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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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. 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. 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. 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. 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. [ABSTRACT FROM AUTHOR]

Axenfeld-Rieger syndrome (ARS) is a rare autosomal disease manifested by a heterogeneity of symptoms. Symptoms include disorders of sight and mainly involve the iris, cornea and iridocorneal angle. The non-ocular symptoms involve the craniofacial region and the oral cavity. The patient presents with characteristic symptoms of the disorder including rare congenital ankylosis. The treatment of ankylosis is difficult as illustrated by the presented case. Computer assisted planning, custom surgical templates as well as temporomandibular joint implants and stabilizing plates were used for treatment planning and surgery. [ABSTRACT FROM AUTHOR]

Axenfeld-Rieger syndrome (ARS) is a rare autosomal disease manifested by a heterogeneity of symptoms. Symptoms include disorders of sight and mainly involve the iris, cornea and iridocorneal angle. The non-ocular symptoms involve the craniofacial region and the oral cavity. The patient presents with characteristic symptoms of the disorder including rare congenital ankylosis. The treatment of ankylosis is difficult as illustrated by the presented case. Computer assisted planning, custom surgical templates as well as temporomandibular joint implants and stabilizing plates were used for treatment planning and surgery. [ABSTRACT FROM AUTHOR]

Currently, oral cancer is a major social problem, no less than other cancers. The surgical removal of lesions is the method of choice. The patients suffer from many psychological and physical problems after surgery. Their appearance and speech are significantly changed. The aim of this work was to present methods of nursing care for patients after surgery to remove cancer of the floor of the mouth, based on a case study. Thanks to accurate nursing diagnoses and the use of nursing interventions, the patient's condition improved both physically and psychiatric, more willingly cooperates with a therapeutic team, which in effect significantly affects his convalescence. [ABSTRACT FROM AUTHOR]

Background: Primary malignant bone tumors are most commonly associated with mutilating surgical procedures that can significantly disturb the motor development of a young patient and are frequently affiliated with major postoperative complications. Unfortunately, despite available autologous tissue donor sites, artificial materials are still most commonly used for the reconstruction of post-resection defects. Reconstructive microsurgery is increasingly recognized as an effective method of functional reconstruction, creating the possibility of performing limb-sparing surgery (LSS) with significant limitation of major postoperative complications at the same time. Methods: The study group consisted of 9 pediatric patients diagnosed with primary malignant bone tumor in the limb location. In order to perform microvascular reconstruction, 9 free fibula flaps were used in combination with a bone allograft (Capanna method). The functional outcome of the reconstruction was assessed on the basis of the MSTS (Musculoskeletal Tumor Society Scoring System) scale. Results: The presented analysis proves the effectiveness of this reconstructive procedure and the possibility of performing LSS with reasonable functional outcomes after appropriate patient qualification. In this study, all limbs included were spared. In all cases, the R0 surgical margins were achieved and no reports of local recurrences were reported during the follow-up. The average score on the MSTS scale was 27/30 points. Conclusions: Microvascular reconstructive surgery is an individually personalized and highly effective method of treating patients with primary bone tumors in the limb location and provides satisfactory functional outcomes. [ABSTRACT FROM AUTHOR]

Introduction Mandibular retrognathia is a common skeletal congenital dysgnathia. In many cases of skeletal class II patients require the surgical operation. Orthognatic surgery offers mandibular bilateral sagittal split osteotomy (BSSO) as the most common procedure to make the advancement of the mandible. However, the alternative, mandibular distraction osteogenesis (MDO), is prevalent nowadays and beneficial in particular cases. Aim The purpose of this study is to show the effect of MDO and BSSO done on 20 patients at Specialist Children's Hospital in Olsztyn, Poland between 2011 and 2013, performed by the same surgeon – KD. Authors would like to present the details of treatment planning and management of these methods as well as the protocol of usage of the distraction device. Material and methods The sample consisted of 74 lateral cephalometric X-rays. Criteria for cephalometric comparison were angular cephalometric variables: SNB and SN/GoGn (Steiner analysis). The criteria for inclusion into this study were as follows: males and females with skeletal class II pattern plus dentofacial and dental abnormalities like skeletal open bite. The mean age of the subjects was 17.9 years. Results and discussion Our comparison study showed that there was no statistically significant difference between results of BSSO postoperatively and MDO post-distraction. However, there is a need of long-term data on stability of both methods. Conclusions Study shows that MDO may offer another option for treatment of skeletal class II malocclusions in growing patients and after growth spurt. [ABSTRACT FROM AUTHOR]

Introduction A transverse maxillary hypoplasia is a deformation often observed in orthodontic patients. Various techniques are used to treat this problem. Aim The aim is to present results of transversal maxillary hypoplasia treatment with a bone-borne device. Material and methods A retrospective analysis included 27 patients, age 17–26 years (17 ± 2.6). Dental casts and X-ray were made before operation period (T1), and post distraction (T2). On the casts were determined points: on cusps of maxillary canines (3-3), first maxillary premolars (4-4) and first maxillary molars (6-6). Angles and distances were measured on standard posterior-anterior (PA) images. Results and discussion At the 3-3 level, the average expansion was 5.8 mm, at the 4-4 level the average expansion was 7.3 mm, at the level of 6-6 palatial cusps the average expansion was 6.11 mm. Measurements in the PA X-ray were performed at the nasal cavity, with the average dimensions being 29.03 mm before treatment, and 31.95 mm post-treatment. The angle was measured between first molars (6-6_ang) and the anterior nasal spine before and after treatment, with a significant change in that angle from 98.93° on average to 102.89° after distraction. Conclusions Use of maxillary distraction osteogenesis with bone-borne device in maxillary expansion is an effective treatment method. Maxillary expansion results in increase of the nasal cavity. Used distractor is easy to operate for the patient. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Highly severe malocclusion cause dysfunctions and deformation of facial features which result in low self-esteem. Treatment method of choice is combined orthodontic and surgical treatment. Time after surgery is difficult for the patients. Large swelling and sensory disturbances result in discomfort and anxiety. Those negative feelings can be minimized by appropriate patient's mental preparation before surgery and personalized care based on patient's needs after surgery. The aim of the study is to present a model of nursing a patient undergoing surgical correction of dental and occlusal abnormalities. [ABSTRACT FROM AUTHOR]

Purpose: The main aim of this paper was to perform the morphological assessment of children's mandibles of different etiology of dys-functions within the temporomandibular joint, from isolated idiopathic ankylosis to craniofacial malformations co-existing with genetic disorders. Methods: The investigations encompassed seven patients at the age of 0–3. Measurements were conducted on the basis of data obtained from computed tomography. In the patients' 3D models 12 characteristic anthropometric points were marked, on the basis of which the researchers calculated 9 linear dimensions, 3 angles and, further on, 5 indices defining the proportions of the mandible. Results: The comparisons of the measurements showed that the mandibles with malformations were smaller than obtained for healthy children. The values of the mandible angles (alpha) revealed high changeability among the examined children. It was also revealed the significant asymmetry between the right and left side. Conclusions: This work shows an innovative approach to the evaluation of anatomical malformations, whereas the developed indices enable an objective quantitative assessment of geometry, which can be applied to the analysis of the degree of the deformation severity. An in-depth analysis of the image diagnostics should be a standard procedure in the pre-operative planning of surgical treatment and the preparation of multi-specialist logopaedic, functional and orthodontic rehabilitation. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Introduction Facial nerve palsy is a condition with considerable consequences, particularly when it occurs early in life. It poses a serious clinical issue due to its initially unclear etiology, limited treatment options and outcomes, as well as its significant implications both functionally and aesthetically. In children with persistent congenital or acquired facial nerve palsy as a result of trauma or following surgical treatment of malignant neoplastic disease, the therapeutic strategy includes surgical techniques combined with intensive rehabilitation. In children with persistent facial nerve palsy, surgical techniques of dynamic facial reanimation may be considered in order to restore both static and dynamic facial symmetry. A frequently performed procedure is the transfer of functioning innervated muscle flaps. Material and Methods The study group comprised 6 pediatric patients aged 11-17 years diagnosed with persistent facial nerve palsy who were surgically treated to restore facial muscle function between 2018 and 2021. All patients included in the study were surgically treated with a free neurovascular flap from the gracilis muscle of the thigh (GF). The average follow-up period was 22 months (range: 15-29 months). The House-Brackmann facial nerve scoring system (HBGS) was applied in order to objectively assess the recovery of function in the grafted innervated muscle flap during treatment and in the course of postoperative recovery. Results At a mean follow-up period of 22 months, all cases demonstrated at least grade 3 according to the HBGS. Although static reconstruction options are acceptable in certain cases, in pediatric patients due to the extensive plasticity capacity of the brain, the benefits of performing correct functional reconstruction using an innervated free flap of the gracilis muscle of the thigh are substantial and should not be ignored. Conclusions Although further research should concentrate on functional, aesthetic and patient-reported outcomes, conducting studies on a large group of subjects may result in the development of a clinical algorithm which could simplify the assessment and effectively facilitate the treatment of facial nerve palsy in children. [ABSTRACT FROM AUTHOR]

Introduction: Class III malocclusion is a result of maxillary deficiency, mandibular prognathism or a combination of both, often accompanied by an anterior crossbite and a concave profile. Aim: The aim of this work was to report and analyze a case of class III malocclusion. Case study: The patient, a 12-year-old boy, attended Maxillo-Facial Clinic with the complaint of his lower jaw being in front. The pre-treatment examination showed a slight flattened subnasal area and the reverse overjet. For the maxillary protraction, de Clerck method of bone anchors and class III elastics were applied. After previous palatal expansion, the boneanchored maxillary protraction was applied. The surgery was performed under general anesthesia. The surgical procedure consisted in placing four miniplates - one in each infrazygomatic buttress of the maxilla, and one in the anterior mandible between, and inferior to the left and right permanent lateral incisor and canine. The miniplates were loaded 3 weeks after the surgical procedure. After 7 months of treatment, the anterior crossbite was corrected. Results and discussion: Cephalometric evaluation between the beginning of treatment and the end of maxillary protraction showed marked increase in ANB and Wits. A counterclockwise rotation of the mandible was observed, as well as a slight clockwise rotation of the maxillary bone. The obtained results correspond to de Clerck's cephalometric investigations in class III patients who were treated with the use of bone-anchored maxillary protraction. Conclusions: Temporary anchorage devices application enabled correction of the anterior crossbite and enhanced midfacial growth in young maxillary-deficient patient. [ABSTRACT FROM AUTHOR]

Introduction: Palatal suture expansion techniques are used to treat severe maxillary narrowing and lead to its base extending. The palatal suture expansion procedure was for the first time described by Angell in 1860. Aim: To review the palatal suture expansion methods, particularly surgically-assisted rapid maxillary expansion technique and indications for their choice as well as possible complications. Gum-teeth complications associated with the use of orthodontic-orthopedic devices and lack of their efficacy in adult patients contributed to designs invention directly fixed in the palatal bone. Discussion: Anatomic structure of palatal suture changes with age. Palatal suture ossification status determines the choice of one of the orthodontic-orthopedic methods of treatment, which include slow maxillary expansion (SME) or rapid maxillary expansion (RME), as well as the surgically-assisted rapid expansion of the maxillae (SARME/SARPE). In orthodontic-orthopedic methods of palatal suture expansion Haas and Hyrax devices are applied. In the method of surgically-assisted rapid palatal expansion (SARPE), a distractor is placed in the maxilla after Le Fort I osteotomy performance. Screws are a source of force for expanding devices both orthodontic-orthopedic and palatal distractors which can be activated by patients. It should be noted that surgically-assisted rapid maxillary expansion (SARME) is a relatively new method, and despite it giving great opportunities of stable maxillary expansion in adult patients, it also raises some concerns related to the surgical intervention and necessity for general anesthesia. Conclusions: SARME expansion technique has particular advantages which include the possibility of application among adult patients, no relapses, as well as the skeletal but not dentoalveolar expanding of maxilla. [ABSTRACT FROM AUTHOR]

Introduction: Apert syndrome is the genetically determined occurrence of several large congenital abnormalities that build up the clinical picture. It is a disease that occurs rarely. Aim of the study: Attempt to develop and present a comprehensive nursing care plan over a child with Apert syndrome, taking into account the patient's bio-psycho-social spheres and his family. Material and methods: An individual case study method was used in the work. Documentation analysis medical supervision, direct observation of the patient, an interview, and the necessary measurements were made vital signs were used to gather the information to research techniques. Results: The most important health problems of a patient with Apert syndrome are life-threatening as a result of increased intracranial pressure, apnoea, retention secretions in the airways, tracheostomy tube in the trachea, and difficulties with food intake. Conclusions: There is a risk that the child is excluded by their peers and society because of their different appearance. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Hemifacial microsomia is a common congenital craniofacial disorder. Facial symmetry, esthetics and function are affected. Patients diagnosed with this condition require multiple-stage reconstructive treatment of the facial skeleton and soft tissues. In a significant number of patients, the muscles of facial expression affected by the disease process are dysfunctional, resulting from the impairment of the supplying facial nerve. As microsurgical techniques developed in recent years, facial nerve resuscitation with the use of a free gracilis muscle flap transplant has been proposed. This article will present the case of a patient with hemifacial microsomia who successfully underwent facial resuscitation with the use of this procedure. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Introduction: There has been much debate in the literature regarding the preferred method of central giant cell granulom (CGCG) treatment. Non-surgical methods are widely accepted, including intralesional corticosteroid injections, calcitonin therapy and the rarely used interferon therapy but surgical excision is the preferred method of treatment. Aim: The objective of this study was to describe our experience in the surgical treatment of CGCG in the paediatric population. Material and methods: A case study of 9 paediatric patients with the average age of 10 years is discussed in this article. Most patients were diagnosed with mandibular CGCG lesions, and multifocal tumours localized in the mandible and the maxilla were noted in 2 cases. Results and discussion: The surgical procedures performed in our department included enucleation (2 patients), en bloc resection (2 patients) and segmental resection (5 patients). Segmental resections require further reconstruction. A surgically created defect is repaired with the involvement of frozen or autologous bone grafts, distraction osteogenesis and microvascular customized multiple tissue flaps. Conclusions: Our experience indicates that despite the use of a meticulous surgical technique, patients at risk of tumour recurrence have to remain under strict clinical observation. [ABSTRACT FROM AUTHOR]

Ankyloses in the area of the temporomandibular joint (TMJ) are mentioned as a potential etiological factor of mandibular growth disorders and facial asymmetry. The aim of this case study was to evaluate the changes in the mandible of a child with zygomatic-coronoid ankylosis during the first five years of life, in which two adhesion release procedures were performed. The adopted symmetrical approach is based on the assumption of symmetry of the structure of the stomatognathic system in relation to the sagittal median plane. However, the assessment of pathological changes in the structure of the skeletal system was performed using an asymmetrical approach. Computed tomography techniques and a system of computer-aided diagnosis (CAD) were used in the case study. During the child's growth, linear and angular measurements were made thrice (at the age of 16, 25 and 54 months). The degree of asymmetry was estimated in the measurements made on the right and left sides of the three-dimensional mandible. Unilateral congenital hypoplasia of the articular process and zygomatic-coronoid adhesion caused asymmetrical growth of the mandible in the child along with shortening of the mandibular branch and body on the damaged side and a visible difference in the size of the mandibular angles. Removal of the adhesions during surgical procedures made it possible to reduce the asymmetry of the mandible and catch-up growth, although at the age of five, the mandible was still smaller than the mandible in healthy peers. It was shown that the early adhesion release procedures supported by the CAD analysis enabled the restoration of mandibular symmetry. [ABSTRACT FROM AUTHOR]