Characteristics of Factors Influencing the Occurrence of Cleft Lip and/or Palate: A Case Analysis and Literature Review.

INTRODUCTION: Cleft lip with or without cleft palate (CL/P) stands as the most common congenital facial anomaly, stemming from multifactorial causes. OBJECTIVE: Our study aimed to ascertain the prevalence and characteristics of cleft palates, identify associated risk factors to inform prevention and prenatal detection for early intervention, and assess postoperative rehabilitation protocols for cleft palates. DESIGN: This study employs a retrospective descriptive and clinical approach. PATIENTS: The study includes 103 children with cleft palates treated at the Department of Head and Neck Surgery Clinic for Children and Young Adults, Department of Clinical Pediatrics, University of Warmia and Mazury. METHODS: We conducted a thorough evaluation of records, considering variables such as sex, cleft type, maternal occupation, parental education, and family history of clefts. Data analysis was carried out using R software version GPL-3 and ordinal logistic regression analyses. RESULTS: Notably, children born to mothers who experienced significant stress during pregnancy exhibited a 9.4-fold increase in the odds of having bilateral cleft palates. Conversely, no substantial evidence was found to support the influence of the child's sex, birth order, body mass, maternal exposure to workplace toxins, infections, or drug toxicity on the dependent variable. CONCLUSIONS: Our findings suggest that children with parents who have a history of clefts and those with less educated mothers are more likely to develop bilateral cleft palates. Additionally, children born to mothers experiencing stress during pregnancy face an increased risk of bilateral cleft palates. It is important to note that there is a paucity of literature on rehabilitation following various cleft palate surgical techniques in children.

Influence of preoperative anatomy and functional status on outcomes after total temporomandibular joint replacement with patient-specific endoprostheses: A retrospective cohort study.

BACKGROUND: Temporomandibular joint (TMJ) replacement may be indicated for various pathological conditions, and the type of condition can affect the surgical procedure and outcomes. The causes of limited range of motion after alloplastic TMJ replacement have not been extensively studied. OBJECTIVES: The present study aimed to evaluate the impact of preoperative jaw anatomy and functional status on the immediate and long-term outcomes of total TMJ replacement using a two-component patient-specific TMJ endoprosthesis. MATERIAL AND METHODS: This retrospective study included 31 patients who underwent total TMJ replacement surgery between 2016 and 2020. The main outcome variable was the maximal incisal opening (MIO) after treatment. Secondary outcome variables included MIO improvement and the presence and type of postoperative complications. The primary predictive variable was the preoperative initial MIO. Secondary predictive variables included sex, age, indications for TMJ replacement, preoperative occlusion, condition of the glenoid fossa and/or condyle, shortening of the mandibular ramus, sagittal mandible position, lateral chin deviation, shape of the coronoid process, and type of surgery. RESULTS: The mean preoperative MIO was 13.0 ±8.0 mm, while the mean MIO 1 month after surgery was 20.6 ±5.5 mm, which was not statistically significant. However, at a later follow-up, functional parameters showed a significant improvement (p = 0.003), with a mean MIO of 32.5 ±5.0 mm 3 years after surgery. Statistical analysis indicated that the initial mouth opening is the strongest predictor of long-term functional recovery after TMJ replacement. Postoperative complications occurred in 4 cases (12.9%) following patient-specific endoprosthesis (PSE) placement. CONCLUSIONS: The use of PSEs for TMJ replacement has enabled the restoration of anatomical relationships in complex clinical cases and an improvement in mouth opening. The preoperative MIO was the only factor that significantly influenced long-term functional outcomes.

Maxillofacial Microvascular Free-Flap Reconstructions in Pediatric and Young Adult Patients-Outcomes and Potential Factors Influencing Success Rate.

Background: Maxillofacial microvascular free-flap reconstructions are significant interventions in the management of congenital defects, traumatic injuries, malignancies, and iatrogenic complications in pediatric and young adult patients. Craniofacial disorders within this demographic can result in profound functional, cosmetic, and psychosocial impairments, highlighting the critical need for thorough investigation into factors that may influence procedural success and postoperative quality of life. This retrospective chart review aims to examine the outcomes and potential influencing factors, aiming to offer valuable insights into optimizing the effectiveness of these reconstructions and improving patient outcomes. Methods: A single head and neck surgical team performed all the included 136 procedures. Demographic and surgical patient data were recorded. Type of transfer performed in each recipient site and major complications were analyzed. Relevant influencing factors, such as age, gender, and etiology of defect were determined using the ANOVA test and χ2 test of independence. Results: The results indicate a 90% success rate. No significant relationship was found between the incidence of total flap loss and patient age, etiology, or graft source. The maxillary reconstructions showed a higher incidence of total flap loss compared to mandibular reconstructions (11 vs. 3 cases). Conclusions: Despite the high success rate, the findings underline the necessity for further research to validate these observations and enhance surgical methods for pediatric and young adult patients.

A European multicenter outcome study on the different perioperative airway management policies following midface surgery in syndromic craniosynostosis: a proposal for a Standard Operating Procedure.

BACKGROUND: Perioperative airway management following midface advancements in children with Apert and Crouzon/Pfeiffer syndrome can be challenging, and protocols often differ. This study examined airway management following midface advancements and postoperative respiratory complications. METHODS: A multicenter, retrospective cohort study was performed to obtain information about the timing of extubation, perioperative airway management, and respiratory complications after monobloc / le Fort III procedures. RESULTS: Ultimately, 275 patients (129 monobloc and 146 Le Fort III) were included; 62 received immediate extubation and 162 delayed extubation; 42 had long-term tracheostomies and nine perioperative short-term tracheostomies. Short-term tracheostomies were in most centers reserved for selected cases. Patients with delayed extubation remained intubated for three days (IQR 2 - 5). The rate of no or only oxygen support after extubation was comparable between patients with immediate and delayed extubation, 58/62 (94%) and 137/162 (85%) patients, respectively. However, patients with immediate extubation developed less postoperative pneumonia than those with delayed, 0/62 (0%) versus 24/161 (15%) (P = 0.001), respectively. Immediate extubation also appeared safe in moderate/severe OSA since 19/20 (95%) required either no or only oxygen support after extubation. The odds of developing intubation-related complications increased by 21% with every extra day of intubation. CONCLUSIONS: Immediate extubation following midface advancements was found to be a safe option, as it was not associated with respiratory insufficiency but did lead to fewer complications. Immediate extubation should be considered routine management in patients with no/mild OSA and should be the aim in moderate/severe OSA after careful assessment.

Planning of physiotherapeutic procedure in patients after mandible reconstruction taking into account donor site: a literature review.

BACKGROUND: Mandible tumors are very rare. One of the main methods of the treatments is resection of the tumor and then reconstruction of the mandible. The donor site is often distant tissue-fibula or ilium. Following this, it is necessary to improve the patient in two ways, on one hand restoring the function of the mandible, and on the other hand, improving the donor site area. For that reason, physiotherapy after tumor resection and reconstruction of the mandible is very complicated. The aim of this bibliographic review was to find the methods of the reconstruction of the mandible in the context of patients' functional assessment after surgeries to create effective physiotherapeutic procedures in the feature. METHODS: PEDro, Medline (PubMed), Cochrane Clinical Trials were searched. RESULTS: 767 articles were found. 40 articles were included to this literature review. CONCLUSIONS: Authors showed different kinds of surgeries strategy for patients with tumors of the mandible. They also showed manners of patients' functional assessment in the localization of transplantation and donor site. It could be useful for physiotherapists during planning of comprehensive physiotherapy.

Biomechanical Evaluation of Temporomandibular Joint Reconstruction Using Individual TMJ Prosthesis Combined with a Fibular Free Flap in a Pediatric Patient.

The main aim of this study was to perform a complex biomechanical analysis for a custom-designed temporomandibular joint (TMJ) prosthesis in combination with a fibular free flap in a pediatric case. Numerical simulations in seven variants of loads were carried out on 3D models obtained based on CT images of a 15-year-old patient in whom it was necessary to reconstruct the temporal-mandibular joints with the use of a fibula autograft. The implant model was designed based on the patient's geometry. Experimental tests on a manufactured personalized implant were carried out on the MTS Insight testing machine. Two methods of fixing the implant to the bone were analyzed-using three or five bone screws. The greatest stress was located on the top of the head of the prosthesis. The stress on the prosthesis with the five-screw configuration was lower than in the prosthesis with the three-screw configuration. The peak load analysis shows that the samples with the five-screw configuration have a lower deviation (10.88, 0.97, and 32.80%) than the groups with the three-screw configuration (57.89 and 41.10%). However, in the group with the five-screw configuration, the fixation stiffness was relatively lower (a higher value of peak load by displacement of 171.78 and 86.46 N/mm) than in the group with the three-screw configuration (where the peak load by displacement was 52.93, 60.06, and 78.92 N/mm). Based on the experimental and numerical studies performed, it could be stated that the screw configuration is crucial for biomechanical analysis. The results obtained may be an indication for surgeons, especially during planning personalized reconstruction procedures.

Use of a Fibula Free Flap for Mandibular Reconstruction in Severe Craniofacial Microsomia in Children with Obstructive Sleep Apnea.

This is a retrospective study describing a multi-stage protocol for the management of severe mandibular hypoplasia in craniofacial microsomia (CFM) with accompanying obstructive sleep apnea (OSA). Patients with severe mandibular hypoplasia require reconstruction functionality and esthetical features. In the cohort, reconstructions based on free fibular flaps (FFF) may be the most effective way. Patients aged 4-17 years with severe mandibular hypoplasia were treated with FFF, which initially improved the respiratory function assessed on polysomnography (AHI). In the next stages of treatment of cases with respiratory deterioration, it was indicated to perform distraction osteogenesis (DO) of the mandible and the structures reconstructed with FFF. All surgeries were planned in accordance with virtual surgery planning VSP. The aim of the study was to prospectively assess the effectiveness of multi-stage mandibular reconstruction in craniofacial microsomia with the use of a free fibula flap in terms of improving respiratory failure due to obstructive sleep apnea (OSA). The FFF reconstruction method, performed with virtual surgical planning (VSP), is proving to be an effective alternative to traditional methods of mandibular reconstruction in patients with severe CFM with OSA.

Children with Rare Nager Syndrome-Literature Review, Clinical and Physiotherapeutic Management.

Nager syndrome is a rare human developmental disorder characterized by craniofacial defects including the downward slanting of the palpebral fissures, cleft palate, limb deformities, mandibular hypoplasia, hypoplasia or absence of thumbs, microretrognathia, and ankylosis of the temporomandibular joint. The prevalence is very rare and the literature describes only about a hundred cases of Nager syndrome. There is evidence of autosomal dominant and autosomal recessive inheritance for Nager syndrome, suggesting genetic heterogeneity. The majority of the described causes of Nager syndrome include pathogenic variants in the SF3B4 gene, which encodes a component of the spliceosome; therefore, the syndrome belongs to the spliceosomopathy group of diseases. The diagnosis is made on the basis of physical and radiological examination and detection of mutations in the SF3B4 gene. Due to the diversity of defects associated with Nager syndrome, patients require multidisciplinary, complex, and long-lasting treatment. Usually, it starts from birth until the age of twenty years. The surgical procedures vary over a patient's lifetime and are related to the needed function. First, breathing and feeding must be facilitated; then, oral and facial clefts should be addressed, followed by correcting eyelid deformities and cheekbone reconstruction. In later age, a surgery of the nose and external ear is performed. Speech and hearing disorders require specialized logopedic treatment. A defect of the thumb is treated by transplanting a tendon and muscle or transferring the position of the index finger. In addition to surgery, in order to maximize a patient's benefit and to reduce functional insufficiency, complementary treatments such as rehabilitation and physiotherapy are recommended. In our study, we describe eight patients of different ages with various cases of Nager syndrome. The aim of our work was to present the actual genetic knowledge on this disease and its treatment procedures.

Dental Implant Healing Screws as Temporary Oral Drug Delivery Systems for Decrease of Infections in the Area of the Head and Neck.

Background: Periimplantitis is continuously one of major threats for the uneventful functioning of dental implants. Current approaches of drug delivery systems are being more commonly implemented into oral- and maxillofacial biomaterials in order to decrease the risk of implant failure due to bacterial infection. Silver nanoparticles and their compounds have been proven in eradicating oral bacteria responsible for peri-implant infections. Nevertheless, their evaluation as coating for implant abutments has not been extensively evaluated so far. This article describes a novel coating consisting of zinc oxide (ZnO) and silver (Ag) nanoparticles (NPs). This coating was used to modify healing abutments that could be used as drug delivery systems in oral implantology. Materials and Method: Nanoparticles with a ZnO + 0.1% Ag composition were produced by microwave solvothermal synthesis and then incorporated into the surface of titanium healing abutments by high-power ultrasonic deposition. Surface morphology, roughness, wettability were evaluated. Ability of biofilm formation inhibition was tested against S. mutans, S. oralis, S. aureus and E. coli. Results: ZnO+0.1%Ag NPs were sufficiently deposed on the surface of the abutments creating nanostructured coating which increased surface roughness and decreased wettability. Modified abutments significantly decreased bacterial biofilm formation. Bacteria present in SEM studies were unlikely to settle and replicate on the experimental abutments as their cells were rounded, insufficiently spread on the surface and covered with released NPs. Conclusion: Experimental nanostructured abutments were easily manufactured by high-power ultrasonic deposition and provided significant antibacterial properties. Such biomaterials could be used as temporary drug delivery abutments for prevention and treatment of intra- and extraoral peri-implant infections in the area of the head and neck.

Analysis of the effects of mandibular reconstruction based on microvascular free flaps after oncological resections in 21 patients, using 3D planning, surgical templates and individual implants.

INTRODUCTION: Post- resection defects in the area of the head and neck frequently result in functional impairment of the masticatory system and unaesthetic outcome in the facial appearance. In pediatric population they exert a devastating effect on speech development, breathing and facial growth leading to secondary deformations which are extremely challenging in reconstruction by means of conventional surgery protocols. MATERIAL AND METHODS: 21 patients (14 males/ 7 females) aged 4-17 years old, treated between 2015 and 2019 due to malignant/benign tumors or congenital deformities requiring mandible resection were enrolled in the study. All patients underwent Virtual Surgical Planning (VSP), mandible tumor resection with use surgery guides and defect reconstruction with microvascular bone graft supported with custom implants. Postoperatively, 3D models used for the planning and postoperative 3D model of the reconstructed mandible were compared using authors method based on easily measurable morphometric measurements (3 angles, 3 linear dimensions), allowing a three-dimensional quantitative assessment of postoperative outcomes of the surgery. Linear regression analysis and one-way analysis of variance were used to evaluate the clinical material. The difference was considered significant if p < 0.05. RESULTS: Free fibula flap (FFF) was most commonly used type of the reconstruction of the pediatric mandible. The average maximum deviation for the analyzed cases was 7.7 mm, and the average minimum deviation was -6.09 mm, however without significant differences and the postoperative position of the mandible condyle was comparable to the position designed in the 3DVSP preoperatively. Position of the whole reconstructed mandible by means of individually planned grafts and plates provided comparable position of the ramus and mandible body and no chin deviation as the differences between the pre-operative axial and sagittal angles and their post-operative equivalents were insignificant (p > 0,05). CONCLUSIONS: This study confirms applicability of the 3DVSP in pediatric craniomaxillofacial surgery. Preoperative planning of the osteotomies, grafting technique and production of the individualized guides and implants provides precise tumor resection and immediate reconstruction adjusted to the specific anatomy of the pediatric patients.

Protocol for Multi-Stage Treatment of Temporomandibular Joint Ankylosis in Children and Adolescents.

Treatment of temporomandibular ankylosis is challenging and frequently leads to re-ankylosis, relapse, dangerous complications and, in turn, the need for multiple operations. In this article, we present a protocol for the treatment of ankylosis of the temporomandibular joints that assumes earlier intervention with the assistance of 3D virtual surgical planning (3DVSP) and custom biomaterials for better and safer surgical outcomes. Thirty-three patients were treated due to either uni- or bilateral temporomandibular ankylosis. Twenty individuals received temporomandibular prosthesis, whereas seventeen required simultaneous 3D virtual surgical/planned orthognathic surgery as the final correction of the malocclusion. All patients exhibited statistically significant improvements in mouth opening (from 1.21 ± 0.74 cm to 3.77 ± 0.46 cm) and increased physiological functioning of the mandible. Gap arthroplasty and aggressive rehabilitation prior to temporomandibular prosthesis (TMJP) placement were preferred over costochondral autografts. The use of 3DVSP and custom biomaterials enables more precise, efficient and safe procedures to be performed in the paediatric and adolescent population requiring treatment for temporomandibular ankylosis.

Efficacy of immediate physiotherapy after surgical release of zygomatico-coronoid ankylosis in a young child: A case report.

BACKGROUND: Temporomandibular joint ankylosis (TMJ ankylosis) manifests itself as a locked jaw and reduced mouth opening. We hypothesized that the efficacy of TMJ ankylosis surgery in a child may largely depend on physiotherapeutic management. CASE DESCRIPTION: In this case report, we present the treatment of a girl between 1 and 4 years of age, who was unable to open her jaws immediately after birth. Congenital ankylosis was diagnosed. INTERVENTION: Two models of therapeutic management were presented, with a surgical procedure to release bone fusion adopted as a starting point. In the first model, the rehabilitation procedure started 21 days after surgery. OUTCOMES: Despite rehabilitation, no clinical improvement was achieved (i.e. the child was still unable to open her jaws). In the second model, rehabilitation started from the day after surgery and management according to the second model allowed for obtaining therapeutic benefits. After therapy, the girl was able to abduct the mandible to a degree allowing for improved speech and the ability to crush food. CONCLUSIONS: This clinical case shows that the efficacy of surgical procedures may depend on the type of postoperative management. It was demonstrated that physiotherapy started immediately after the procedure to release the ankylosis improved the mandible mobility outcomes for this child.

Treacher Collins Syndrome: Genetics, Clinical Features and Management.

Treacher Collins syndrome (TCS) is associated with abnormal differentiation of the first and second pharyngeal arches, occurring during fetal development. Features of TCS include microtia with conductive hearing loss, slanting palpebral fissures with possibly coloboma of the lateral part of lower eyelids, midface hypoplasia, micrognathia as well as sporadically cleft palate and choanal atresia or stenosis. TCS occurs in the general population at a frequency of 1 in 50,000 live births. Four subtypes of Treacher Collins syndrome exist. TCS can be caused by pathogenic variants in the TCOF1, POLR1D, POLR1C and POLR1B genes. Genetically, the TCOF1 gene contains 27 exons which encodes the Treacle protein. In TCOF1, over 200 pathogenic variants have been identified, of which most are deletions leading to a frame-shift, that result in the formation of a termination codon. In the presented article, we review the genetics and phenotype of TCS as well as the management and surgical procedures utilized for treatment.

Protocol and Evaluation of 3D-Planned Microsurgical and Dental Implant Reconstruction of Maxillary Cleft Critical Size Defects in Adolescents and Young Adults.

Functional and esthetic final reconstruction of the cleft maxilla is still challenging. Current reconstructive and augmentation techniques do not provide sufficient bone and soft tissue support for the predictable rehabilitation with dental implants due to presence of maxillary bone critical size defects and soft tissue deficiency, scaring and poor vascularity. In this article the protocol for the use of 3D virtual surgical planning and microvascular tissue transfers for the reconstruction and rehabilitation of cleft maxilla is presented. Twenty-five patients (8 male/17 female) aged 14-41 years old with cleft-associated critical size defects were treated by 3D-virtual planned microvascular tissue transfers taken either from fibula, iliac crest, radial forearm, or medial femoral condyle. Follow-up lasted 1-5 years. No significant bone resorption (p > 0.005) nor volume loss of the graft was observed (p = 0.645). Patients received final permanent prosthetic reconstruction of the anterior maxilla based on 2-5 dental implants, depending on the defect severity. This is the first study presenting the use of virtual planning in the final restoration of the cleft maxilla with microvascular tissue transfers and dental implants. Presented protocol provide highly functional and aesthetic results.

Diagnosis, Early Care, and Treatment of Hallermann-Streiff Syndrome: A Review of the Literature.

Hallermann-Streiff syndrome is a rare congenital disorder characterized by a wide spectrum of craniofacial abnormalities. A review of the available literature reveals that only approximately 200 cases of the disease have been reported worldwide. For this article, we performed a literature review as a basis for a proposed scheme for early care and treatment. A comprehensive database search was carried out with the use of Medline (PubMed), ISI Web of Science, and ScienceDirect/Scopus. Of the 551 studies initially found, an evaluation using inclusion and exclusion criteria ultimately resulted in a total of 33 articles. Most of the articles are case reports, and only approximately 20% of these articles include treatment options. We propose an early care and treatment schedule based on the presented symptoms. [Pediatr Ann. 2021;50(5):e227-e231.].

Physical therapy of temporomandibular disorder in a child with arthrogryposis multiplex congenita: A case report and literature review.

Background: Arthrogryposis multiplex congenita (AMC) is a rare congenital disorder characterized by the occurrence of polyarticular contractures. Temporomandibular disorders (TMD) affect 25% of patients with arthrogryposis.Clinical Presentation: In this case report, the authors describe rehabilitation of a child between 8 and 24 months of age with AMC accompanied by Pierre-Robin Sequence. Physiotherapy directed to the TMJ included manual therapy, food intake training, and sensory training.Conclusion: Increase in the mobility of the TMJ and improvement in food intake, swallowing, speech, and breathing were achieved. Further research is necessary for investigating the effects of the proposed protocol of physiotherapy on a larger number of children with AMC and co-occurrence of TMD.

Alterations of upper airway volume caused by Le Fort III osteodistraction in children.

PURPOSE: The aim of the study was to assess changes in the upper respiratory tract and sleep quality in patients who were suffering from midfacial hypoplasia and treated with the movement of underdeveloped middle segment of the face with an Le Fort III osteotomy and distraction. METHODS: In this study patients aged 7-19, suffering from Crouzon syndrome, Apert syndrome, or other craniosynostosis were treated with Le Fort III osteotomy and midface distraction. Patients were subjected to radiological examination and polysomnography before and after the treatment. Typical anthropometric points were identified on lateral cephalograms, and were used to take linear and angular measurements. The surface and the volume of the upper respiratory tract were measured with the Dolphin Imaging software. Apnoea Hypopnea Index (AHI) was used to assess the sleep quality. RESULTS: In all 18 patients the analysis showed statistically significant changes of the AHI and in the linear, angular and volumetric measurements. Mean change of the volume of the upper respiratory tract was 12,4 ± 11,3cm3(p = 0,0001) and of the surface was 615 ± 521 mm2 (p = 0,0000000002). Mean improvement of AHI was 9 ± 6,2 (p = 0,00006). In three cases patients had tracheostomy prior to operation and none of them required tracheostomy after the operation. CONCLUSIONS: The use of distraction osteogenesis of the middle segment of the face combined with Le Fort III osteotomy results in dilation of the upper respiratory tract at the nasopharyngeal level and at the soft palate level resulting in elimination of sleep and respiration disorders. Further studies with polysomnography are necessary, as well as observation of patients over time and monitoring of treatment stability.

Virtual surgical planning and cone beam computed tomography in reconstruction of head and neck tumors - pilot study.

<b>Introduction:</b> The aim of the study was to prove that a combination of visual surgical planning (VSP) and cone beam computed tomography (CBCT) is an optimal technique in fibular free flap reconstructions after complex tumor resections in the head and neck region and that it leads to better functional and aesthetic outcomes. <br><b>Material and method:</b> Six patients (3 females, 3 males) with head and neck tumors were included in the study. The region concerned midface in 2 cases and mandible in 4 patients. On the basis of computed tomography of the head, fibular free flap (FFF) reconstruction was planned with the VSP technique. The 3D-printed models were prepared. At the beginning of the operation and a few minutes after the reconstruction, an xCAT CBCT by XORAN was performed. Minor corrections of the angles of the reconstructed bony parts were made where needed. The time of the operation was assessed for each case. Functional and cosmetic results were evaluated in a 1-year follow-up. <br><b>Results:</b> The mean time of operation was 6 hours and 48 minutes, which was approximately 1hour and 40 minutes less than standard reconstructive surgery. Functional recovery was achieved in all patients. Aesthetic result was unsatisfactory for 2 patients due to insufficient soft tissue masses of FFF. <br><b>Conclusions:</b> The authors claim that intraoperative CBCT imaging, regardless of the cost, improves the accuracy of aesthetic outcome of reconstructive surgeries based on VSP, especially in the region of the midface and the mandible. Further studies on a higher number of subjects are required.

Bone changes on lateral cephalograms and CBCT during treatment of maxillary narrowing using palatal osteodistraction with bone-anchored appliances.

The most common problem in surgical and orthodontic treatment involves abnormal transverse dimension of the maxilla. Behaviour of the facial skeleton in its anterior-posterior dimension during treatment of maxillary narrowing using surgical assist is interesting to observe. Assessment of bone changes of the maxillary location assessed on lateral cephalograms and CBCT during surgically assisted maxillary expansion with bone-anchored appliances. The analysed material included documentation of 78 patients, the mean age of 16.86±2.65, treated with transverse maxillary distraction using a bone-anchored appliance. The software (Dolphin Imaging) was used to measure parameters on lateral cephalograms. Results obtained analysis of correlation between planes, angles and diameters between teeth before and after treatment. Simple Regression - SNA vs. SN-OCCL change of the anterior height with regard to changes in the occlusal angle refer to the opening and dropping of the maxilla in the anterior section. Simple Regression - SNA vs. S-PNS-ANS describe changes in the anterior section such as opening of the S-PNS-ANS angle, and in a correlation with the SNA angle opening it indicates maxillary dropping and protrusion. Dropping and protrusion of the maxilla can be observed during surgically assisted maxillary expansion with bone-anchored appliances. Maxillary anterior movement may depend on a surgical procedure.

Craniofacial and cutaneous findings in Noonan, Costello and LEOPARD syndromes.

Noonan, Costello and LEOPARD syndromes belong to a family of cardiofaciocutaneous disorders and share common genetic traits. As they are associated with a germline mutation in genes encoding proteins involved in RAS/MAPK, patients suffering from these syndromes are at a greater risk of cancer and abnormal myelopoiesis in infancy. Patients with cardio faciocutaneous syndromes share some clinically overlapping syndromes, therefore differential diagnosis can be problematic. In this paper we aim at demonstrating distinctive craniofacial and cutaneous manifestations of Noonan, Costello and LEOPARD syndromes which can be useful for clinicians who aim at treatment of children with rare diseases.