Pycnodysostosis: Characteristics of teeth, mouth and jaws.

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.

Quantification of temporomandibular joint space in patients with juvenile idiopathic arthritis assessed by cone beam computerized tomography.

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.

Orthodontic and orthopedic management of dentofacial deformity from juvenile idiopathic arthritis: a systematic literature review.

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).

Management of temporomandibular joint arthritis in children and adolescents: An introduction for orthodontists.

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.

Management of unilateral craniofacial microsomia with orthopaedic functional appliances: A systematic literature review.

The study aimed to summarize current knowledge regarding the use of orthopaedic functional appliances (OFA) in managing unilateral craniofacial microsomia (UCM). The eligibility criteria for the review were (1) assessing use of OFA as a stand-alone treatment and (2) using OFA in combination during or after MDO. The PICO (population, intervention, comparison and outcome) format formulated clinical questions with defined inclusion and exclusion criteria. No limitations concerning language and publication year were applied. Information sources: A literature search of Medline, Scopus, Embase, Cochrane Central Register of Controlled Trials, Web of Science databases without restrictions up to 30 September 2022. The risk of bias was assessed. According to Cochrane and PRISMA guidelines, two independent authors conducted data extraction. The level of evidence for included articles was evaluated based on the Oxford evidence-based medicine database. Due to the heterogeneity of studies and insufficient data for statistical pooling, meta-analysis was not feasible. Therefore, the results were synthesized narratively. A total of 437 articles were retrieved. Of these, nine met inclusion criteria: five assessing OFA and four assessing OFA during or after MDO. There is limited evidence to suggest that stand-alone and combination treatment with OFA is beneficial for treating mild-to-moderate UCM-related dentofacial deformities in short term. No studies assessed the burden of care. In the management of UCM, there is insufficient evidence supporting the efficacy of OFA as a stand-alone treatment or when combined with MDO. Additionally, there is a lack of evidence regarding treatment protocols and the effect on the condyles and the TMJ. The study was registered at Prospero database number CRD42020204969.

Obstructive sleep apnoea in pycnodysostosis: A three-dimensional upper airway analysis.

AIM: To assess the upper airway (UA) morphology in patients with pycnodysostosis with a 3D analysis, compare results with normative data and investigate the correlation of the total volume (TV) with other UA morphology variables. MATERIALS AND METHODS: Cone beam computed tomography (CBCT) images of eight Danish patients with pycnodysostosis (4 males and 4 females with a mean age of 31.8 years, SD: 16.3 years) were analyzed using Mimics® (Materialise® ) and compared with a sex- and age-matched control group (6 males and 8 females with a mean age of 33.6 years, SD: 18.6 years). RESULTS: The distance from the tip of the epiglottis (E) to the Frankfurt horizontal plane (Fp) was significantly shorter in the pycnodysostosis group (P < .042). Regarding the cross-sectional measurements, at the 'maximum constriction' (P < .005), the 'upper airway limit' (P < .001) and the 'lower airway limit' (P < .035) cross-sections were significantly smaller in the pycnodysostosis group. The volumes 'nasopharynx' (P < .002) and 'total airway' (TV) (P < .01) were also significantly smaller. CONCLUSION: Patients with pycnodysostosis have a reduced total airway as well as nasopharyngeal volume compared with matched controls. Additionally, they have a reduced cross-sectional area in the upper and lower borders of the UA, and the area of maximum constriction is also reduced. These factors might explain the high prevalence of obstructive sleep apnoea in pycnodysostosis. Total airway is positively correlated with total length and cross-sections at all levels including the maximum constriction area as well as the anteroposterior dimension at the upper and lower airway borders.

Comparing Clinical and Radiological Manifestations of Adolescent Idiopathic Condylar Resorption and Juvenile Idiopathic Arthritis in the Temporomandibular Joint.

PURPOSE: This cross-sectional study compared orofacial manifestations at the time of diagnosis in 2 temporomandibular joint (TMJ) conditions: adolescent idiopathic condylar resorption (ICR) and TMJ involvement from juvenile idiopathic arthritis (JIA). MATERIALS AND METHODS: This retrospective study included 19 JIAs, 19 ICRs, and 19 control patients, all treated at the Section of Orthodontics, Aarhus University Craniofacial Clinic, Denmark. From patient files, we retrieved radiological data from cone-beam computed tomographies along with information on symptoms and orofacial function at the time of diagnosis. Validated methodologies were used to evaluate TMJ and dentofacial morphology. RESULTS: We found no statistically significant intergroup differences in severity of deformation of TMJ structures (TMJ deformity) between JIA and ICR patients. However, the ICR group showed significantly greater signs of dentofacial deformity on 4 outcome variables: mandibular inclination, posterior/anterior lower face height ratio, mandibular sagittal position, and mandibular occlusal plane inclination. Significant intergroup differences in clinical presentation were seen in 5 of 20 variables. Thus, the JIA group reported significantly more symptoms of TMJ pain, TMJ morning stiffness, and TMJ pain on palpation during the clinical examination, whereas the ICR group reported significantly more TMJ clicking during function and had a higher proportion of patients with anterior open bite. CONCLUSIONS: Cone-beam computed tomography examination showed a similar degree of TMJ deformity in ICR and JIA patients at the time of diagnosis. ICR patients presented with a significantly higher degree of dentofacial deformity than JIA patients and healthy controls, which relates to the timing of the diagnosis. Arthralgia, TMJ morning stiffness, and TMJ palpitation pain were more common in JIA patients, whereas TMJ clicking during function and anterior open bites were more common in ICR patients.

Influence of Infant Cleft Dimensions on Velopharyngeal Function in 5-Year-Old Danish Children Born With Unilateral Cleft Lip and Palate.

AIM: To examine the association of cleft severity at infancy and velopharyngeal competence in preschool children with unilateral cleft lip and palate operated with early or delayed hard palate repair. DESIGN: Subgroup analysis within a multicenter randomized controlled trial of primary surgery (Scandcleft). SETTING: Tertiary health care. One surgical center. PATIENTS AND METHODS: One hundred twenty-five infants received cheilo-rhinoplasty and soft palate repair at age 3 to 4 months and were randomized to hard palate closure at age 12 or 36 months. Cleft size and cleft morphology were measured 3 dimensionally on digital models, obtained by laser surface scanning of preoperative plaster models (mean age: 1.8 months). MAIN OUTCOME MEASUREMENTS: Velopharyngeal competence (VPC) and hypernasality assessed from a naming test (VPC-Sum) and connected speech (VPC-Rate). In both scales, higher scores indicated a more severe velopharyngeal insufficiency. RESULTS: No difference between surgical groups was shown. A low positive correlation was found between posterior cleft width and VPC-Rate (Spearman = .23; P = .025). The role of the covariate "cleft size at tuberosity level" was confirmed in an ordinal logistic regression model (odds ratio [OR] = 1.17; 95% confidence interval [CI]:1.01-1.35). A low negative correlation was shown between anteroposterior palatal length and VPC-Sum (Spearman = -.27; P = .004) and confirmed by the pooled scores VPC-Pooled (OR = 0.82; 95% CI: 0.69-0.98) and VPC-Dichotomic (OR = 0.82; 95% CI: 0.68-0.99). CONCLUSIONS: Posterior cleft dimensions can be a modest indicator for the prognosis of velopharyngeal function at age 5 years, when the soft palate is closed first, independently on the timing of hard palate repair. Antero-posterior palatal length seems to protect from velopharyngeal insufficiency and hypernasality. However, the association found was significant but low.

Palatal morphology in unilateral cleft lip and palate patients: Association with infant cleft dimensions and timing of hard palate repair.

OBJECTIVES: To assess the impact of cleft severity and timing of hard palate repair on palatal dimensions in unilateral cleft lip and palate (UCLP) children. SETTING AND SAMPLE POPULATION: Single-centre analysis within a multicenter RCT of primary surgery; 122 UCLP randomized to early hard palate closure (EHPC) at 12 months or delayed hard palate closure (DHPC) at 36 months; 28 frequency-matched controls. METHODS: Linear measurements of palatal height, width and length were performed on 116 digital models of UCLP subjects (8.21 years, SD = 0.53) and 28 models of non-cleft individuals (8.44 years SD = 0.72). Cleft dimensions at infancy (mean 1.8 months) were considered. In a pilot study, shell-to-shell distances between the 3D cleft palate objects and a reference mesh were calculated and differences between the groups assessed. Morphological differences were visualized using colour mapping. RESULTS: Compared to controls, UCLP subjects presented a higher palate at the level of the anterior scar (P = 0.002), but generally a lower palate in the middle region (P < 0.001). Comparing UCLP subgroups, the DHPC subjects showed a flatter palate posteriorly (P = 0.048) and the EHPC group exhibited more transversal constriction (P = 0.003 at M1 level). 3D analysis revealed a shallower palate in the DHPC group both in the middle (P = 0.002) and the posterior part (P = 0.008). Anterior cleft severity correlated negatively with palatal height (P = 0.01). CONCLUSIONS: Unilateral cleft lip and palate palates differ from controls in width and height. DHPC may represent an advantage for the transversal dimension, but a disadvantage for palatal height. Infant cleft dimensions partially explain differences in palatal height.

An Algorithm for Management of Dentofacial Deformity Resulting From Juvenile Idiopathic Arthritis: Results of a Multinational Consensus Conference.

PURPOSE: Juvenile idiopathic arthritis (JIA) frequently affects the temporomandibular joints and may cause dentofacial deformity and dysfunction. The adverse effects of JIA on dentofacial growth, morphology, and function may be due to erosion of the existing mandibular condyle(s), the inhibitory effect of the arthritis on the growing mandible, or both. No algorithm exists for management of JIA-induced skeletal and dental abnormalities; treatment varies widely. MATERIALS AND METHODS: On the basis of the available literature and expert opinion obtained by a consensus conference held by the Temporomandibular Joint Juvenile Arthritis (TMJAW) group-a multidisciplinary and multinational clinical and research network dedicated to the diagnosis and management of temporomandibular joint arthritis caused by JIA-we present an algorithm to be used as a conceptual framework for management of dentofacial deformity resulting from JIA. RESULTS: An algorithm for management of dentofacial deformity resulting from JIA is presented and exemplified by 5 clinical cases. CONCLUSIONS: A standardized algorithm will improve clinical decision making and facilitate outcome research by allowing valid comparisons between published research studies. We emphasize the importance of multidisciplinary evaluation, management, and long-term follow-up.

Novel 3-D Analysis for the Assessment of Cleft Dimensions on Digital Models of Infants With Unilateral Cleft Lip and Palate.

OBJECTIVE: To present a 3-D standardized analysis of cleft dimensions. MATERIALS: Thirty-one plaster casts of unilateral cleft lip and palate (UCLP) infants were laser scanned. Landmarks and coordinate system were defined. Linear distances and surfaces were measured, and the ratio between cleft and palatal area was calculated (3-D infant cleft severity ratio [3D ICSR]). The digitally measured areas were compared with silicone membranes, adapted to the plaster casts, and analyzed by optic microscopy. MAIN OUTCOME MEASURES: Repeatability, reproducibility, and validity. RESULTS: Bland-Altman plots showed minor bias for anterior cleft width (0.16 mm), arch length (0.23 mm), and palatal surface (-9.18 mm2). The random error was maximum 0.78 mm for linear measurements and 12.1 mm2 for area measurements, reliability of 3D ICSR was 0.99 (intraclass correlation coefficient), differences between measurements on digital and plaster models were less than 2%. CONCLUSIONS: The method offers valid and reproducible 3-D assessment of cleft size and morphology. 3-D infant cleft severity ratio can be used as baseline value for surgical planning and may have a role in predicting dentofacial development.

Evaluation of facial asymmetry in patients with juvenile idiopathic arthritis: Correlation between hard tissue and soft tissue landmarks.

INTRODUCTION: The aims of this study were to assess the correlation between facial hard and soft tissue asymmetry in patients with juvenile idiopathic arthritis, to identify valid soft tissue points for clinical examination, and to assess the smallest clinically detectable level of dentofacial asymmetry. METHODS: Full-face cone-beam computed tomography scans and 3-dimensional photographs were used to assess facial hard and soft tissue asymmetry in 21 patients with juvenile idiopathic arthritis. A survey was conducted to assess how asymmetry is perceived observationally based on cone-beam computed tomography scans and 3-dimensional photographs. RESULTS: Significant linear correlations were seen between the hard and soft tissue landmark deviations at both the transverse and vertical positions. Among medial soft tissue points, glabella had the smallest deviation and pogonion the largest deviation from the midsagittal plane. Professionals could identify facial asymmetry based on images beyond a cutoff threshold of 2 mm for both pogonion and gonion. CONCLUSIONS: Soft tissue pogonion and gonion were identified as the most appropriate landmarks to clinically predict hard tissue facial asymmetry. Facial asymmetries are most pronounced in the lower facial third in patients with juvenile idiopathic arthritis. Professionals can accurately identify asymmetry exceeding 2 mm.

No association between types of unilateral mandibular condylar abnormalities and facial asymmetry in orthopedic-treated patients with juvenile idiopathic arthritis.

INTRODUCTION: Dentofacial asymmetries are often observed in patients with juvenile idiopathic arthritis (JIA) and temporomandibular joint (TMJ) involvements. The aim of this split-face study was to associate types of radiologic TMJ abnormalities with the degree of dentofacial asymmetry in patients with unilateral TMJ involvements assessed with cone-beam computed tomography. METHODS: Forty-seven JIA patients and 19 nonarthritic control subjects were included in the study. Normal condylar radiologic cone-beam computed tomography appearance in at least 1 TMJ was the inclusion criterion for all patients with JIA. The contralateral TMJ was thereafter scored as either "normal," "deformed," or "erosive," consistent with predefined criteria. Based on the bilateral radiologic TMJ appearances, 3 JIA groups were assigned: normal/normal, normal/deformed, and normal/erosive. The severity of the dentofacial asymmetry was compared between the JIA groups and control subjects. Dentofacial asymmetry was expressed as interside ratios and angular measurements. RESULTS: Eighty-seven percent of the JIA patients were being treated or had previously received treatment with a functional orthopedic appliance at the time of the cone-beam computed tomography. Significantly greater dentofacial asymmetries were observed in the 2 groups of JIA patients with unilateral condylar abnormalities (deformation or erosion) than in the other groups. A similar degree of dentofacial asymmetry was observed in JIA patients with bilateral normal TMJs and in the nonarthritic control group. CONCLUSIONS: JIA patients with unilateral condylar abnormalities (deformation or erosion) exhibited significantly more severe dentofacial asymmetries than did the JIA patients without condylar abnormalities and the control subjects. We found the same degree of dentofacial asymmetry when dividing patients with condylar abnormalities into deformation and erosion groups.

Idiopathic condylar resorptions: 3-dimensional condylar bony deformation, signs and symptoms.

INTRODUCTION: Our aim was to describe 3-dimensional condylar deformation of the temporomandibular joint (TMJ) and symptoms and signs of temporomandibular dysfunction (TMD) in patients with idiopathic condylar resorption (ICR). METHODS: We included 25 patients with ICR and 25 controls. We performed cone-beam computed tomographic scans and analyzed condylar width, length, and height as well as the condylar axial angle and the condylar neck angle. TMJ cross sections were evaluated for degenerative characteristics and location of bony deformations. Furthermore, symptoms and signs of TMD were described in the ICR group. RESULTS: In the ICR group, we found statistically significantly reduced condylar width (mean difference, 2.0 mm), height (mean difference, 4.9 mm), and condylar axial angle (mean difference, 10.6°); 84% of the TMJs had a posterior condylar neck angle (control group, 22%). The most common degenerative changes were noncongruent shape of the condyle-fossa relationship (72%), condylar resorption (56%), and nonintact cortex (40%). More than 70% of the joints with bony deformations showed changes along the entire condylar head. Most patients with ICR showed symptoms and signs of TMD; nevertheless, 12% had no signs or symptoms of TMD. CONCLUSIONS: ICR in the TMJ changes the shape and reduces the size of the condyle. Deformity locations are unspecified, and the entire condyle is often affected. Most patients with ICR have signs or symptoms of TMD; however, a small group was asymptomatic and without clinical signs.

Signs and symptoms after temporomandibular joint washing and cannula placement assessed by cone beam computerized tomography.

OBJECTIVE: Analyses of temporomandibular joint synovial fluid using the hydroxocobalamin push-pull technique are increasingly used. However, objective complications and subjective experiences from this procedure have not been described. Firstly, this study aimed to describe discomfort and potential side-effects of this method with special emphasis on symptoms related to the arthrocentesis to be used for future patient information and Ethical Committee applications. Secondly, this study aimed to evaluate the use of cone beam computed tomography (CBCT) as control of intra-capsular cannula placement. METHODS: Twenty healthy, young adult volunteers were included. Extensive objective and subjective questionnaires were completed before and 14 days after the synovial fluid sampling. With the cannula inside the joints a CBCT was done to investigate if this procedure can be used to verify intra-capsular cannula position. RESULTS: The subjective findings: Most subjects did experience mild pain or discomfort post-operatively. In 12 of 20 subjects symptoms had resolved after 2 days and no subjects had symptoms for more than a week. The longer lasting symptoms were mainly transient joint sounds on mandibular movement. Objective findings: 14 days after the sampling mandibular protrusion had improved 1 mm, but all other objective measures were equal compared to baseline. CBCT showed a large variation in cannula position and no conclusions could be drawn from this. CONCLUSION: The hydroxocobalamin push-pull synovial fluid sampling may cause minor, transient symptoms. CBCT does not seem to provide any clinical benefits concerning the correct cannula position in relation to the upper joint compartment and disc.

Long-Term Follow-Up of Orthognathic Surgery in 19 Patients with Juvenile Idiopathic Arthritis.

Objectives: Dentofacial deformity following juvenile idiopathic arthritis with temporomandibular joint involvement is associated with functional, aesthetic, and psychosocial impairment. Surgical treatment may involve combinations of orthognathic surgery. The aims of this retrospective study were to assess orofacial symptoms, functional and aesthetic status, and stability after orthognathic surgery. Material and Methods: Nineteen patients with juvenile idiopathic arthritis of the temporomandibular joint (TMJ) and dentofacial deformities were included. All patients were treated with combinations of bilateral sagittal split osteotomy, Le Fort I and/or genioplasty, between September 10, 2007 and October 17, 2017. Analysis of patient symptoms and clinical registrations, and frontal/lateral cephalograms was performed pre- and postoperative and long-term (mean: 3.8 and 2.6 years, respectively). Results: Patients experienced no changes in orofacial symptoms or TMJ function, and stable normalisation of horizontal and vertical incisal relations at long-term (horizontal overbite; vertical overbite: P < 0.05). Mandibular lengthening was achieved postoperatively (from mean 79.7 to 87.2 mm; P = 0.004) and was stable. Sella-nasion to A point (SNA) and sella-nasion to B point (SNB) angles increased postoperatively (SNA, mean 79.9° to 82.8°; P = 0.022 and SNB, mean 73.9° to 77.8°; P = 0.003), however, largely reverted to preoperative status at long-term. Conclusions: Orthognathic surgery normalized incisal relations while providing stable mandibular lengthening without long-term deterioration of temporomandibular joint function or orofacial symptoms. No long-term effect on jaw advancements was observed.

Impact of physiotherapy on orofacial manifestations of juvenile idiopathic arthritis.

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.

Odontostomatologic involvement in juvenile localised scleroderma of the face.

AIM: Localised scleroderma of the face (LSF) can lead to significant aesthetical and functional abnormalities. Despite their quite frequent clinical observation, the odontostomatologic complications are not thoroughly described. Aim of the study was to describe the clinical features of the most frequent odontostomatologic abnormalities of LSF and to propose clinical and radiologic criteria for the assessment and follow-up of these complications. METHODS: We performed a cross-sectional, multicenter study involving a multidisciplinary team formed by paediatric rheumatologists, orthodontists and radiologists. Patients with a diagnosis of LSF underwent a comprehensive rheumatologic evaluation, dental examination, conventional radiology (orthopantomography, teleradiography) and cone beam computed tomography. RESULTS: 16 patients, nine F, seven M, aged 6.5-21.9 years, were investigated. The mean disease duration was 7.7 years, 62.5% had extracutaneous complications. All patients reported at least one odontostomatologic complication. The main alterations were: malocclusion (94%), overgrowth tendency of the anterior lower third of the face (82%), gnatologic alterations (69%), dental anomalies (63%), skeletal asymmetry (56%), bone involvement (50%) and temporomandibular joint involvement (19%). CONCLUSIONS: We found a high incidence of odontostomatologic abnormalities in LSF. Cone beam computed tomography represents a new feasible technique to evaluate and monitor soft and hard tissue changes in LSF. The observed findings highlight the importance of a multidisciplinary and standardised management of this challenging and rare condition.

Zygomatic repositioning and Le Fort II distraction with intraoral devices in Apert syndrome: A case report.

The aim of this case report is to describe the surgical technique and outcome using internal intraoral distraction devices in LeFort II distraction with zygomatic repositioning (LF2ZR). In Apert syndrome the midface is characterized by a complex hypoplasia, with the central part being more affected than the lateral orbito-zygomatic complex. In LF2ZR, the zygomas are repositioned and internally fixated, and the central midface is further advanced through a LeFort II distraction. In previous publications, the distraction has been performed using external halo-based devices. It seems that the LF2ZR procedure can be planned and performed with adequate accuracy using virtual surgical planning tools. Knowledge about the possibility of using internal intraoral distraction devices in LF2ZR is important, as the inconspicuous placement of intraoral devices can be advantageous for some patients.