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INTRODUCTION: Neuromuscular scoliosis (NMS) is associated with an abnormal muscle tone. Traditional conservative treatments, with the historical practice of early posterior fusion, have proven ineffective. Recently, growth-sparing techniques have gained traction owing to their ability to maximize trunk height. However, these techniques have a substantial risk of complications, particularly rod breakage, with reported incidence rates ranging from 15 % to 42 %. The objective of this study was to conduct a descriptive analysis of NMS patients who experienced rod breakage following the minimally invasive fusionless surgery (MIFS) technique. METHODS: This was a single-center, retrospective study that included all NMS patients who underwent surgery between January 2015 and January 2021 and subsequently presented with rod breakage after MIFS. The MIFS technique is based on proximal fixation with double hook claws made of pedicular and -sus laminar hooks and pelvic fixation with iliosacral screws. RESULTS: The mean follow-up was 5.2 ± 2.2 years. The mean dominant etiology of NMS was cerebral palsy (67 %). Of the 217 patients who underwent surgery, 15 (6.9 %) developed rod breakage. Rod breakage occurred 2.7 ± 1.3 years after the initial surgery. Four cases of rod fracture recurrence were reported in ambulatory patients with dystonia or hyperactivity. CONCLUSION: Compared with other fusionless techniques, the minimally invasive bipolar technique appears promising for patients with NMS, with a lower rate of rod breakage. We recommend the use of a four-rod construct for ambulatory patients or for those with dystonia or hyperactivity.
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BACKGROUND AND AIMS: Alveolar soft part sarcoma (ASPS) is an ultra-rare chemo-resistant sarcoma in children, occurring preferentially in young adults. We aimed to describe and compare its clinical presentation and behaviour in children and young adults to determine whether the same therapeutic strategy should be addressed for both populations. METHODS: National retrospective multicentre study of children (0-18 years) vs. young adults (19-30 years) included in the "ConticaBase" sarcoma database, treated for ASPS between 2010 and 2019 with pathology reviewed via the NETSARC + network. RESULTS: Overall, 45 patients were identified, 19 children (42%) and 26 young adults (58%). All ASPS diagnoses were confirmed with TFE3 rearrangement by immunohistochemistry or FISH. All clinical characteristics were balanced between both populations with frequent metastases at diagnosis (8/19 vs. 10/26). The therapeutic strategy was based on surgery (17/19 vs. 21/26), radiotherapy (8/19 vs. 12/26) ± systemic treatment (8/19 vs. 9/26). In patients with initially localized disease, metastatic relapse occurred only in adults (8/16), whereas metastatic progression was present in both metastatic groups (5/8 vs. 8/10). After a median follow-up of 5.2 years (range, 0.2-12.2), 5-year EFS was 74% [95%CI, 56-96] vs. 47% [30-74] (p = 0.071) respectively, and 5-year OS was 95% [85-100] vs. 85% [70-100] (p = 0.84). For localized tumours, 5-year MFS was 100% [100-100] vs. 60% [39-91] (p = 0.005). The 5-year OS of all patients with metastasis at diagnosis was 80.2% (62.2%-100%). CONCLUSIONS: ASPS appears to have the overall same clinical characteristics, but a more aggressive behaviour in young adults than in children. However, despite frequent metastases at diagnosis, long-term survival is high in both groups. Overall, the same therapeutic strategies may be considered for both populations.
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Congenital pseudarthrosis of the tibia (CPT) is a severe pathology marked by spontaneous bone fractures that fail to heal, leading to fibrous nonunion. Half of patients with CPT are affected by the multisystemic genetic disorder neurofibromatosis type 1 (NF1) caused by mutations in the NF1 tumor suppressor gene, a negative regulator of RAS-mitogen-activated protein kinase (MAPK) signaling pathway. Here, we analyzed patients with CPT and Prss56-Nf1 knockout mice to elucidate the pathogenic mechanisms of CPT-related fibrous nonunion and explored a pharmacological approach to treat CPT. We identified NF1-deficient Schwann cells and skeletal stem/progenitor cells (SSPCs) in pathological periosteum as affected cell types driving fibrosis. Whereas NF1-deficient SSPCs adopted a fibrotic fate, NF1-deficient Schwann cells produced critical paracrine factors including transforming growth factor-ß and induced fibrotic differentiation of wild-type SSPCs. To counteract the elevated RAS-MAPK signaling in both NF1-deficient Schwann cells and SSPCs, we used MAPK kinase (MEK) and Src homology 2 containing protein tyrosine phosphatase 2 (SHP2) inhibitors. Combined MEK-SHP2 inhibition in vivo prevented fibrous nonunion in the Prss56-Nf1 knockout mouse model, providing a promising therapeutic strategy for the treatment of fibrous nonunion in CPT.
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Ratones Noqueados , Neurofibromina 1 , Proteína Tirosina Fosfatasa no Receptora Tipo 11 , Seudoartrosis , Células de Schwann , Animales , Femenino , Humanos , Masculino , Ratones , Diferenciación Celular/efectos de los fármacos , Fibrosis , Quinasas de Proteína Quinasa Activadas por Mitógenos/metabolismo , Quinasas de Proteína Quinasa Activadas por Mitógenos/antagonistas & inhibidores , Neurofibromatosis 1/patología , Neurofibromatosis 1/metabolismo , Neurofibromatosis 1/complicaciones , Neurofibromina 1/metabolismo , Neurofibromina 1/genética , Proteína Tirosina Fosfatasa no Receptora Tipo 11/metabolismo , Proteína Tirosina Fosfatasa no Receptora Tipo 11/antagonistas & inhibidores , Seudoartrosis/patología , Seudoartrosis/metabolismo , Seudoartrosis/congénito , Células de Schwann/metabolismo , Células de Schwann/efectos de los fármacos , Células de Schwann/patología , Células Madre/metabolismo , Células Madre/efectos de los fármacos , Tibia/patologíaRESUMEN
BACKGROUND: The induced membrane technique is now widely used for pediatric diaphyseal bone loss due to various etiologies. Although consolidation rates remain satisfactory, complications, and healing delays may occur requiring additional procedures. We studied a series of induced membrane bone reconstructions in which the second stage included an embedded endomembranous non vascularized fibular shaft, in addition to iliac bone grafts. The purpose of this study was to analyze the results in terms of bone consolidation and complications. METHODS: This is a retrospective comparative and multicentric study of 32 children with large bone loss treated with the induced membrane reconstruction technique. Patients were divided into 2 groups according to the graft used during the second stage. The first group (G1) of 16 patients had a nonvascularized fibula embedded inside the membrane in addition with the corticocancellous grafts from the iliac crest. The second group (G2) of 16 patients underwent reconstruction using the original technique, with iliac crest graft only. RESULTS: The 2 groups were similar in terms of etiologies of bone loss and follow-up (mean: 44 mo for G1 and 49 mo for G2). Mean bone losses were 15.4 cm (range: 2 to 25; SD: 5.6) for G1 and 10.6 cm (range: 3 to 19; SD: 5.2) for G2. In the first group, all patients healed primarily, with a mean time of 5.9 months (range: 4 to 8; SD: 1.6). In the second group, 2 of 16 patients did not healed; for the others 14, healing mean time was 6.9 months (range: 3 to 12; SD: 2.7). The short-term and long-term complications rates were 38% to 19% for G1 and 50% to 31% for G2, respectively. Regarding the donor site, the fibulas reconstructed spontaneously with a mean time of 4.8 months (range: 3 to 6; SD: 1.2). CONCLUSIONS: The integration of a nonvascularized fibula during the second stage of the induced membrane technique appears to improve the consolidation rate in the pediatric population. LEVEL OF EVIDENCE: Level III-Retrospective comparative study.
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Trasplante Óseo , Peroné , Ilion , Humanos , Niño , Estudios Retrospectivos , Trasplante Óseo/métodos , Masculino , Femenino , Ilion/trasplante , Peroné/trasplante , Adolescente , Estudios de Seguimiento , Preescolar , Resultado del Tratamiento , Procedimientos de Cirugía Plástica/métodos , Diáfisis/cirugíaRESUMEN
OBJECTIVES: There are no established criteria for stiffness after fusionless surgery for neuromuscular scoliosis (NMS). As a result, there is no consensus regarding the surgical strategy to propose at long-term follow-up. This study reports the first use of shear wave elastography for assessing the mechanical response of lumbar intervertebral discs (IVDs) after fusionless bipolar fixation (FBF) for NMS and compares them with healthy controls. The aim was to acquire evidence from the stiffness of the spine following FBF. PATIENTS AND METHODS: Nineteen NMS operated on with FBF (18 ± 2y at last follow-up, 6 ± 1 y after surgery) were included prospectively. Preoperative Cobb was 89 ± 20° and 35 ± 1° at latest follow-up. All patients had reached skeletal maturity. Eighteen healthy patients (20 ± 4 y) were also included. Shear wave speed (SWS) was measured in the annulus fibrosus of L3L4, L4L5 and L5S1 IVDs and compared between the two groups. A measurement reliability was performed. RESULTS: In healthy subjects, average SWS (all disc levels pooled) was 7.5 ± 2.6 m/s. In NMS patients, SWS was significantly higher at 9.9 ± 1.4 m/s (p < 0.05). Differences were significant between L3L4 (9.3 ± 1.8 m/s vs. 7.0 ± 2.5 m/s, p = 0.004) and L4L5 (10.3 ± 2.3 m/s vs. 7.1 ± 1.1 m/s, p = 0.0006). No difference was observed for L5S1 (p = 0.2). No correlation was found with age at surgery, Cobb angle correction and age at the SWE measurement. CONCLUSIONS: This study shows a significant increase in disc stiffness at the end of growth for NMS patients treated by FBF. These findings are a useful adjunct to CT-scan in assessing stiffness of the spine allowing the avoidance of surgical final fusion at skeletal maturity.
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Anillo Fibroso , Diagnóstico por Imagen de Elasticidad , Disco Intervertebral , Enfermedades Neuromusculares , Escoliosis , Fusión Vertebral , Humanos , Anillo Fibroso/diagnóstico por imagen , Escoliosis/diagnóstico por imagen , Escoliosis/cirugía , Reproducibilidad de los Resultados , Disco Intervertebral/diagnóstico por imagen , Enfermedades Neuromusculares/cirugía , Resultado del TratamientoRESUMEN
Background: The aim of this study was to describe the epidemiology, physiopathology, and outcomes of elbow "unhappy triad" trauma in children, combining a posterior dislocation, a proximal radius fracture, and a third lesion (i.e. bony or capsuloligamentous injury). Methods: A retrospective bicentric study was conducted between 1999 and 2020. All skeletally immature children who presented to the emergency department and underwent surgery for a proximal radius injury were selected. Among this selection, only patients with two associated ipsilateral elbow injuries (i.e. posterior elbow dislocation and a bony and/or capsuloligamentous injury) were included. Active elbow ranges of motion, Mayo Elbow Performance Score and Quick-Disabilities Of The Arm, Shoulder And Hand scores and standard radiographs were recorded at last follow-up. Results: Twenty-one patients met the inclusion criteria (mean age at surgery = 11.4 years) among 737 selected. The "unhappy triad" diagnosis was made preoperatively in nine cases (bone lesion only), intraoperatively in nine cases, and postoperatively in one case. The third lesions were surgically treated when the lesion was a bony fracture or if the elbow remains unstable between 60° and 90° of flexion (i.e. capsuloligamentous injury). Twenty patients were reviewed (mean follow-up = 5.8 years). The complications and re-operations rates were of 10%. Conclusion: The "unhappy" triad of the child's elbow is a rare injury, where the preoperative diagnosis is frequently missed and lead to 10% of complications and re-operations. Level of evidence: level III.
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PURPOSE: To confirm the overall benignity of fat-containing soft tissue tumors (STT) on a pediatric cohort and to define the clinical and imaging features that warrant a biopsy. METHODS: A retrospective monocentric study was conducted on patients aged less than 25 years consecutively referred for fat-containing STT to our Comprehensive Cancer Center between 1998 and 2022. Tumor imaging characteristics at diagnosis (US, CT, or MRI) were correlated with pathology. RESULTS: The database extraction identified 63 fat-containing tumors with clinical, histologic, and imaging data available for review. In total, 58 (92%) were benign tumors: 36 lipoblastomas and lipomas, 12 fibrous hamartomas of infancy (FHI), 5 lipofibromatosis, 2 lipomas arborescens, 2 lipomatosis and 1 spindle-cell lipoma. Five patients (8%) were diagnosed with liposarcoma. Factors significantly correlated with malignancy were age >10 years old (p < 0.001), having a cancer-predisposing condition (p < 0.001), a percentage of fat <25% (p = 0.002), and a presence of myxoid zones (p < 0.001) on imaging. CONCLUSION: Most fat-containing STT in children may be classified as benign tumors based on clinics and imaging. The indication for biopsy could be limited to patients aged 10 years or more with either a cancer-predisposing condition or imaging features demonstrating either a low-fat component (<25%) or the presence of myxoid zones.
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PURPOSE: Osteogenesis imperfecta (OI) is a genetic disorder responsible for various symptoms including deformities and frequent fractures. Bone allografting is poorly documented in this condition. The objective of this study was to describe our experience and assessments in a consecutive series of OI patients. METHODS: Thirty-nine lower limb allograft procedures (28 femurs, 11 tibias) were performed in 26OI patients (mean age, 12.9 years). They were classified as type III of Sillence (17), type IV (6), and 3 recessive forms. The indications for surgery were correction of deformity (19), fracture (16), and non-union (4). In all cases, bone allografting was added to reinforce areas of fragility and in 28 cases for osteosynthesis to lock the rotations at the osteotomy site and to avoid screwed metallic plate. The duration of bone consolidation and allograft fusion was assessed. Complications and Gillette functional score were reported. RESULTS: The mean follow-up was 6.7years (range, 2 to 10 years). On average, bone consolidation was achieved after 3.3 months and graft fusion after 7.7 months. No bone allograft-related complications were observed and there was any secondary displacement. The Gillette functional score was improved in 23 patients and stable in three cases. Complications were reported in two cases: one partial allograft resorption and one delayed consolidation of a non-union. One refracture was observed but after a significant trauma in a child who had regained significant physical activity. CONCLUSIONS: Bone allografting in children with OI is a reliable method of biological fixation, allowing efficient fusion and contributing to increased bone capital and functional outcome.
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Fracturas Óseas , Osteogénesis Imperfecta , Humanos , Niño , Osteogénesis Imperfecta/cirugía , Osteogénesis Imperfecta/complicaciones , Fracturas Óseas/cirugía , Fijación Interna de Fracturas/efectos adversos , Fémur/cirugía , Trasplante Homólogo/efectos adversosRESUMEN
INTRODUCTION: In patients who have hereditary multiple osteochondroma (HMO), progressive deformity of the forearm skeleton may lead to radial head dislocation. The latter is permanent, painful and causes weakness. HYPOTHESIS: There is a relationship between the amount of ulnar deformity and the presence of radial head dislocation in patients with HMO. MATERIALS AND METHODS: This was a cross-sectional radiographic study comprising an analysis of anterior-posterior (AP) and lateral x-rays of 110 forearms in children having a mean age of 8 years and 4 months who were followed for HMO between 1961 and 2014. Four factors reflecting on the ulnar deformity in the coronal plane were investigated on the AP view and three factors in the sagittal plane were investigated on the lateral view to identify any relationship between ulnar deformity and radial head dislocation. The forearms were separated into two groups: with radial head dislocation (26 cases) and without radial head dislocation (84 cases). RESULTS: Ulnar bowing, intramedullary angle of ulnar bowing, tangent ulnar angle and overall ulnar angle were significantly higher in the group of children who had a radial head dislocation (0.05 vs 0.03, p<.001; 161 vs 167, p<001; 156 vs 162, p<001; 50 vs 30, p<.001) in univariate and multivariate analyses. DISCUSSION: Ulnar deformity, evaluated using the method described here, is more often associated with radial head dislocation than other previously published radiological parameters. This provides new insight on this phenomenon and may help to determine which factors are associated with radial head dislocation and how to prevent it. CONCLUSION: Ulnar bowing in the context of HMO, especially when evaluated on AP radiographs, is significantly associated with radial head dislocation. LEVEL OF EVIDENCE: III; case-control study.
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Exostosis Múltiple Hereditaria , Luxaciones Articulares , Niño , Humanos , Radio (Anatomía)/diagnóstico por imagen , Radio (Anatomía)/cirugía , Exostosis Múltiple Hereditaria/complicaciones , Exostosis Múltiple Hereditaria/diagnóstico por imagen , Exostosis Múltiple Hereditaria/cirugía , Estudios de Casos y Controles , Estudios Transversales , Estudios Retrospectivos , Cúbito/diagnóstico por imagen , Cúbito/cirugía , Luxaciones Articulares/etiología , Luxaciones Articulares/complicacionesRESUMEN
BACKGROUND: The initial management of patients with sarcoma is a critical issue. We used the nationwide French National Cancer Institute-funded prospective sarcoma database NETSARC to report the management and oncologic outcomes in adolescents and young adults (AYAs) patients with sarcoma at the national level. PATIENTS AND METHODS: NETSARC database gathers regularly monitored and updated data from patients with sarcoma. NETSARC was queried for patients (15-30 years) with sarcoma diagnosed from 2010 to 2017 for whom tumor resection had been performed. We reported management, locoregional recurrence-free survival (LRFS), progression-free survival (PFS), and overall survival (OS) in AYA treated in French reference sarcoma centers (RSC) and outside RSC (non-RSC) and conducted multivariable survival analyses adjusted for classical prognostic factors. RESULTS: Among 3,227 patients aged 15-30 years with sarcoma diagnosed between 2010 and 2017, the study included 2,227 patients with surgery data available, among whom 1,290 AYAs had been operated in RSC, and 937 AYAs in non-RSC. Significant differences in compliance to guidelines were observed including pre-treatment biopsy (RSC: 85.9%; non-RSC 48.1%), pre-treatment imaging (RSC: 86.8%; non-RSC: 56.5%) and R0 margins (RSC 57.6%; non-RSC: 20.2%) (p < 0.001). 3y-OS rates were 81.1% (95%CI 78.3-83.6) in AYA in RSC and 82.7% (95%CI 79.4-85.5) in AYA in non-RSC, respectively. Whereas no significant differences in OS was observed in AYAs treated in RSC and in non-RSC, LRFS and PFS were improved in AYAs treated in RSC compared to AYAs treated in non-RSC (Hazard Ratios (HR): 0.58 and 0.83, respectively). CONCLUSIONS: This study highlights the importance for AYA patients with sarcoma to be managed in national sarcoma reference centers involving multidisciplinary medical teams with paediatric and adult oncologists.
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Sarcoma , Neoplasias de los Tejidos Blandos , Humanos , Adolescente , Adulto Joven , Niño , Estudios Prospectivos , Sarcoma/diagnóstico , Sarcoma/cirugía , Neoplasias de los Tejidos Blandos/cirugía , Bases de Datos Factuales , Supervivencia sin ProgresiónRESUMEN
Mucopolysaccharidosis type I-H (MPS I-H) is a rare lysosomal storage disorder caused by α-L-Iduronidase deficiency. Early haematopoietic stem cell transplantation (HSCT) is the sole available therapeutic option to preserve neurocognitive functions. We report long-term follow-up (median 9 years, interquartile range 8-16.5) for 51 MPS I-H patients who underwent HSCT between 1986 and 2018 in France. 4 patients died from complications of HSCT and one from disease progression. Complete chimerism and normal α-L-Iduronidase activity were obtained in 84% and 71% of patients respectively. No difference of outcomes was observed between bone marrow and cord blood stem cell sources. All patients acquired independent walking and 91% and 78% acquired intelligible language or reading and writing. Intelligence Quotient evaluation (n = 23) showed that 69% had IQ ≥ 70 at last follow-up. 58% of patients had normal or remedial schooling and 62% of the 13 adults had good socio-professional insertion. Skeletal dysplasia as well as vision and hearing impairments progressed despite HSCT, with significant disability. These results provide a long-term assessment of HSCT efficacy in MPS I-H and could be useful in the evaluation of novel promising treatments such as gene therapy.
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Trasplante de Células Madre Hematopoyéticas , Mucopolisacaridosis I , Adulto , Humanos , Mucopolisacaridosis I/terapia , Estudios de Seguimiento , Estudios Retrospectivos , Terapia Genética , Iduronidasa/uso terapéuticoRESUMEN
BACKGROUND: Spinal osteochondroma (or exostosis) is a rare benign tumour whose clinical manifestations are delayed due to their slow growth and location. Few studies have addressed the characteristics and the diagnostic and therapeutic peculiarities of spinal osteochondroma in children. The objective of this multicentre observational study was to assess the outcomes of a cohort of children after surgery for spinal osteochondroma. HYPOTHESIS: Surgical excision of spinal osteochondroma in children is not followed by complications or recurrences. MATERIAL AND METHODS: We included consecutive children who had surgery between 2010 and 2018 at any of eight participating centres to remove spinal osteochondromas. The cause, clinical manifestations, and location of the lesions were collected. The surgical outcomes were evaluated after at least 2 years' follow-up. RESULTS: We identified 22 patients who had surgery to remove 26 spinal osteochondromas at a mean age of 12.8±2.6 years. Among them, 7 had a solitary osteochondroma (SO group) and 15 had hereditary multiple osteochondromas (HMO group). At diagnosis, 72% of patients had clinical signs (spinal pain, n=4; one or more lumps, n=5; and neurological manifestations, n=3). In the HMO group, the diagnosis was made during routine MRI screening for tumours involving the spinal canal. Most osteochondromas involved the cervical spine (n=13), with no difference between the two groups (p=0.9). The lamina was the most common location but 54% of the tumours were growing within the canal (92% in the HMO group). After a mean follow-up of 5.2±4.4 years, no patients had experienced any recurrences or complications related to the disease or treatment. DISCUSSION: Surgical excision of spinal osteochondromas in children is effective, with no medium-term recurrences. Our results also confirm the low peri-operative morbidity, even when the canal is involved, and the absence of any effect at last follow-up on spinal alignment. All patients with neurological manifestations at diagnosis made a full recovery. LEVEL OF EVIDENCE: IV, retrospective observational cohort study.
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Osteocondroma , Neoplasias de la Columna Vertebral , Adolescente , Vértebras Cervicales , Niño , Humanos , Osteocondroma/diagnóstico por imagen , Osteocondroma/cirugía , Estudios Retrospectivos , Neoplasias de la Columna Vertebral/diagnóstico por imagen , Neoplasias de la Columna Vertebral/cirugía , Resultado del TratamientoRESUMEN
This single-centre retrospective study reports our management of carpal tunnel syndrome in 52 children (103 hands) with mucopolysaccharidoses and mucolipidoses. All except one were bilateral. The median age at surgery was 4 years (range 1.5 to 12). The diagnosis of carpal tunnel syndrome was confirmed by an electromyogram (EMG) in all patients; 38% of these presented without any clinical signs. Surgical neurolysis was performed in all hands, combined with epineurotomy in 52 hands (50%) and flexor tenosynovectomy in 75 hands (73%). Surgery was bilateral in 98% of children (102 hands). The mean follow-up was 12 years (range 1 to 19) and the EMG was normalized in 78% of hands. Ten patients suffered recurrence, eight of whom required further surgery. Screening for carpal tunnel syndrome is essential for the management of children mucopolysaccharidoses and mucolipidoses. Surgical treatment should be carried out early with follow-up by EMG to detect recurrence.Level of evidence: IV.
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Síndrome del Túnel Carpiano , Deformidades Congénitas de la Mano , Mucolipidosis , Mucopolisacaridosis , Síndrome del Túnel Carpiano/diagnóstico , Síndrome del Túnel Carpiano/etiología , Síndrome del Túnel Carpiano/cirugía , Niño , Preescolar , Mano , Humanos , Lactante , Mucolipidosis/complicaciones , Mucolipidosis/diagnóstico , Mucolipidosis/cirugía , Mucopolisacaridosis/complicaciones , Mucopolisacaridosis/diagnóstico , Mucopolisacaridosis/cirugía , Estudios RetrospectivosRESUMEN
STUDY DESIGN: Retrospective longitudinal study. OBJECTIVE: The main goal of this study was to measure the disability after AIS correction, according to the LIV. SUMMARY OF BACKGROUND DATA: Adolescent idiopathic scoliosis (AIS) is a three-dimensional deformity of the spine that may require surgical correction. If the upper and lower instrumented levels (UIV and LIV) of these fusions are defined by the characteristics of the curve, the long-term consequences of the LIV choices are still partially unknown. METHODS: This retrospective longitudinal study collected demographic, radiologic (Lenke classification, Cobb angle), and surgical data (approach, LIV, UIV) on 116 patients operated for AIS fusion on a specialized pediatric spine center were collected. All participants answered SRS30, SF12, lumbar and leg pain Visual Analogue Scales (VAS) at last follow-up. Statistical analysis between LIV (T12L1, L2, L3 or L4L5) and clinical data at last follow-up was realized. RESULTS: The mean follow-up was 87months. The mean increase of back pain VAS per UIV level was 9 mm. No statistically significant difference between the different LIV was found, for SRS30 or SF12 MCS (mental component scale). There was a statistically significant difference between L3 UIV and L4L5 UIV for SF-12 PCS (physical component scale); ( P = 0.03). CONCLUSION: The long-term consequences of LIV choice mostly affect levels distal to L3. If the LIV is mostly defined by the characteristics of the curve, one level caudally corresponds to +9 mm of back pain VAS at 7 years of follow-up. Surgeons may be aware of the long-term consequence of LIV choice and patients be informed.
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Cifosis , Escoliosis , Fusión Vertebral , Adolescente , Dolor de Espalda , Niño , Estudios de Seguimiento , Humanos , Estudios Longitudinales , Vértebras Lumbares/cirugía , Calidad de Vida , Radiografía , Estudios Retrospectivos , Escoliosis/diagnóstico por imagen , Escoliosis/cirugía , Fusión Vertebral/efectos adversos , Fusión Vertebral/métodos , Vértebras Torácicas/cirugía , Resultado del TratamientoRESUMEN
The incidence of untreated Monteggia fracture-dislocations is not insignificant. But there is no consensus as to the surgical indication and best surgical technique to use. Single-stage reduction of the radial head through an elbow arthrotomy and ulnar osteotomy is a complex surgical procedure that requires stabilization of the ulnar segments during every step of the intervention. Here, we describe the possibility of using an external fixator intraoperatively to facilitate the various steps of the procedure: (1) gradual distraction and angulation of the osteotomy site to allow thorough excision of any interposed capsular tissue, (2) stable hold on the ulnar fragments independent of the surgical assistant, which allows the stability of the radial head reduction to be tested in pronation and supination and (3) facilitation of plate fixation at the ulnar osteotomy site. LEVEL OF EVIDENCE: IV; case series.
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Lesiones de Codo , Articulación del Codo , Luxaciones Articulares , Fractura de Monteggia , Articulación del Codo/diagnóstico por imagen , Articulación del Codo/cirugía , Fijadores Externos , Humanos , Luxaciones Articulares/cirugía , Fractura de Monteggia/diagnóstico por imagen , Fractura de Monteggia/cirugía , Resultado del Tratamiento , Cúbito/cirugíaRESUMEN
INTRODUCTION: Complete surgical resection constitutes the mainstay of treatment for locally aggressive, rarely metastazing tumor and low-grade soft tissue sarcomas (LAS). Local relapse is the most common tumor event, especially in the presence of positive margins (R1 margins). The aims of this study are to assess the impact of the national network on patient care and to evaluate the role of immediate re-excision in children, adolescents and young adults with incompletely resected LAS. METHODS: National retrospective multicenter study of all young patients (≤25 years) included in the Sarcoma "ConticaBase" treated for LAS between 2005 and 2017 for whom pathology/biology review was available via the national NETSARC + network. RESULTS: A total of 96 patients were identified (median age: 16 years). Tumors were localized in 99% of cases (1 N+ tumor). With a median follow-up of 4.7 years (range: 0.1-11.9), eight local relapses and two distant metastases were observed. No patient died. Overall 5-year event-free survival (EFS) was 90.4% [95%CI, 84.3-97]. Five year EFS for R1 patients (n = 51) with (n = 24) and without (n = 27) immediate re-excision was 90.5% [95%CI, 78.8-100.0] and 80.3% [95%CI, 64.7-99.9], respectively (p = 0.34). The 37 patients directly treated in a reference center more commonly had a diagnostic biopsy (78% vs. 21%; p < 0.001), more complete surgery (R0: 65% vs. 14%; p < 0.001) and less commonly underwent re-excision (16% vs. 54%; p < 0.001). CONCLUSIONS: This large series indicates that LAS are rare in young patients and have a favorable prognosis. Immediate management in reference centers is associated with better standard of care. The main tumor events are local relapses.
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Sarcoma , Neoplasias de los Tejidos Blandos , Adolescente , Niño , Humanos , Márgenes de Escisión , Recurrencia Local de Neoplasia/patología , Estudios Retrospectivos , Sarcoma/cirugía , Neoplasias de los Tejidos Blandos/cirugía , Adulto JovenRESUMEN
BACKGROUND: Osteosynthesis of leg fractures and deformities in children with osteogenesis imperfecta should align the skeleton and overcome its fragility during growth with a telescopic effect. A high rate of mechanical complications is associated with various surgical techniques described in the literature. PURPOSE: The objective of this work was to assess the long-term clinical and radiologic outcomes of tibial sliding elastic nailing technique. METHODS: A total of 22 children with an average age of 4.7 years were operated using the technique between 2004 and 2018 unilaterally (6) or bilaterally (16), that is, 38 operations. They were listed according to the Sillence classification into type I (3), III (17), or V (2). The nails were introduced percutaneously at the distal tibial epiphysis through the medial malleolus, and in the prespinal area for the proximal tibial epiphysis. The stainless-steel rods diameter was 1.5 to 2.5 mm, adapted to the size and weight of the patient. Realignment osteotomies were performed if necessary. Radiographic data including the correction of the deformation in the frontal and sagittal planes, as well as the width at mid-shaft of the tibia in the frontal and sagittal planes, were reviewed. Gillette Functional Score, assessment of pain, mechanical and infectious complications were collected. RESULTS: The average follow-up was 8.6 years. In the frontal plane, preoperative average varus was 8 degrees (maximum, 40 degrees), 5 degrees (maximum, 13 degrees) postoperatively, and 6 degrees (maximum, 12 degrees) at last follow-up. Preoperative valgus was 11 degrees (maximum, 22 degrees), 9 degrees (maximum, 15 degrees) postoperatively, and 9 degrees (maximum, 14 degrees) at the last follow-up. In the sagittal plane, the mean sagittal bowling of the tibia was 32 degrees (4 to 75 degrees) preoperatively, 9 degree (1 to 26 degrees) postoperatively, and 9 degrees (1 to 24 degrees) at last follow-up. The width at mid-shaft of the tibia in the frontal plane was 1.1 cm (0.6 to 1.8 cm) preoperatively and 1.3 cm at the last follow-up (0.7 to 2.0 cm). In the sagittal plane, it was 1.25 cm (0.7 to 2.7 cm) preoperatively and 1.27 cm (0.8 to 2.8 cm) at the last follow-up. Ten patients did not require revision surgery during their follow-up. Sixteen mechanical complications occurred in 12 patients (12 fractures or deformities following a lack of overlap of the 2 rods at an average time of 4.9 years after the initial surgery, 3 prominence of the nail, 1 pseudarthrosis). No infectious complication was reported. Gillette Functional Score was 20.54/65. Fifteen patients were able to walk at last follow-up, and 18 had no painful discomfort. CONCLUSIONS: The tibial sliding elastic nailing technique provides satisfactory clinical and radiologic results over time. Performed in case of fracture or as a preventive treatment, it allows a good correction of angular deformations. It is particularly suitable for young patients with a narrow medullary shaft. LEVEL OF EVIDENCE: Level IV-therapeutic study.
Asunto(s)
Fijación Intramedular de Fracturas , Osteogénesis Imperfecta , Fracturas de la Tibia , Clavos Ortopédicos , Niño , Preescolar , Fijación Intramedular de Fracturas/efectos adversos , Humanos , Osteogénesis Imperfecta/diagnóstico por imagen , Osteogénesis Imperfecta/cirugía , Tibia/diagnóstico por imagen , Tibia/cirugía , Fracturas de la Tibia/diagnóstico por imagen , Fracturas de la Tibia/cirugía , Resultado del TratamientoRESUMEN
Juvenile hyaline fibromatosis is a rare disorder characterized by an extracellular accumulation of hyaline deposit. In the extremities, lesions may remain quiescent or gradually increase in size, eventually resulting in skin ulceration. There is no curative treatment. Surgery may allow some recovery of function, but recurrence is possible. We report a case of juvenile hyaline fibromatosis in both hands of a 25-year-old man who required multiple surgical procedures to address problems with function, pain, and appearance.
Asunto(s)
Fibroma , Síndrome de Fibromatosis Hialina , Neoplasias Cutáneas , Adulto , Mano/patología , Mano/cirugía , Humanos , Hialina , Síndrome de Fibromatosis Hialina/diagnóstico , Síndrome de Fibromatosis Hialina/patología , Síndrome de Fibromatosis Hialina/cirugía , Masculino , Dolor , Extremidad Superior/patologíaRESUMEN
INTRODUCTION: Expandable endoprostheses are used to restore limb function and compensate for the sacrifice physis involved in carcinologic resection. Long-term outcomes of the last generation of knee "non-invasive" expandable endoprostheses are required. Objectives were to report on oncologic results of bone sarcoma resection around the knee with expandable endoprosthesis reconstruction and to compare the surgical outcomes of the "non-invasive" expandable endoprostheses used in our department. MATERIALS AND METHODS: Retrospective study that included all children with bone sarcoma around the knee that underwent tumor resection reconstructed with non-invasive expandable prosthesis. Phenix-Repiphysis was used from 1994 to 2008 followed by Stanmore JTS non-invasive from 2008 to 2016. Survival and complications were recorded. Functional outcomes included Musculoskeletal Tumor Society (MSTS) score, knee range of motion, lower limb discrepancy (LLD). RESULTS: Forty children (Sex Ratio = 1) aged a mean 8.8 years (range, 5.6-13.8) at surgery were included in the study. There were 36 osteosarcoma and 4 Ewing sarcoma that involved 33 distal femur and 7 proximal tibia. Cohort (n = 40) consisted of 28 Phenix-Repiphysis and 12 Stanmore with a mean follow-up of 9.8 ± 5.8 years and 6.1 ± 3.1 years, respectively. Postoperative infection rate was 7.5% in the cohort (3 Repiphysis). Functional results were significantly better in the Stanmore group with a mean MSTS of 87.6 ± 5.4% and knee flexion of 112 ± 38°. At last follow-up, implant survival was 100% in Stanmore group, whereas all living Phenix-Repiphysis were explanted. Mechanical failure was the primary cause for revision of Phenix-Repiphysis. Limb length equality was noted in 79% patients with Phenix-Repiphysis and 84% with Stanmore at last follow-up. CONCLUSION: Chemotherapy and limb-salvage surgery yield good oncologic outcomes. Expandable endoprostheses are effective in maintaining satisfactory function and lower limb equality. With improvements made in the last generation of "non-invasive" prostheses, implants' survival has been substantially lengthened.