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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]

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]

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. 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. 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. [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]

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]

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]

Extended postoperative mandibular reconstructions due to orofacial disease involving the temporomandibular joint (TMJ) in immature patients remain a challenge as a result of ongoing growth, which is usually affected by the disease and treatment. Current reconstructive techniques based fully on alloplastic total joint replacement fail to meet fully the anatomical and functional requirements for the masticatory system and speech development. Fourteen children aged 12.6 ± 2.6 with tumors or congenital deformities affecting the mandible and TMJ were included in the study. Radical surgical treatment according to our own protocol was performed through microvascular anastomotic flap reconstruction of the soft tissues and bones, together with total TMJ custom replacements. Follow-up lasted 2–6 years. During the follow-up, increases in the mandible body (13% growth) and ramus (12% growth) were observed, both of which were related (p < 0.001). This is the first report concerning the immediate reconstruction of the mandible with ramus and total TMJ in children and adolescents that combines a free vascularized graft and total individual prosthesis of the TMJ. The presented technique enabled optimal function of the TMJ, growth of the mandible, and further rehabilitation of the patients. The technique was demonstrated to be safe, reliable, and provide good functional and cosmetic outcomes. [ABSTRACT FROM AUTHOR]

Introduction: 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. Material and method: 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. Results: 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. Conclusions: 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. [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]

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]