treatment at the time of the extractions. No patient developed osteonecrosis. Further studies and data that allow clinicians to design adequate and safe treatment plans for this unique population are needed.