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PURPOSE: The aim of this study was to analyze the predictability of virtual surgical planning of mandibular reconstruction by osteogenic distraction (OD) with a custom-made osteogenic distraction device (DEOS) after ballistic trauma and to make recommendations to mitigate potential sources of discrepancy. METHODS: This retrospective study involved 12 patients who were victims of facial mandibular ballistic trauma and were reconstructed with osteogenic distraction with a DEOS device. Postoperative images were compared to the planned situation by means of several measures made in two plans : distraction plane and frontal plane. RESULTS: The mean bone loss was 54.2mm. The anteroposterior difference was systematically inferior or equal postoperatively (10.8mm). The interrami angle difference in the frontal plane was positive, with an average of 4.8°. There was a significant negative correlation in univariate analysis between the antero-posterior difference measurement and the difference of the interrami angle in distraction. CONCLUSION: A significant difference between the planning and postoperative results was shown, explained by the device being too adjustable leading to the loosening of the locking screws of the device. A static analysis showed that there are significant rotational forces at the carriages, which can lead to the loosening of the locking elements. It is then proposed that the device be optimized by customization of some elements.
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Traumatismos Faciales , Reconstrucción Mandibular , Osteogénesis por Distracción , Humanos , Estudios Retrospectivos , Mandíbula/cirugía , Reconstrucción Mandibular/métodos , Osteogénesis por Distracción/métodosRESUMEN
Congenital sinuses of the upper lip are rare congenital malformations. There have been only 40 cases described in the literature. We report 3 cases of congenital midline upper lip sinus in Caucasian children. Two of those lesions were associated with other anomalies (complete cleft palate and hemifacial macrosomia or submucous cleft palate with bifid uvula). The pathophysiology remains unexplained yet. Congenital upper lip sinuses can be considered as possible microforms of cleft-lips. Associated anomalies are frequent and must be sought. The treatment is a full excision of the sinus tract and of the skin around the punctum in order to avoid risks of recurrence.
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Labio Leporino/patología , Fisura del Paladar/patología , Fístula/congénito , Enfermedades de los Labios/congénito , Anomalías Múltiples , Niño , Preescolar , Labio Leporino/cirugía , Fisura del Paladar/cirugía , Femenino , Fístula/cirugía , Humanos , Recién Nacido , Enfermedades de los Labios/cirugía , MasculinoRESUMEN
We report a case of microcystic adnexal carcinoma in a 80-year-old woman. This is a rare malignant adnexal cutaneous tumor with glandular and follicular differentiation, rare, often asymptomatic, with late diagnosis and slow growth, locally aggressive and rarely metastatic. The distinction with other benign and malignant skin tumors is difficult.
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Carcinoma de Apéndice Cutáneo/patología , Neoplasias de los Labios/patología , Anciano de 80 o más Años , Carcinoma de Apéndice Cutáneo/diagnóstico , Carcinoma de Apéndice Cutáneo/cirugía , Diagnóstico Diferencial , Femenino , Humanos , Neoplasias de los Labios/diagnóstico , Neoplasias de los Labios/cirugía , ReoperaciónRESUMEN
PURPOSE: Maxillomandibular ossifying fibroma is a benign tumor that affects young adults. Complete excision can allow satisfactory management with no recurrence. During a humanitarian mission, one is confronted with many types of damage from these fibromas. Their management requires wide resection (mandibulectomy interrupter or maxillectomy) and free flap reconstruction. However, technical conditions during a humanitarian mission might not allow the performance of a free flap reconstruction. How can such patients be managed? Should these patients receive a straightforward intervention performed on site during the mission or should they go to another country with a technical platform suitable for microsurgical reconstruction? PATIENTS AND METHODS: During a humanitarian mission in Ouagadougou, Burkina Faso, 6 patients with large ossifying fibromas traveled to France to undergo wide excision of the lesion and free flap reconstruction using the fibula. The Enfants du Noma paid for the travel and medical costs. RESULTS: No flap was lost. Four patients (67%) had local (disunity of scar or local infection) or general (malaria) complications that quickly resolved. CONCLUSIONS: Most teams agree that free flaps should not be performed during humanitarian missions, and only 1 German team practices in Sokoto, Nigeria. Therefore, medical travel is an attractive solution that allows optimal management and requires financial assistance from humanitarian organizations.
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Enfermedades del Desarrollo Óseo/cirugía , Enfermedades Maxilomandibulares/cirugía , Adolescente , Adulto , Enfermedades del Desarrollo Óseo/diagnóstico por imagen , Enfermedades del Desarrollo Óseo/patología , Burkina Faso , Niño , Humanos , Enfermedades Maxilomandibulares/diagnóstico por imagen , Enfermedades Maxilomandibulares/patología , Masculino , Enfermedades Mandibulares/diagnóstico por imagen , Enfermedades Mandibulares/patología , Enfermedades Mandibulares/cirugía , Reconstrucción Mandibular/métodos , Enfermedades Maxilares/diagnóstico por imagen , Enfermedades Maxilares/patología , Enfermedades Maxilares/cirugía , Misiones Médicas , Tomografía Computarizada por Rayos X , Adulto JovenRESUMEN
Inflammatory myofibroblastic tumors (IMT) are rare tumors in children and young adults, considered by the World Health Organization to be intermediate malignancies and rarely metastasizing, with the presence of an anaplastic lymphoma kinase rearrangement in about 50% of the cases. We report the case of a teenager who presented with a metastatic aggressive IMT that was life-threatening despite multiple treatments, and which responded repeatedly to anaplastic lymphoma kinase-targeted crizotinib therapy. Crizotinib induced drastic primary tumor regression, which was sufficient to allow surgical resection and to control distant disease. This case shows that crizotinib is a promising therapy in IMT, even in adolescents and young adults.
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Inflamación , Metástasis de la Neoplasia , Neoplasias de Tejido Muscular/tratamiento farmacológico , Adolescente , Quinasa de Linfoma Anaplásico , Crizotinib , Femenino , Reordenamiento Génico , Humanos , Neoplasias de Tejido Muscular/patología , Neoplasias de Tejido Muscular/cirugía , Inhibidores de Proteínas Quinasas/uso terapéutico , Pirazoles/administración & dosificación , Pirazoles/uso terapéutico , Piridinas/administración & dosificación , Piridinas/uso terapéutico , Proteínas Tirosina Quinasas Receptoras/antagonistas & inhibidores , Proteínas Tirosina Quinasas Receptoras/genéticaRESUMEN
The purpose of this study was to determine the stiffness of mandibular soft tissues during mandibular distraction, from the perspective of improving distraction devices such as automated continuous distractors. Uncompleted osteotomy was performed on 11 fresh human hemimandibles via a greenstick fracture, to preserve the uplift of the internal periosteum of the mandibular corpus. In order to replicate continuous distraction, direct measurements were performed through a uniaxial quasi-static tensile test. For all specimens, linear regression was applied to the force-displacement curve for a force region of 10-20 N, and the slope extracted. The mean stiffness was estimated to be 9.12 ± 3.56 N/mm. This study is the first to measure directly the stiffness of the surrounding tissues of the human mandibular corpus, and paves the way to the design of a new generation of distractor devices.
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Mandíbula , Osteogénesis por Distracción , Humanos , Osteogénesis por Distracción/instrumentación , Osteogénesis por Distracción/métodos , Mandíbula/cirugía , Fenómenos Biomecánicos , Resistencia a la Tracción , Estrés Mecánico , Cadáver , Masculino , Persona de Mediana Edad , Femenino , AncianoRESUMEN
OBJECTIVE: In bilateral cleft lip cases, surgeons have long known that the medial nasal prominence or prolabium is devoid of muscle. Despite cheiloplasty restoring orbicular muscle continuity, moustache hair growth is less developed on the philtral skin. We sought to understand this hair disparity. BASIC PROCEDURES: The authors conducted a comprehensive literature search using reference materials on the physiological and pathological development of the face in utero and the PubMed database using the keywords 'embryology', 'bilateral cleft lip' and 'hair'. MAIN RESULTS: In bilateral cleft lip, the normal fusion between the maxillary and intermaxillary process does not occur, and the migration of cranial neural crest cells (CNCC) is disturbed. CNCCs from different locations and with different myoblastic differentiation potential are arrested on each side of the cleft. Therefore, a reduced concentration of myoblasts and myocytes as well as hormone receptors in the medial process could hinders the normal physiological development of the different layers of the philtral skin and especially the skin appendages. CONCLUSION: Philtral hair sparsity in patients with bilateral cleft lip can be explain by a defect in the migration of CNCCs to the prolabium, whose cells are not able to develop hair follicles, due to a disruption of the exchange of Wnt/ß-catenin, EDA, and or Noggin signals and due to the absence of androgen receptor.
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Labio Leporino , Procedimientos de Cirugía Plástica , Humanos , Labio Leporino/diagnóstico , Labio Leporino/cirugía , Labio/anomalías , Labio/patología , Labio/cirugía , CabelloRESUMEN
BACKGROUND: Skin tumors are common. Recommended treatment in most cases is surgery, with margins adapted. Except in the case of simple resection and suture, it is necessary to know the status of the margins before reconstructing the defect. A one-stage technique is possible with frozen section analysis, which gives the surgeon an intraoperative assessment of resection quality. The aim of our work is to study the reliability of the frozen section procedure. METHOD: A retrospective study included 689 patients who underwent surgery for skin tumor (excluding melanoma) between January 2011 and December 2019 at the University Hospital of Caen, France. RESULTS: In 639 patients (92.75%), the frozen section analysis found healthy margins. There were 21 cases of discrepancy between the frozen section analysis and final histology. Infiltrating and scleroderma-like basal cell carcinomas showed a significantly higher frequency of affected margins on frozen section analysis (p < 0.001). The tumor size and location played a significant role in the margin status. CONCLUSION: In our department, the frozen section procedure is the reference examination indicating immediate flap reconstruction. The present study demonstrated its interest and overall reliability. However, it is to be used according to histologic type, size, and location.
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Melanoma , Neoplasias Cutáneas , Humanos , Secciones por Congelación/métodos , Estudios Retrospectivos , Reproducibilidad de los Resultados , Neoplasias Cutáneas/cirugía , Neoplasias Cutáneas/patología , Melanoma/cirugíaRESUMEN
Background and Objectives: Ear cartilage malformations are commonly encountered problems in reconstructive surgery, since cartilage has low self-regenerating capacity. Malformations that impose psychological and social burden on one's life are currently treated using ear prosthesis, synthetic implants or autologous flaps from rib cartilage. These approaches are challenging because not only they request high surgical expertise, but also they lack flexibility and induce severe donor-site morbidity. Through the last decade, tissue engineering gained attention where it aims at regenerating human tissues or organs in order to restore normal functions. This technique consists of three main elements, cells, growth factors, and above all, a scaffold that supports cells and guides their behavior. Several studies have investigated different scaffolds prepared from both synthetic or natural materials and their effects on cellular differentiation and behavior. Methods and Results: In this study, we investigated a natural scaffold (alginate) as tridimensional hydrogel seeded with progenitors from different origins such as bone marrow, perichondrium and dental pulp. In contact with the scaffold, these cells remained viable and were able to differentiate into chondrocytes when cultured in vitro. Quantitative and qualitative results show the presence of different chondrogenic markers as well as elastic ones for the purpose of ear cartilage, upon different culture conditions. Conclusions: We confirmed that auricular perichondrial cells outperform other cells to produce chondrogenic tissue in normal oxygen levels and we report for the first time the effect of hypoxia on these cells. Our results provide updates for cartilage engineering for future clinical applications.
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INTRODUCTION: Few articles discuss the management of a free fibula flap (FFF) necrosis in maxillofacial reconstruction. MATERIAL & METHODS: Between 2005 and 2020, 170 FFF were used for maxillofacial reconstruction at the University Hospital of Caen, among which 16 cases suffered total necrosis and flap removal. The authors categorized these 16 cases into three groups based on the proposed salvage treatment and analyzed the post-operative follow-up, including complications, length of hospital stay and delay in radiotherapy. RESULTS: In Group A, two patients underwent immediate reconstruction with a new free flap. There were no postoperative complications, and the average hospitalization duration after removal of the first flap was 10 days. In Group B, eleven patients underwent reconstruction with a pedicled musculocutaneous pectoralis major flap. These patients experienced numerous complications, with 73% of them requiring at least one additional surgery, and all of them had an indication for later FFF reconstruction. The average hospitalization duration in this group was 37 days. In Group C, three patients underwent conservative management with debridement and mucosal closure. Two of them experienced complications, and all of them underwent later FFF reconstruction. The average hospitalization duration in this group was 15 days. DISCUSSION: Based on our experience and the literature review, the use of an immediate new free flap appears to be the best approach after the removal of a FFF. This generates shorter healing times and shorter hospitalization stays, and this allows better results in terms of function and aesthetics. At least, this is the approach that causes the least delay for radiation therapy if indicated. The other approaches should only be proposed in case of patient's poor general condition or in case of refuse of the patient.
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Colgajos Tisulares Libres , Procedimientos de Cirugía Plástica , Humanos , Peroné/cirugía , Necrosis/etiología , Necrosis/cirugía , Complicaciones Posoperatorias/epidemiología , Complicaciones Posoperatorias/etiología , Complicaciones Posoperatorias/cirugía , Cicatrización de HeridasRESUMEN
Hallermann Streiff syndrome (HSS) is a rare congenital abnormality with about 200 case reports in the literature. Its etiology is unknown although it may be due to a sporadic mutation. Diagnosis is based on the association of craniofacial malformation, dental abnormalities, hypotrichosis, atrophy of the skin, proportionate nanism, congenital cataract and bilateral microphtalmos. Cranio-facial deformities are the main signs detected and the most easily recognizable. We report cranio-facial and oral signs from a systemic literature review, and illustrate our findings with two of our patients diagnosed with HSS. Common cranio-facial manifestations are craniofacial malformation with a « parrot beak ¼ nose, micrognathia, aprominent skull, sutures closing anomaly, malocclusion, dental anomalies, eyebrows and eyelash lack and atrophy of the nose skin. Knowledge of these signs should allow for early diagnosis and adequate treatment and follow up.
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Síndrome de Hallermann , Maloclusión , Atrofia/complicaciones , Cara , Síndrome de Hallermann/complicaciones , Síndrome de Hallermann/diagnóstico , Síndrome de Hallermann/cirugía , Humanos , CráneoRESUMEN
OBJECTIVES: The objective of this study was to evaluate the efficiency of three methods of isolated mandibular fracture intraoperative reduction. MATERIALS AND METHODS: This 6-year retrospective study included patients with isolated extra-articular mandibular fractures who would benefit from osteosynthesis. The endpoint was postoperative occlusion according to the type of intraoperative immobilization: screws, arch, or manual reduction. RESULTS: A total of 145 patients were included, with 233 fractures. Forty-five patients underwent manual reduction without maxillo-mandibular fixation (MMF), 51 MMF with screws, and 49 MMF with arch, with 11.1%, 5.9% and 4.1% of patients in these groups experiencing postoperative malocclusion, respectively. The overall malocclusion rate was 6.9%. There was no significant difference among the 3 methods according to univariate statistical analysis (p = 0.42) or after comparing MMF (grouping screws and arches) to manual reduction without MMF (p = 0.29). CONCLUSION: This study did not show a significant difference between the different methods of intraoperative reduction of isolated extra-articular mandibular fractures, even though intraoperative MMF was much more commonly used for complex fractures. However, there is a non-significant tendency to get a better post-operative occlusal result with MMF, which remains the reference traitement. Intraoperative manual reduction without MMF may be used within trained teams in some instances.
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Maloclusión , Fracturas Mandibulares , Fijación Interna de Fracturas/métodos , Humanos , Técnicas de Fijación de Maxilares , Fracturas Mandibulares/cirugía , Estudios RetrospectivosRESUMEN
The main objective of this study was to evaluate long-term stability of rigid osteosynthesis in the context of advancement genioplasty. Bone stability was defined as a long-term bone loss of less than 2 mm. Measurements were performed on lateral cephalograms, in the sagittal and vertical planes, at three times: preoperative (T0, less than one month before surgery), early postoperative (T1, at least one month post-operatively) and late postoperative (T2, at least one year after surgery). 25 patients were included in the study, with a mean follow-up of 3.47 years (range 1-9.42 years). The mean sagittal bone advancement at T1 was 4.06 mm ± 1.34, with a bone loss of 0.65 mm at T2 (p = 0.001). The mean vertical bone movement was 1.25 mm ± 0.47 at T1, with a relapse at T2 of 0.34 mm (p = 0.27). The soft-to-hard tissue ratio was 78% in the sagittal plane. Rigid osteosynthesis offers long-term stability, with very little change in clinical outcome, in advanced genioplasty.
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Mentoplastia , Avance Mandibular , Cefalometría , Fijación Interna de Fracturas , Humanos , Estudios RetrospectivosRESUMEN
INTRODUCTION: The vessel-depleted neck situation is a challenge for the surgeon in search of suitable recipient vessels for microvascular reconstruction of the head and neck. The internal thoracic vessels (ITVs) have proven useful as "rescue" recipient vessel resource. The objective of this report is to assess the feasibility of using ITVs by rerouting the pedicle for free flap reconstruction of the head and neck by comparing two different approaches. MATERIAL AND METHODS: Two surgical approaches were assessed: the classical one is parasternal, but cardiac surgeons commonly use median sternotomy. We conducted an anatomical study, comparing on the same subject the lengths and diameters of both internal thoracic artery (ITA) and internal thoracic vein (ITV) at the sixth, fifth, and fourth intercostal spaces (ICSs) through parasternal approach on one side and by median sternotomy on the other side. RESULTS: The study was performed on 13 subjects. We found a superiority of length of the ITVs with the median sternotomy approach. Regarding the mean length of the ITA, the sternotomy approach allowed a significant greater length with 119/89/67 mm, compared with parasternal approach with 91/62/42 mm, respectively at the sixth, fifth, and fourth ICS (p<0.001). Similarly, we observed a significant greater length of the ITV with 116/85/63 mm versus 89/62/42 mm (p<0.001). The mean arterial and venous diameters were 2.9 mm and 2.1 mm in the sixth ICS, 3.3 mm and 2.3 mm in the fifth ICS, and 3.9 mm and 2.9 mm in the fourth ICS, respectively. CONCLUSION: These results help to guide the choice of surgical approach and the level of harvesting.
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Colgajos Tisulares Libres , Arterias Mamarias , Procedimientos de Cirugía Plástica , Anastomosis Quirúrgica/métodos , Colgajos Tisulares Libres/irrigación sanguínea , Humanos , Arterias Mamarias/cirugía , Cuello/irrigación sanguínea , Cuello/cirugía , Procedimientos de Cirugía Plástica/métodosRESUMEN
INTRODUCTION: Orbital floor fracture repair is a complex surgery with intra-orbital hematoma being the most feared complication as it can lead to visual loss if not treated in good time. This is why currently patients are monitored for almost 48 hours as inpatients. The purpose of this study was to find out if orbital floor repair surgery could be safely undertaken as a day case by reviewing the experience of the last 11 years at the Caen University Hospital. MATERIALS AND METHODS: A retrospective, monocentric study was conducted at the Caen University Hospital. All patients undergoing orbital floor reconstruction in a trauma setting from January 2008 to December 2019 were included. RESULTS: Of the 130 included patients, none presented a post-operative complication such as intra-orbital hematoma. 3 patients had their surgery performed as a day case. DISCUSSION: In the literature, more and more surgeons are proposing orbital floor fracture repair to be undertaken as day case. Indeed, the theoretical risk of intra-orbital hematoma is greater within the first 6 hours after surgery and can persist up to 10 days postoperatively. Provided patients meet the classic criteria for outpatient surgery, and are provided with a precise post-operative care protocol. Under these conditions, orbital floor fractures may be repair in ambulatory surgery.
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Procedimientos Quirúrgicos Ambulatorios , Fracturas Orbitales , Humanos , Procedimientos Quirúrgicos Ambulatorios/efectos adversos , Estudios Retrospectivos , Tomografía Computarizada por Rayos X , Fracturas Orbitales/diagnóstico , Fracturas Orbitales/cirugía , Hematoma/etiología , Hematoma/cirugíaRESUMEN
INTRODUCTION: Bilateral cleft lip surgery recently evolved with the generalization of cheilorhinoplasty replacing cheiloplasty alone. The aim of this study was to analyze lip and nasal deformities of bilateral cleft lip operated on with the Le Mesurier procedure, and then to evaluate possible secondary surgical solutions used in our Cleft Center. PATIENTS AND METHODS: We retrospectively reviewed a total of 39 patients born between 1991 and 2009 with a bilateral cleft all type included (isolated lip, lip and alveolar, associated or not with a cleft palate), all treated at the Caen University Hospital with a Le Mesurier procedure for the primary cleft lip closure. We studied population characteristics, the number of secondary surgical procedures for lip and nasal deformities. We analysed therapeutic approaches of the different secondary surgical procedures used and their morphological results. RESULTS: 85% of patients needed at least one secondary surgical procedure, 28% two procedures, 15% three procedures and 8% four or more procedures. Median age at secondary surgery was 6,8. 79% had a "scar repositioning", 33% managed the vermilion, 18% a restoration of the volume of the lip, 12% a philtral tissue sacrifice, 9% a procedure on Cupid's bow, 24% a surgery for nasal tip projection, 45% a columella lengthening and 58% a reposition of the alar base. DISCUSSION: Le Mesurier procedure for bilateral clefts leads to deformities that require secondary surgical procedures. We propose an algorithm to manage loop scar in this procedure.
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Advanced mandibular osteoradionecrosis requires a segmental mandibulectomy with reconstruction using a free fibular flap. The conservation of labiomental sensation by lateralization of the inferior alveolar nerve is unusual during a segmental mandibulectomy. A protocol for the evaluation of labiomental sensation has been created for the clinical follow-up of operated patients. The patients included were patients with mandibular osteoradionecrosis who underwent a segmental mandibulectomy with reconstruction by free fibular flap and whose pre-operative labiomental sensation was preserved. All patients were followed-up by the same examiner and operated on by the same surgeon. The neuro-sensitive examination analyses the different forms of sensation at 1 week and at 12 months postoperatively. Between May 2017 and May 2018, 3 consecutive patients were assessed. The results of the labiomental sensitive evaluation using our evaluation protocol attest to the preservation of labiomental sensation. The operating time was increased by an average of 35 min per surgical procedure. The conservation and re-routing of the inferior alveolar nerve in segmental mandibulectomy with fibula free flap reconstruction in patients with osteoradionecrosis allows for the preservation of labiomental sensation, which improves the quality of life of patients. Our protocol can be used in all surgical procedure that affect sensibility.
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Osteorradionecrosis , Humanos , Nervio Mandibular/cirugía , Osteotomía Mandibular , Osteorradionecrosis/diagnóstico , Osteorradionecrosis/cirugía , Calidad de Vida , Estudios Retrospectivos , SensaciónRESUMEN
Histone methyltransferase EZH2 is upregulated during osteoarthritis (OA), which is the most widespread rheumatic disease worldwide, and a leading cause of disability. This study aimed to assess the impact of EZH2 inhibition on cartilage degradation, inflammation and functional disability. In vitro, gain and loss of EZH2 function were performed in human articular OA chondrocytes stimulated with IL-1ß. In vivo, the effects of EZH2 inhibition were investigated on medial meniscectomy (MMX) OA mouse model. The tissue alterations were assayed by histology and the functional disabilities of the mice by actimetry and running wheel. In vitro, EZH2 overexpression exacerbated the action of IL-1ß in chondrocytes increasing the expression of genes involved in inflammation, pain (NO, PGE2, IL6, NGF) and catabolism (MMPs), whereas EZH2 inhibition by a pharmacological inhibitor, EPZ-6438, reduced IL-1ß effects. Ex vivo, EZH2 inhibition decreased IL-1ß-induced degradation of cartilage. In vivo, intra-articular injections of the EZH2 inhibitor reduced cartilage degradation and improved motor functions of OA mice. This study demonstrates that the pharmacological inhibition of the histone methyl-transferase EZH2 slows the progression of osteoarthritis and improves motor functions in an experimental OA model, suggesting that EZH2 could be an effective target for the treatment of OA by reducing catabolism, inflammation and pain.
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Cartílago Articular/patología , Proteína Potenciadora del Homólogo Zeste 2/genética , Osteoartritis/patología , Anciano , Anciano de 80 o más Años , Animales , Benzamidas/farmacología , Compuestos de Bifenilo/farmacología , Cartílago Articular/efectos de los fármacos , Condrocitos/efectos de los fármacos , Condrocitos/fisiología , Modelos Animales de Enfermedad , Proteína Potenciadora del Homólogo Zeste 2/antagonistas & inhibidores , Proteína Potenciadora del Homólogo Zeste 2/metabolismo , Regulación de la Expresión Génica , Humanos , Interleucina-1beta/farmacología , Masculino , Ratones Endogámicos C57BL , Persona de Mediana Edad , Morfolinas/farmacología , Factor de Crecimiento Nervioso/metabolismo , Técnicas de Cultivo de Órganos , Piridonas/farmacologíaRESUMEN
PURPOSE: Craniosynostoses are managed by surgical and anaesthetic teams in specialist centres. Despite the availability of international guidelines, the perioperative management of craniosynostoses remains highly variable between centres. The aim of our study was to describe the different protocols for the management of non-syndromic craniosynostoses in France in 2015. MATERIALS AND METHODS: This descriptive study consisted of a survey about the protocols of pre-operative, per-operative and post-operative management of craniosynostoses. The survey was sent to the departments in French university hospitals that perform this surgery. RESULTS: Nineteen departments out of twenty replied to the survey. Sixteen departments organised multi-disciplinary meetings. The most frequent preoperative imaging requested was a Computed Tomography. More than half of the centres organised a follow-up until early adulthood. CONCLUSION: This study showed a great variability in the management of craniosynostoses. A recommendation from the study is to establish a scientific committee of practitioners in order to establish a standardised protocol. In addition, this study showed the need to create a specific section in the French rare diseases database (CeMaRa) for craniosynostoses.