Predictors of prolonged treatment time intervals in oral cavity cancer.

OBJECTIVES: Delays in treatment time intervals have been associated with overall survival in oral cavity squamous cell carcinoma (OCSCC). The aim of this study was to identify bottlenecks leading to prolonged treatment intervals. MATERIAL AND METHODS: A retrospective analysis was conducted using a cohort of OCSCC patients who underwent surgery and adjuvant radiation therapy. The endpoints of interest were prolonged treatment intervals. Multivariable logistic regression was used to adjust for patient and tumour characteristics. RESULTS: Median diagnosis-to-treatment interval (DTI) and surgery to initiation of postoperative radiation therapy interval (S-PORT) were 39 days (IQR 30-54) and 64 days (IQR 54-66), respectively. Prolonged DTI was associated with older age, worse Charlson Comorbidity index scores and worse T stages. Patients with prolonged DTI had longer times to preoperative imaging reports (25 vs 9 days; P < 0.01). Time to preoperative pathology did not differ. Prolonged S-PORT was associated with longer times to pathology report (28 vs 18 days; P < 0.01), to maxillofacial consult (38 vs 15 days; P < 0.01) and to maxillofacial approval of radiation (50 vs 28 days; P < 0.01). In patients requiring medical oncology consults, those with prolonged S-PORT had longer waiting times until consultation (58 vs 38 days; P = 0.02). Multivariate analysis showed independent predictors of prolonged DTI: time to preoperative imaging; and prolonged S-PORT: time to pathology report, time to maxillofacial consult, and time to medical oncology consult. CONCLUSIONS: Strategies targeting these organizational bottlenecks may be effective for shortening treatment time intervals, hence representing potential opportunities for improving oncological outcomes in OCSCC patients.

Reliability and accuracy of a semi-automatic segmentation protocol of the nasal cavity using cone beam computed tomography in patients with sleep apnea.

OBJECTIVES: The objectives of this study included using the cone beam computed tomography (CBCT) technology to assess: (1) intra- and inter-observer reliability of the volume measurement of the nasal cavity; (2) the accuracy of the segmentation protocol for evaluation of the nasal cavity. MATERIALS AND METHODS: This study used test-retest reliability and accuracy methods within two different population sample groups, from Eastern Asia and North America. Thirty obstructive sleep apnea (OSA) patients were randomly selected from administrative and research oral health data archived at two dental faculties in China and Canada. To assess the reliability of the protocol, two observers performed nasal cavity volume measurement twice with a 10-day interval, using Amira software (v4.1, Visage Imaging Inc., Carlsbad, CA). The accuracy study used a computerized tomography (CT) scan of an OSA patient, who was not included in the study sample, to fabricate an anthropomorphic phantom of the nasal cavity volume with known dimensions (18.9 ml, gold standard). This phantom was scanned using one NewTom 5G (QR systems, Verona, Italy) CBCT scanner. The nasal cavity was segmented based on CBCT images and converted into standard tessellation language (STL) models. The volume of the nasal cavity was measured on the acquired STL models (18.99 ± 0.066 ml). RESULTS: The intra-observer and inter-observer intraclass correlation coefficients for the volume measurement of the nasal cavity were 0.980-0.997 and 0.948-0.992 consecutively. The nasal cavity volume measurement was overestimated by 1.1%-3.1%, compared to the gold standard. CONCLUSIONS: The semi-automatic segmentation protocol of the nasal cavity in patients with sleep apnea and by using cone beam computed tomography is reliable and accurate. CLINICAL RELEVANCE: This study provides a reliable and accurate protocol for segmentation of nasal cavity, which will facilitate the clinician to analyze the images within nasoethmoidal region.

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.

Automatic Pulp and Teeth Three-Dimensional Modeling of Single and Multi-Rooted Teeth Based on Cone-Beam Computed Tomography Imaging: A Promising Approach With Clinical and Therapeutic Outcomes.

Background Cone-beam computed tomography (CBCT) imaging offers high-quality three-dimensional (3D) acquisition with great spatial resolution, given by the use of isometric voxels, when compared with conventional computed tomography (CT). The current literature supports a median reduction of 76% (up to 85% reduction) of patients' radiation exposure when imaged by CBCT versus CT. Clinical applications of CBCT imaging can benefit both medical and dental professions. Because these images are digital, the use of algorithms can facilitate the diagnosis of pathologies and the management of patients. There is pertinence to developing rapid and efficient segmentation of teeth from facial volumes acquired with CBCT. Methodology In this paper, a segmentation algorithm using heuristics based on pulp and teeth anatomy as a pre-personalized model is proposed for both single and multi-rooted teeth. Results A quantitative analysis was performed by comparing the results of the algorithm to a gold standard obtained from manual segmentation using the Dice index, average surface distance (ASD), and Mahalanobis distance (MHD) metrics. Qualitative analysis was also performed between the algorithm and the gold standard of 78 teeth. The Dice index average for all pulp segmentation (n = 78) was 83.82% (SD = 6.54%). ASD for all pulp segmentation (n = 78) was 0.21 mm (SD = 0.34 mm). Pulp segmentation compared with MHD averages was 0.19 mm (SD = 0.21 mm). The results of teeth segmentation metrics were similar to pulp segmentation metrics. For the total teeth (n = 78) included in this study, the Dice index average was 92% (SD = 13.10%), ASD was low at 0.19 mm (SD = 0.15 mm), and MHD was 0.11 mm (SD = 0.09 mm). Despite good quantitative results, the qualitative analysis yielded fair results due to large categories. When compared with existing automatic segmentation methods, our approach enables an effective segmentation for both pulp and teeth. Conclusions Our proposed algorithm for pulp and teeth segmentation yields results that are comparable to those obtained by the state-of-the-art methods in both quantitative and qualitative analysis, thus offering interesting perspectives in many clinical fields of dentistry.

Orofacial Pain and Snoring/Obstructive Sleep Apnea in Individuals with Head and Neck Cancer: A Critical Review.

AIMS: (1) To summarize current knowledge on the prevalence, intensity, and descriptors of orofacial pain and snoring/obstructive sleep apnea (OSA) before and after head and neck cancer (HNC) treatment; and (2) to propose future directions for research. METHODS: The median prevalence for each condition was estimated from the most recent systematic reviews (SRs) and updated with new findings retrieved from the PubMed, Web of Science, Embase, and Cochrane databases up to December 2021. RESULTS: The prevalence of HNC pain seems relatively stable over time, with a median of 31% before treatment in three studies to a median of 39% at 1 month to 16 years after treatment in six studies. HNC pain intensity remains mild to moderate. There was a threefold increase in temporomandibular pain prevalence after surgery (median 7.25% before to 21.3% after). The data for snoring prevalence are unreliable. The OSA/HNC prevalence seems relatively stable over time, with a median of 72% before treatment in three studies to 77% after treatment in 14 studies. CONCLUSION: With the exception of temporomandibular pain, the prevalence of HNC pain and OSA seems to be stable over time. Future studies should: (1) compare the trajectory of change over time according to each treatment; (2) compare individuals with HNC to healthy subjects; (3) use a standardized and comparable method of data collection; and (4) assess tolerance to oral or breathing devices, since HNC individuals may have mucosal sensitivity or pain.

Platelet-rich fibrin as a treatment option for osteoradionecrosis: A literature review.

The aim of this review is to establish the usefulness and effectiveness of using platelet-rich fibrin (PRF) in the treatment of osteoradionecrosis (ORN) lesions. A review of the literature was performed using keywords through the PubMed-Medline and Cochrane Library search engine. Inclusion criteria were: (1) original publication in either the French or the English language, (2) studies conducted in humans, (3) presence of ORN lesions following head and neck radiotherapy (RT), (4) use of PRF or derivates in the treatment of ORN lesions, (5) clinical variables and outcomes mentioned in the study. Overall, four case reports were retained. Two publications were removed from the initial seven results after application of the inclusion criteria. A recent randomised clinical trial was not considered since the group analysed the effectiveness of leukocyte-enriched Plasmas-Rich-Fibrin (LPRF) in preventing ORN, but not in treating it. Therefore, four publications were retained for analysis. Results suggest that using PRF as an adjunct to surgical therapy is beneficial in treating ORN lesions although no controlled studies were found. Therefore, additional controlled clinical studies are warranted to better define the effectiveness and recommendation of this approach.

Comparison of anatomic and aerodynamic characteristics of the upper airway among edentulous mild, moderate, and severe obstructive sleep apnea in older adults.

STUDY OBJECTIVES: First, to compare the upper airway's anatomic and aerodynamic characteristics of the edentulous older adults who experience mild, moderate, and severe obstructive sleep apnea (OSA). Second, to examine the correlation between the severity of OSA and the anatomic and aerodynamic characteristic(s) of the upper airway in these edentulous individuals. METHODS: NewTom5G cone beam computed tomography scans of 58 edentulous individuals with mild, moderate, and severe OSA were included in this analysis. 1) Computational models of the upper airway were reconstructed based on cone beam computed tomography images and the anatomical and aerodynamic characteristics of the upper airway were examined by an observer blind to OSA severity. 2) Pearson correlation analysis was used to determine the correlation between apnea-hypopnea index and the anatomic and aerodynamic characteristics of the upper airway. RESULTS: Compared with edentulous patients with mild and moderate OSA, those with severe OSA have a more hourglass-shaped upper airway. The severity of OSA, namely, apnea-hypopnea index, was significantly correlated with the length, shape, and minimum cross-sectional area of the upper airway. During inspiration, the mean velocity of the airflow within the upper airway of the edentulous patients with severe OSA was higher than that of patients with mild and moderate OSA. During both inspiration and expiration, apnea-hypopnea index was found to be significantly correlated with maximum velocity (P = .05) and airway resistance (P = .024, 0.038). CONCLUSIONS: The edentulous patients with severe OSA have a more hourglass-shaped upper airway. The findings also suggest that, during inspiration, the airflow travels faster in edentulous patients with severe OSA than in those with mild or moderate OSA. CLINICAL TRIAL REGISTRATION: Registry: ClinicalTrials.gov; Name: The Effect of Nocturnal Wear of Dentures on Sleep and Oral Health Related Quality of Life; URL: https://clinicaltrials.gov/ct2/show/NCT01868295; Identifier: NCT01868295. CITATION: Chen H, Elham E, Li Y, et al. Comparison of anatomic and aerodynamic characteristics of the upper airway among edentulous mild, moderate, and severe obstructive sleep apnea in older adults. J Clin Sleep Med. 2022;18(3):759-768.

Do postoperative antibiotics influence one-year peri-implant crestal bone remodelling and morbidity? A double-blinded randomized clinical trial.

OBJECTIVES: The primary objective of this study was to assess whether giving postoperative antibiotics to healthy patients after straightforward platform-switched implant placement would influence peri-implant crestal bone levels and postoperative morbidity after 1 year. METHODS: Thirty-eight healthy individuals were recruited in this pilot, randomized, double-blinded, placebo-controlled clinical trial. The intervention group (n = 18) received two grams of amoxicillin one hour before implant placement followed by a 7 days postoperative regimen (500 mg tid). The control group (n = 20) took the same preoperative dose of amoxicillin and an identical placebo postoperatively. Mesial and distal peri-implant crestal bone levels were measured at baseline, four months and one year later with standardized periapical radiographs. Postoperative pain severity was assessed through self-administered questionnaires for 7 days. Surgery-associated morbidities were evaluated after one, three, 16 weeks and 1 year. Descriptive and bivariate analyses were used. RESULTS: Thirty-seven participants completed the trial. At the one-year follow-up, the mean combined peri-implant crestal bone changes for the intervention (n = 18) and control (n = 19) groups were - 0.44 ± 0.41 mm and - 0.27 ± 0.56 mm, respectively. The difference between the groups (intervention-control) for mean combined crestal bone level changes was not statistically significant. There were no significant differences in surgery-associated morbidities between the intervention and control groups. The one-year implant survival rate was 100% in both groups. CONCLUSIONS: Study results suggest that a routine postoperative antibiotic regimen for healthy patients undergoing straightforward platform-switched implant placement might not be necessary to prevent postoperative peri-implant bone loss and complications.

Impact of piezocision-assisted orthodontics on root resorption and alveolar bone: a prospective observational study.

OBJECTIVES: To assess the impact of orthodontic treatment combined with piezocision (OT-PC) on root structure and alveolar bone. MATERIALS AND METHODS: Twelve adults were treated with OT-PC. Pre- and post-treatment CBCT examinations evaluated apical root resorption (ARR) and alveolar bone height and thickness changes. Pre- and post-treatment differences were compared using one-sample t test and Wilcoxon signed-rank test. RESULTS: ARRs were generalized and significantly more severe in both anterior sextants compared with posterior sextants. Bone thickness decreased significantly in the maxilla at mid-root and apex areas. The majority of mandibular alveolar bone dehiscences occurred on the buccal aspect at the mid-root level, especially where thickness was less than 0.3 mm. Overall bone height decreased twice as much on the buccal aspect (1.43 mm, P < 0.001) compared with the lingual aspect (0.67 mm, P = 0.001), most significantly in the lower incisors, where the average median loss was 2.10 mm (P = 0.003). CONCLUSION: OT-PC causes minor negative effects on both alveolar bone and root resorption. CLINICAL RELEVANCE: Orthodontic treatment combined with piezocision causes minor negative effects on periodontal support. Nevertheless, mild bone height loss on the buccal aspect of the mandibular teeth and root resorption in both anterior sextants have been detected with this new treatment.

Potential of iterative reconstruction for maxillofacial cone beam CT imaging: technical note.

Iterative reconstruction has been proven to be an effective tool for low-dose computed tomography imaging. However, this technology is currently not available in commercial diagnostic maxillofacial cone beam CT. For this technical note, an iterative reconstruction technique was applied to cone beam CT raw data of two maxillofacial clinical cases to explore its potential for dose reduction and metal artifact reduction. Low-dose imaging was emulated by using only fractions of the clinical projection dataset. The reconstruction algorithms tested were filtered backprojection (FBP) as a reference method, and a total variation minimization (TV) regularized ordered subsets convex (OSC-TV) method as the iterative technique. Upon qualitative examination, the OSC-TV technique was found to conserve most diagnostic information using half the projections. Test images have also shown that at 1/4 of the projections, OSC-TV was more robust than FBP with respect to streaking and metal artifacts.

Iterative reconstruction for image enhancement and dose reduction in diagnostic cone beam CT imaging.

BACKGROUND: Iterative reconstruction is well-established in diagnostic multidetector computed tomography (MDCT) for dose reduction and image quality enhancement. Its application to diagnostic cone beam computed tomography (CBCT) is only emerging and warrants a quantitative evaluation. METHODS: Several phantoms and a canine head specimen were imaged using a commercially available small-field CBCT scanner. Raw projection data were reconstructed using the Feldkamp-Davis-Kress (FDK) method with different filters, including denoising via total variation (TV) minimization (FDK-TV). Iterative reconstruction was carried out using the TV-regularized ordered subsets convex technique (OSC-TV). Signal-to-noise ratio (SNR), noise power spectrum (NPS) and spatial resolution of images were estimated. Dose levels were measured via the weighted computed tomography dose index, while low-dose image quality degradation was estimated via structural similarity (SSIM). RESULTS: OSC-TV and FDK-TV were shown to significantly improve image signal-to-noise ratio (SNR) compared to FDK with a standard filter, 5.8 and 4.0 times, respectively. Spatial resolution attained with different algorithms varied moderately across different experiments. For low-dose acquisitions, image quality decreased dramatically for FDK but not for FDK-TV nor OSC-TV. For low-dose canine head images acquired using about 1/5 of the dose compared to a reference image, SSIM dropped to about 0.3 for FDK, while remaining at 0.92 for FDK-TV and 0.96 for OSC-TV. CONCLUSION: OSC-TV was shown to improve image quality compared to FDK and FDK-TV. Moreover, this iterative approach allowed for significant dose reduction while maintaining image quality.

Piezocorticision-assisted orthodontics: Efficiency, safety, and long-term evaluation of the inflammatory process.

OBJECTIVES: The aim of this work was to compare the duration of treatment between orthodontic treatment combined with piezocorticision (OT-PC) and conventional orthodontic treatment (COT), as well as to evaluate the safety, inflammatory process, periodontal health, and soft tissue healing in the OT-PC group. METHODS: Twelve patients were included in each group, and their treatment times were compared for preliminary bracket alignment (PBA) and for overall treatment. In the OT-PC group, the inflammatory process was evaluated by quantifying cytokines in the gingival crevicular fluid. A calibrated examiner measured the probing depth (PD), the distance between the gingival margin and the cementoenamel junction (GM-CEJ), and the clinical attachment level (CAL), before and after OT-PC. The presence of gingival scars was evaluated. Bone and root injuries were assessed with the use of cone-beam computed tomography. RESULTS: The treatment time was significantly reduced in the OT-PC group for PBA in both maxilla (45%; P = 0.001) and mandible (31%; P = 0.023), as well as for overall treatment (52%; P < 0.0001). Although not statistically significant, several inflammatory mediators demonstrated peaks at 3-5 and 16 weeks. There were not significant changes in PD and GM-CEJ after OT-PC treatment, unlike CAL (0.09 ± 0.12 mm; P = 0.024); 47.5% of piezocorticisions caused gingival scars. Only one patient showed no scars. Four cortical bones did not heal completely, and 2 roots had piezoelectric injuries. CONCLUSION: OT-PC was effective at reducing the orthodontic treatment time.

Decision criteria influencing the therapeutic approach to invasive cervical resorption: a case series.

Invasive cervical resorption (ICR) is a dental lesion starting in the cervical region and involving the loss of dental hard tissue as a result of odontoclastic action. Due to its localization and invasive pattern, this process represents a challenging clinical situation. When feasible, the major aim of an ICR treatment is to completely remove the pathologic tissue (specifically at the entry point of the lesion) and to seal the resulting defect, without compromising tooth rehabilitation. In this context, choosing how to access the resorptive lacuna is essential. Two main options have been described in the literature: an external approach, requiring the surgical exposure of the resorptive lacuna, and an internal approach, taking advantage of the endodontic access cavity. However, there are no guidelines that indicate which approach to choose for the treatment of an ICR. This article is based on four clinical cases. It aims to provide specific clinical and radiologic features that should be considered in order to take the most appropriate decision, when choosing between the internal and the external approaches. It is proposed to base the therapeutic strategy on the accessibility and the size of the portal of entry of the lesion. When the entry point is wide, its extension along the root must also be taken into account. Other important parameters are the circumferential and vertical extents of the lesion in the radicular dentin. Although it is not a determining factor, the pulpal involvement of the lesion can also be considered.

Pre-irradiation dental care: Ready-to-use templates for oropharyngeal cancers.

AIM: To develop a tool in order to guide pre-irradiation dental care (PIDC) for patients with oropharyngeal cancers. BACKGROUND: Osteoradionecrosis of the jaws is a potential complication of radiotherapy (RT) for head and neck cancers. To prevent this complication, PIDC can involve multiple dental extractions as a preventative measure to avoid post-RT complications. However, there is no standardized tool to guide PIDC. MATERIALS AND METHODS: From January 2005 to October 2015, 120 head and neck cancer patients were prospectively included in a study investigating dysgeusia after RT. From this cohort, patients were enrolled according to the following inclusion criteria: histopathological confirmation of oropharyngeal squamous cell carcinoma; stage T1-4 N1-3 M0; ≤10 missing teeth. Individual teeth were retrospectively delineated on planning computed tomography and doses to dentition were assessed to generate templates. RESULTS: Thirty-three patients were included. Molars received highest doses with a mean dose of 50 Gy (range; 19-75 Gy). Ipsi-lateral and contralateral wisdom teeth received RT dose superior to 50 Gy in 92% and 56% of cases, respectively. Patients with advanced disease (T4 or N2c-3) received higher mean doses on inferior and ipsi-lateral dental arches compared to other patients (T1-3 N0-2b): 42 Gy vs. 39 Gy and 44 Gy vs. 39 Gy (p < 0.05), respectively. CONCLUSION: Pre-RT dose distribution templates are an objective way to prepare PIDC. Further studies with a larger cohort are needed to validate these templates.

Dento-maxillo-facial phenotype and implants-based oral rehabilitation in Ectodermal Dysplasia with WNT10A gene mutation: report of a case and literature review.

PURPOSE: To report the dento-craniofacial phenotype of a family affected by a WNT10A HED and to describe the implant-based oral rehabilitation of a patient presenting a severe oligodontia linked to this mutation. A molecular hypothesis concerning the involvement of Wnt-ß-catenin pathway in implant osteointegration will be proposed. MATERIAL AND METHODS: Patients affected by a WNT10A mutation were included from a large group of HED patients. WNT10A gene was sequenced in second intention for patients negative for EDA-EDAR-EDARADD mutations. Dento-craniofacial phenotype was described based on clinical and radiological data. RESULTS: Severe oligodontia was observed in the patient affected by a compound heterozygous mutation of WNT10A gene. CT exams showed marked maxillary bone hypoplasia in the posterior areas with a sub-normal mandible treatment consisted in the placement of 4 mandibular implants and in 2 implant-supported bridges. In the maxilla, an autogenous bone graft was indicated. The post-operative radiological follow-up showed partial bone resorption of the grafts, treated with ramus bone shaving and a membrane, followed by the placement of 4 maxillary implants. CONCLUSION: Patients affected by WNT10A HED require multi-disciplinary dental diagnosis and treatment. A close post-operative radiological follow-up appears necessary given the biological functions of Wnt-ß-catenin in bone repair.

RSK2 is a modulator of craniofacial development.

BACKGROUND: The RSK2 gene is responsible for Coffin-Lowry syndrome, an X-linked dominant genetic disorder causing mental retardation, skeletal growth delays, with craniofacial and digital abnormalities typically associated with this syndrome. Craniofacial and dental anomalies encountered in this rare disease have been poorly characterized. METHODOLOGY/PRINCIPAL FINDINGS: We examined, using X-Ray microtomographic analysis, the variable craniofacial dysmorphism and dental anomalies present in Rsk2 knockout mice, a model of Coffin-Lowry syndrome, as well as in triple Rsk1,2,3 knockout mutants. We report Rsk mutation produces surpernumerary teeth midline/mesial to the first molar. This highly penetrant phenotype recapitulates more ancestral tooth structures lost with evolution. Most likely this leads to a reduction of the maxillary diastema. Abnormalities of molar shape were generally restricted to the mesial part of both upper and lower first molars (M1). Expression analysis of the four Rsk genes (Rsk1, 2, 3 and 4) was performed at various stages of odontogenesis in wild-type (WT) mice. Rsk2 is expressed in the mesenchymal, neural crest-derived compartment, correlating with proliferative areas of the developing teeth. This is consistent with RSK2 functioning in cell cycle control and growth regulation, functions potentially responsible for severe dental phenotypes. To uncover molecular pathways involved in the etiology of these defects, we performed a comparative transcriptomic (DNA microarray) analysis of mandibular wild-type versus Rsk2-/Y molars. We further demonstrated a misregulation of several critical genes, using a Rsk2 shRNA knock-down strategy in molar tooth germs cultured in vitro. CONCLUSIONS: This study reveals RSK2 regulates craniofacial development including tooth development and patterning via novel transcriptional targets.

A possible cranio-oro-facial phenotype in Cockayne syndrome.

BACKGROUND: Cockayne Syndrome CS (Type A - CSA; or CS Type I OMIM #216400) (Type B - CSB; or CS Type II OMIM #133540) is a rare autosomal recessive neurological disease caused by defects in DNA repair characterized by progressive cachectic dwarfism, progressive intellectual disability with cerebral leukodystrophy, microcephaly, progressive pigmentary retinopathy, sensorineural deafness photosensitivity and possibly orofacial and dental anomalies. METHODS: We studied the cranio-oro-facial status of a group of 17 CS patients from 15 families participating in the National Hospital Program for Clinical Research (PHRC) 2005 « Clinical and molecular study of Cockayne syndrome ¼. All patients were examined by two investigators using the Diagnosing Dental Defects Database (D[4]/phenodent) record form. RESULTS: Various oro-facial and dental anomalies were found: retrognathia; micrognathia; high- arched narrow palate; tooth crowding; hypodontia (missing permanent lateral incisor, second premolars or molars), screwdriver shaped incisors, microdontia, radiculomegaly, and enamel hypoplasia. Eruption was usually normal. Dental caries was associated with enamel defects, a high sugar/carbohydrate soft food diet, poor oral hygiene and dry mouth. Cephalometric analysis revealed mid-face hypoplasia, a small retroposed mandible and hypo-development of the skull. CONCLUSION: CS patients may have associated oro-dental features, some of which may be more frequent in CS children - some of them being described for the first time in this paper (agenesis of second permanent molars and radiculomegaly). The high susceptibility to rampant caries is related to a combination of factors as well as enamel developmental defects. Specific attention to these anomalies may contribute to diagnosis and help plan management.

Previously undescribed pulpal and periodontal ligament calcifications in systemic sclerosis: a case report.

Systemic sclerosis (SSc), a multisystem autoimmune disease characterized by widespread fibrosis, vascular alterations, autoimmunity, and inflammation, has effects on the hard and soft tissues of the orofacial region. The most common oral radiographic features correspond to widening of the periodontal ligament space and to mandibular resorption. In this report, cone-beam computerized tomography (CBCT) confirmed not only the well described periodontal features associated with SSc but also revealed previously undescribed calcifications within the periodontal ligament space of most maxillary teeth. Moreover, CBCT showed pulp calcifications in some incisors and premolars with these calcifications leading to root canal obliterations. Such manifestations (which could be linked to different major pathogenic features of SSc such as calcinosis, vasculopathy, and fibrosis) contribute to the phenotypic spectrum of the disease.