OBJECTIVE:Unilateral craniofacial microsomia (UCM) presents varying unilateral deficiencies of the temporomandibular joint, mandibular ramus, masticatory muscles, and adjacent soft tissues and ear. Reduced mandibular and maxillary dimensions may decrease upper airway volume and may increase the risk of sleep-disordered breathing (SDB). This retrospective pilot study aimed to evaluate a novel three-dimensional (3D) method for assessing nasal cavity (NC) and pharyngeal airway (PA) dimensions in growing patients with UCM compared with healthy controls, and to explore the potential associations between airway dimensions and SDB risk using the pediatric Sleep Questionnaire (PSQ). MATERIAL AND METHODS:This retrospective case-control pilot study analysed Cone-beam computed tomography (CBCT) scans from 11 patients with UCM (mean age 12.7 ± 4.4 years) and 11 age-matched controls (mean age 12.1 ± 1.1 years). Two calibrated investigators performed airway measurements, including total and partial NC and PA volumes, minimal cross-sectional area (MCS), and hydraulic diameter. SDB risk in the UCM group was assessed using PSQ scores. Reliability was evaluated with intra-class correlation coefficients and Dahlberg's formula. Group differences were tested using unpaired t-tests. RESULTS:Patients with UCM showed significantly smaller NC volume (9714.9 ± 3032 mm3) than controls (12773.9 ± 1963 mm3; p < 0.0001). PA volume and MCS did not differ significantly. Four of the eleven patients with UCM showed a high PSQ-based risk of SDB and tended to have smaller airway dimensions. Measurement reliability was acceptable. CONCLUSIONS:CBCT-based 3D assessment of upper airway morphology was feasible in patients with UCM. Patients with UCM demonstrated significantly reduced nasal cavity volume compared with controls. Exploratory analysis suggested that some patients may be at increased risk of SDB; however, these findings require confirmation in larger studies.

ObjectiveThis prospective study investigates the efficacy of biologics in combination with methotrexate (MTX) or leflunomide (LEF) on juvenile idiopathic arthritis (JIA)-related temporomandibular joint (TMJ) arthritis measured by magnetic resonance imaging (MRI)-based inflammation score and deformity score.MethodsA prospective, single-center observational cohort study of 18 consecutive patients was performed between September 2018 and April 2023. Inclusion criteria were (1) diagnosis of JIA, (2) MRI-verified TMJ arthritis leading to treatment with tumor necrosis factor inhibitor (TNFi), (3) MRI at 6 months and 24 months after treatment initiation, and (4) clinical follow-up together with an MRI by a pediatric rheumatologist and an orthodontist.ResultsWe included 18 patients (89% female). At the time of the first MRI, median age was 13.2 years (IQR 9.9-17.4), median disease duration was 7.8 years (IQR 3.4-11.1), and 4 received MTX or LEF. During the observation period, significant improvements were observed in TMJ movement pain (P= 0.01), morning stiffness (P= 0.004), opening capacity (P= 0.03), and maximal incisal openingP= 0.006). The inflammation score decreased significantly from a median of 2 (IQR 1-3) at baseline to a median of 1 (IQR 0-2) at 24 months (P= 0.009). In 17 of 36 TMJs (47%), the deformity score improved or remained stable and no significant increase in the median score was observed.ConclusionThis is the first prospective, observational study with evidence to support that the orofacial signs, symptoms, and MRI-derived inflammation score in TMJ arthritis can be reduced by treatment with TNFi.

This investigation aimed to develop a radiographic 3D cephalometric index to grade severity of dentofacial deformity in patients with juvenile idiopathic arthritis (JIA), and to perform a validation against expert evaluations. Data were collected from a population-based Nordic JIA cohort of 240 patients that received a cone-beam computed tomography (CBCT) scan approximately 17 years after onset of JIA. The cohort was randomized into two groups: A baseline group for establishing the index (n = 210) and a test group (n = 30). The standardized index generation process led to the inclusion of six radiographic outcome measures, each representing different facets of dentofacial deformities. Specific severity scores were established for three domains; dentofacial asymmetry (0-8), vertical deviation (0-8), and sagittal deviation (0-8). An overall dentofacial deformity index score was computed by aggregating the scores from the six variables, with the total score ranging from 0 to 24. The validity of the index was assessed by comparing the index score with expert evaluations. Based on the generated index, 10 patients exhibited severe deformities. Reliable agreement between data-generated index scores and expert assessments was found. A 3D radiographic dentofacial deformity index was generated and tested. This index can support an objective assessment of the severity of dentofacial deformities related to JIA in a clinical setting and future research.

Dentofacial deformity following juvenile idiopathic arthritis (JIA) with temporomandibular joint (TMJ) involvement is associated with functional, aesthetic, and psychosocial impairment. Corrective surgical treatment includes combinations of orthognathic surgeries (OS). The aims of this study were to assess orofacial symptoms, functional and aesthetic status, and stability after OS including mandibular distraction osteogenesis (MDO). A prospective study was conducted of 32 patients with JIA of the TMJ and dentofacial deformities who underwent MDO as the only surgery or in combination with bilateral sagittal split osteotomy, Le Fort I, and/or genioplastybetween 2003 and 2018. Data from clinical examinations and cephalograms performed pre- and postoperative and at long-term (mean 4 years) were analysed. Patients experienced unchanged orofacial symptoms (all P > 0.05), short-term TMJ functional impairment (all P < 0.001), and long-term morphological improvements in SNB angle (P < 0.001), anterior facial height (P < 0.001), mandibular length (P = 0.049), overjet (P < 0.001 and P = 0.005), and posterior facial symmetry (P = 0.046). MDO as the only surgery or with secondary adjunctive OS improved dentofacial morphology in terms of mandibular advancement, anterior facial height, posterior facial symmetry, and incisal relationships without long-term deterioration in TMJ function or orofacial symptoms.

Juvenile idiopathic arthritis (JIA) commonly affects the temporomandibular joint (TMJ), leading to dentofacial deformities and orofacial symptoms. Timely diagnosis and treatment initiation are essential for optimizing patient outcomes. However, clinical examination—the primary screening method— has limited accuracy, increasing the risk of delayed TMJ involvement detection. This study develops, trains and tests an artificial intelligence (AI) model for predicting TMJ involvement in newly diagnosed JIA patients and compares its model performance with expert clinician assessments for validation. A longitudinal dataset of 6,153 standardized orofacial examinations from 1,054 patients with JIA was used to train an Extreme Gradient Boosting (XGBoost) model to predict TMJ involvement. An independent cohort of 55 newly diagnosed patients was used to evaluate the model. Twenty-six clinically relevant features were selected and preprocessed for model input. Model performance was evaluated based on classification accuracy and concordance with expert clinician assessments. Model interpretability was analysed using Shapley additive explanations (SHAP) to identify key predictive features. The XGBoost model achieved an overall accuracy of 85.5% in predicting TMJ involvement. Model predictions showed significant concordance with expert clinician assessments (p < 0.001), although the model identified a higher prevalence of TMJ involvement than experts. The most influential predictive features were reduced condylar translation, facial asymmetry, protrusion, patient-reported orofacial pain and reduced mouth-opening capacity. The developed AI model demonstrates strong predictive performance for TMJ involvement based on clinical examination. By facilitating earlier detection, the model has the potential to support clinical decision-making, enable timely intervention, and improve patient outcomes.

Technological advancements have made the integration of clear aligner therapy (CAT) with orthognathic surgery a reality. This case report outlines an innovative, patient-specific workflow that merges CAT with the surgery first orthognathic approach (SFOA), developed in collaboration with engineers to facilitate in-house production of surgical guides and pre-adapted titanium plates. Objective evaluation was performed using the peer assessment rating (PAR) index. The patient achieved the intended occlusal relationship without discomfort in the temporomandibular joint (TMJ) and experienced no surgical complications. The surgical and occlusal outcomes have remained stable with reduced scores in the PAR index, reflecting improved objective assessment post treatment. This case report is the first of its kind to describe a single-jaw, multi-segmented maxillary osteotomy in SFOA using CAT. It demonstrates precise individualized planning in a multidisciplinary approach integrating CAT with SFOA making it feasible in cases involving a single jaw segmented Le Fort 1 osteotomy.

OBJECTIVES:To describe the clinical and radiographic oro-dental characteristics of patients with pycnodysostosis (PDO). MATERIALS & METHODS:A short interview and clinical examination of seven patients with PDO were performed as well as assessment of the temporomandibular joints and masticatory muscles using the diagnostic criteria for temporomandibular disorders, DC-TMD form. A full set of records were taken including photos and intraoral scan. Finally, existing cone beam computed tomography (CBCT) images and radiographs were also studied. RESULTS:All patients presented with bimaxillary micrognathia, five had a convex profile, and two had a straight profile. In addition, posterior open bite, Angle Class III molar relation with accompanying anterior crossbite and a grooved median palate were common findings. No patient showed symptoms of temporomandibular disorder (TMD) apart from some clicking. Finally, the main radiographic findings were the obtuse mandibular angle, the frontal bossing, the elongation of the coronoid/condylar process and the presence of hypercementosis with obliterated pulp chambers. CONCLUSION:The examined patients with PDO were characterized by dental crowding, malocclusion (anterior crossbite, posterior open bite), hypercementosis, obliterated pulp chambers and deviations in mandibular morphology. In conclusion, patients with PDO have a specific need for dental and orthodontic monitoring with focus on crowding and posterior open bite. The patients will benefit from a long-term orthodontic plan including extractions.

BACKGROUND:An update on the knowledge regarding the orthopedic/orthodontic role in treating JIA-related dentofacial deformities is relevant.OBJECTIVES:This systematic review aimed to assess the level of evidence regarding the management of dentofacial deformity from juvenile idiopathic arthritis (JIA) with orthodontics and/or dentofacial orthopedics.SEARCH METHODS:The following databases were searched without time or language restrictions up to 31 January 2024 (Medline, Embase, Cochrane Central Register of Controlled Trials, Scopus, Web of Science, and Latin American and Caribbean Health Sciences Literature).SELECTION CRITERIA:Inclusion criteria were studies dealing with JIA subjects receiving treatment with orthodontic and/or dentofacial orthopedic functional appliances.DATA COLLECTION AND ANALYSIS:After the removal of duplicate studies, data extraction, and risk of bias assessment according to ROBINS-I guidelines were conducted. Data extraction was conducted by two independent authors.RESULTS:The electronic database search identified 397 eligible articles after the removal of duplicates. Following the application of the pre-defined inclusion and exclusion criteria, 11 articles were left for inclusion. Two trials were associated with a severe risk of bias, four trials were at moderate risk of bias, and the other five presented a low risk of bias. Various research groups employed and documented the effects of different types of appliances and methodologies. The study heterogeneity did not allow for meta-analyses. In addition, a lack of uniformity in treatment objectives was observed across the included studies. After treatment with dentofacial orthopedics skeletal improvement was demonstrated in 10 studies, and a decrease in orofacial signs and symptoms was reported in 7 studies.CONCLUSIONS:Across the available literature, there is minor evidence to suggest that dentofacial orthopedics may be beneficial in the management of dentofacial deformities from JIA. There is little evidence to suggest that it can reduce orofacial signs and symptoms in patients with JIA. Based on current evidence, it is not possible to outline clinical recommendations for specific aspects of orthopedic management in growing subjects with JIA-related dentofacial deformity.REGISTRATION:PROSPERO (CRD42023390746).

Continuous advancements in technology have made it possible to integrate clear aligner therapy (CAT) with orthognathic surgery. This case report presents a novel, individually-planned workflow, combining CAT with a surgery-first orthognathic approach (SFOA) in collaborating with engineers for an in-house production of surgical guides and customized titanium plates. The patient was evaluated subjectively, using the Oral Health-Related Quality of Life-14 (OHIP-14) questionnaire and Orthognathic Quality of Life questionnaire (OQLQ), and objectively with the Peer Assessment Rating (PAR) index. The patient displayed the planned occlusal relationship with no report of discomfort in the temporomandibular joint (TMJ) or post-surgical complications. The surgical and occlusal outcomes have remained consistent and stable after debonding. A decreased score was reported in both questionnaires and the PAR after treatment, thereby indicating improvements in both subjective and objective evaluations. This case report demonstrates that with proper individual planning, satisfactory subjective and objective outcomes can be achieved when combining SFOA with CAT.

OBJECTIVE:To assess the agreement between child- and parent-reported orofacial symptoms in the Danish version of the patient questionnaire Assessment of Orofacial Symptoms in Juvenile Idiopathic Arthritis. METHOD:This cross-sectional study was conducted at Aarhus University in March 2023. Eligible candidates were consecutive subjects with juvenile idiopathic arthritis (JIA) and temporomandibular joint involvement accompanied by a parental proxy for examination in the Craniofacial Clinic. After obtaining written informed consent, the questionnaire was completed individually and separately by the child and the parent without any communication between them. The level of agreement was analysed using Cohen's (weighted) kappa for nominal and ordinal outcome variables (orofacial pain frequency, pain location, jaw function, orofacial symptoms, and changes since last visit) and the intraclass correlation coefficient for linear outcome variables (orofacial pain intensity and functional disability of the jaw). RESULTS:The 34 included dyads had an overall 'poor' to 'moderate' child-proxy reporting agreement on the questionnaire for the assessment of JIA-related orofacial symptoms. After dividing the children into two age groups, < 13 and ≥ 13 years old, we found substantial agreement on pain frequency and moderate to excellent agreement on pain intensity for the older group. The child-proxy agreement for children aged < 13 years was slight on pain frequency and poor to moderate on pain intensity. CONCLUSION:The child-proxy reporting agreement on JIA-related orofacial symptoms is inconsistent. We suggest collecting information from both children and parents, especially when assessing orofacial pain and symptoms in children < 13 years of age.

BACKGROUND:Juvenile idiopathic arthritis (JIA) frequently affects the temporomandibular joint (TMJ), which can alter mandibular growth and development and result in dentofacial deformities.OBJECTIVE:To assess the outcomes of orthopedic treatment with distraction splint (DS) in patients with JIA-related dentofacial deformity.METHODS:The retrospective study involved 30 patients with JIA and unilateral TMJ involvement, another study group of 20 patients with JIA and bilateral TMJ involvement, and a control group of 18 non-JIA orthodontic patients with Class II and III malocclusions. The inclusion criteria were DS treatment and cone-beam computed tomography (CBCT) scans before (T0) and 2 years after treatment (T1). Dentofacial morphology and deformity were evaluated based on a validated three-dimensional CBCT-based morphometric analysis. Intergroup differences in outcome measures were compared at T0 and T1, and intragroup changes between T0 and T1 were assessed using the Kruskal-Wallis test.RESULTS:Initial evaluations at T0 revealed significant differences between the unilateral and bilateral JIA groups and the control group for three out of eight dentofacial deformity variables: inter-side difference in total posterior mandibular height, mandibular axial angle, and posterior/anterior face height (ratio). At follow-up (T1), significant inter-group differences were only observed in total posterior mandibular height indicating that intergroup differences were less pronounced after splint treatment. Assessing inter-group changes between T0 and T1 showed that all parameters remained constant except posterior/anterior face height ratio, which significantly decreased between T0 and T1.CONCLUSIONS:The findings demonstrate the potential of DS treatment for patients with JIA and unilateral or bilateral TMJ involvement to generally support normal dentofacial growth or at least limit further deterioration of dentofacial deformities.

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease during childhood and adolescence. The temporomandibular joints (TMJ) are among the most frequently affected joints in patients with JIA, and mandibular growth is especially vulnerable to arthritic changes of the TMJ in children. A clinical examination is the most cost-effective method to diagnose TMJ involvement, but clinicians find it difficult to interpret and inaccurate when used only on clinical examinations. This study implemented an explainable artificial intelligence (AI) model that can help clinicians assess TMJ involvement. The classification model was trained using Random Forest on 6154 clinical examinations of 1035 pediatric patients (67% female, 33% male) and evaluated on its ability to correctly classify TMJ involvement or not on a separate test set. Most notably, the results show that the model can classify patients within two years of their first examination as having TMJ involvement with a precision of 0.86 and a sensitivity of 0.7. The results show promise for an AI model in the assessment of TMJ involvement in children and as a decision support tool.

Recent technological innovations allow for the integration of clear aligner therapy (CAT) with orthognathic surgical procedures, making the surgery-first orthognathic approach (SFOA) a reality. Combining CAT with this surgery-first strategy offers a compelling solution for both patients and orthodontists seeking a faster and less visible treatment. Although the combination of CAT and SFOA presents potential advantages for practitioners in orthognathic surgery, it also introduces new challenges. A significant obstacle is the effective transfer of the virtual surgical planned occlusion and jaw relations into the aligner software to allow for aligner manufacturing prior to surgery. To date, no protocol has described effective transfers of the virtual surgical plan (VSP) to the CAT software. This article presents a novel method for this transfer, utilizing the freely available software Meshmixer 3.5 (Autodesk, Armonk, NY) in two patient scenarios.

Aim of the study: This article introduces an in-house workflow implementing patient-specific surgical cutting guides and spacers combined with pre-adapted osteosynthesis in two Surgery First Orthognathic Approach (SFOA) scenarios. Background: The integration of three-dimensional virtual surgical planning (VSP) in combination with computer aided design and computer aided manufacturing (CAD/CAM) has advanced the field of orthognathic surgery. With the use of surgical guides and patient specific implants (PSI) the accuracy of the surgical outcome could potentially increase. While PSI approaches offer advantages, commercial PSIs are characterized by a prolonged turnaround time and high costs.These drawbacks linked with commercial PSIs might restrict their utilization and acceptance. Technical note: The VSP was completed in Dolphin Imaging software. 3-Matic software was used to design the cutting guides and spacers. The anterior spacers where specifically designed to utilize the wedge-technique during positioning of the anterior segment in segmented Le Fort I osteotomy. The surgical guides and spacers were manufactured in Form 3B 3D printer with BioMed Clear V1 Resin with 0.100 mm layer thickness. Titanium plates 2.0 from Biomet were customized based on a 3D-model of the planned postsurgical outcome based on the VSP. Conclusion: The in-house methodology for developing and employing personalized surgical guides, spacers and pre-adapted titanium plates offers a viable workflow for SFOA, with the potential to enhance the reliability and precision of maxillary repositioning.

Juvenile idiopathic arthritis (JIA) is the most common inflammatory rheumatic disease of childhood. JIA can affect any joint and the temporomandibular joint (TMJ) is one of the joints most frequently involved. TMJ arthritis impacts mandibular growth and development and can result in skeletal deformity (convex profile and facial asymmetry), and malocclusion. Furthermore, when TMJs are affected, patients may present with pain at joint and masticatory muscles and dysfunction with crepitus and limited jaw movement. This review aims to describe the role of orthodontists in the management of patients with JIA and TMJ involvement. This article is an overview of evidence for the diagnosis and treatment of patients with JIA and TMJ involvement. Screening for the orofacial manifestation of JIA is important for orthodontists to identify TMJ involvement and related dentofacial deformity. The treatment protocol of JIA with TMJ involvement requires an interdisciplinary collaboration including orthopaedic/orthodontic treatment and surgical interventions for the management of growth disturbances. Orthodontists are also involved in the management of orofacial signs and symptoms; behavioural therapy, physiotherapy and occlusal splints are the suggested treatments. Patients with TMJ arthritis require specific expertise from an interdisciplinary team with members knowledgeable in JIA care. Since disorders of mandibular growth often appear during childhood, the orthodontist could be the first clinician to see the patient and can play a crucial role in the diagnosis and management of JIA patients with TMJ involvement.

OBJECTIVE:To estimate the cumulative incidences of orofacial conditions related to temporomandibular joint (TMJ) juvenile idiopathic arthritis (JIA) between diagnosis in childhood to transition into adult care, and to identify features in JIA associated with TMJ involvement.METHODS:A population-based cohort analysis was conducted of patients with JIA involving longitudinal data on orofacial health from 2000 to 2018. Regardless of TMJ status, the patients were referred to the Regional Specialist Craniofacial Clinic of Western Denmark for routine orofacial examinations. Data collection included information about disease-specific background characteristics, TMJ involvement, JIA-induced dentofacial deformity, and orofacial symptoms and dysfunction.RESULTS:A total of 613 patients were followed up with a mean clinical TMJ observation time of 4.0 years. From JIA onset to transition into adult care, the cumulative incidence of patients with JIA involvement of the TMJ was 30.1%. Furthermore, 20.6% of the cohort had developed arthritis-induced dentofacial deformity. A substantial proportion of the cohort experienced several events with orofacial symptoms (23.5%) and dentofacial dysfunction (52%). Young age at diagnosis (<9 years), female gender, and antinuclear antibody positivity were significantly associated with TMJ involvement.CONCLUSION:Orofacial signs and symptoms were frequent findings in children and adolescents with JIA. TMJ involvement was seen in 30.1% of the cohort; and 20.6% of the total cohort developed JIA-related dentofacial deformity before transition into adult care. This is the first population-based study in the era of available biologic treatments to document these frequent orofacial complications in children with JIA.

Background Physiotherapy appears as a promising therapy option for patients with Juvenile Idiopathic Arthritis (JIA) [ 1 , 2 ], but the effects of physiotherapy and jaw exercises on JIA-related orofacial symptoms remain unknown [ 3 ]. The aim of this proof-of-concept study was to assess the impact of orofacial physiotherapy and home-exercise programs in patients with JIA and temporomandibular joint (TMJ) involvement. Methods Twelve patients with JIA and TMJ involvement received a treatment of physiotherapy, complemented by prescribed home exercises spanning over eight weeks. Orofacial symptoms and dysfunction were monitored pre-treatment, during treatment, after treatment, and at a three-months follow-up. Results Orofacial pain frequency and intensity significantly decreased during the course of the treatment (p = 0.009 and p = 0.006), with further reductions observed at the three-month follow-up (p = 0.007 and p = 0.002). During treatment, the mandibular function improved significantly in terms of maximal mouth opening capacity, laterotrusion, and protrusion. Conclusions This proof-of-concept study shows favourable effects of physiotherapy and home excercises in the management of JIA-related orofacial symptoms and dysfunctions.

OBJECTIVE To describe a method to calculate the total intra-articular volume (inter-osseous space) of the temporomandibular joint (TMJ) determined by cone-beam computed tomography (CBCT). This could be used as a marker of tissue proliferation and different degrees of soft tissue hyperplasia in juvenile idiopathic arthritis (JIA) patients. MATERIALS AND METHODS Axial single-slice CBCT images of cross-sections of the TMJs of 11 JIA patients and 11 controls were employed. From the top of the glenoid fossa, in the caudal direction, an average of 26 slices were defined in each joint (N = 44). The interosseous space was manually delimited from each slice by using dedicated software that includes a graphic interface. TMJ volumes were calculated by adding the areas measured in each slice. Two volumes were defined: Ve-i and Vi , where Ve-i is the inter-osseous space, volume defined by the borders of the fossa and Vi is the internal volume defined by the condyle. An intra-articular volume filling index (IF) was defined as Ve-i /Vi , which represents the filling of the space. RESULTS The measured space of the intra-articular volume, corresponding to the intra-articular soft tissue and synovial fluid, was more than twice as large in the JIA group as in the control group. CONCLUSION The presented method, based on CBCT, is feasible for assessing inter-osseus joint volume of the TMJ and delimits a threshold of intra-articular changes related to intra-articular soft tissue proliferation, based on differences in volumes. Intra-articular soft tissue is found to be enlarged in JIA patients.