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Background: Women are underrepresented in orthopaedic and trauma surgery worldwide, with proportions of 4%-17% across countries and 9.1% in France. The annual meeting of the French Society for Orthopaedic and Trauma Surgery (SOFCOT) provides opportunities for quantifying the representation of women, which has not yet been accurately evaluated. The objectives of this retrospective study were: 1) to evaluate the proportion of women relative to men who submitted abstracts accepted for presentation at annual SOFCOT meetings, 2) to analyse this proportion in each abstract category, and 3) to compare the professional profiles of submitting authors., Hypothesis: Among authors with abstracts accepted for presentation at annual SOFCOT meetings, only a minority were women., Methods: This retrospective study evaluated all abstracts accepted for presentation at the annual SOFCOT meetings held in 2021, 2022, and 2023. For each accepted abstract, the gender and professional profile of the submitting author were assessed. The female/male ratio was determined for each abstract category and professional profile for each of the three years., Results: The female/male ratio among authors of abstracts accepted in 2021, 2022, and 2023 was 161/923 (14.9% women), 128/541 (19.1%), and 120/550 (17.9%). The proportion of women was significantly less than the proportion of men for abstracts on the knee (41/333, 11% women (p = 0.0008)), hip (23/209, 9.9% (p = 0.002)), trauma (53 /358, 12,9% (p = 0.017)), and tumours (14/119, 10.5% (p = 0.04)). The proportion of women was also significantly smaller than the proportion of men among residents/fellows (154/491, 23.5% (p < 0.0001)), senior surgeons in non-university institutions (42/400, 11.5% (p < 0.0001)), and senior surgeons in university hospitals (23/164, 12.3% (p = 0.009))., Discussion: The representation of women compared to men remains low at annual SOFCOT meetings despite being greater than the overall representation of women among orthopaedic surgeons in France. These results highlight the appeal of research and growing interest for orthopaedic surgery among women. Scientific societies and surgical teams must continue to encourage this dynamic., Level of Evidence: III; retrospective case-control study., (Copyright © 2024 Elsevier Masson SAS. All rights reserved.)

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., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

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., Competing Interests: Each author certifies that there are no funding or commercial associations (consultancies, stock ownership, equity interest, patent/licensing arrangements, etc.) that might pose a conflict of interest in connection with the submitted article related to the author or any immediate family members. The authors declare no conflicts of interest., (Copyright © 2024 The Author(s). Published by Wolters Kluwer Health, Inc.)

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., Competing Interests: Declaration of competing interest All Authors report no conflict of interest., (Copyright © 2024 French Society of Pediatrics. Published by Elsevier Masson SAS. All rights reserved.)

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-β 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.

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., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2023.)

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., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Purpose: Posterior spinal fusion (PSF) at skeletal maturity is still the gold standard in children with neuromuscular scoliosis (NMS) who underwent fusionless surgery. The aim of this computed tomography (CT) study was to quantify the spontaneous bone fusion at the end of a lengthening program by minimally invasive fusionless bipolar fixation (MIFBF), that could avoid PSF., Methods: NMS operated on with MIFBF from T1 to the pelvis and at final lengthening program were included. CT was performed at least five years postoperatively. The autofusion was classified as completely or not fused at the facets joint (on both coronal and sagittal plane, right and left side, from T1 to L5), and around the rods (axial plane, right and left side, from T5 to L5). Vertebral body heights were assessed., Results: Ten patients were included (10.7y ± 2 at initial surgery). Mean Cobb angle was 82 ± 20 preoperatively and 37 ± 13 at last follow-up. CT were performed on average 6.7y ± 1.7 after initial surgery. Mean preoperative and last follow-up thoracic vertebrae height were respectively 13.5 mm ± 1.7 and 17.4 mm ± 1.7 (p < 0.001). 93% facets joints were fused (out of 320 analyzed joints), corresponding to 15/16 vertebral levels. Ossification around the rods was observed in 6.5±2.4 levels out of 13 in the convex side, and 4.2 ± 2.2 in the concave side (p = 0.04)., Conclusions: This first computed quantitative study showed MIFBF in NMS preserved spinal growth, while it induced 93% of facet joints fusion. This could be is an additional argument when questionning the real need for PSF at skeletal maturity., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

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., (© 2023. The Author(s) under exclusive licence to SICOT aisbl.)

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.

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., (© 2022. The Author(s).)

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., (© 2023. The Author(s).)

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., (Copyright © 2022 Elsevier Masson SAS. All rights reserved.)

Peripheral nerve injuries of the upper limb are rare in children and poorly documented. The aim of this retrospective study was to analyze long-term sensory and motor results, and to determine predictive factors for recovery after surgery. Eleven children, with a mean age at injury of 9.7 years (5-15), operated on between 2006 and 2018, were included. Sensory perception was measured on monofilament test and static 2-point discrimination test. Grip strength was measured with a dynamometer and motor strength was assessed on the Medical Research Council scale. Quality of life was assessed on QuickDASH. The injury involved the radial (n = 1), median (n = 9), or combined median and ulnar (n = 1) nerves and was repaired by primary direct suture (n = 11). The mechanism involved glass laceration (n = 10) or a road accident (n = 1). The dominant limb was involved in 7 cases. At a mean 7.7 years' follow-up, touch sensitivity was normal or slightly deficient on monofilament test. Discrimination test was normal or adequate. Strength was complete in 10 patients. Mean QuickDASH score was 5.99 (range, 0-18.18). There was no significant difference in sensory or motor recovery according to partial or complete lesion or to injury location. There was better sensory recovery in children <12 years (p < 0.05). Sensory prognosis was also better in the absence of associated lesions (p < 0.05). Sensory, motor and functional results after surgical treatment of peripheral nerve injuries of the upper limb in children were globally satisfactory. Sensory recovery was better at an early age and in the absence of associated lesions. LEVEL OF EVIDENCE: IV., (Copyright © 2022 SFCM. Published by Elsevier Masson SAS. All rights reserved.)

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., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

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., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH, DE part of Springer Nature.)

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.

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., Competing Interests: Declaration of competing interest None., (Copyright © 2021 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

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., (Copyright © 2021 Elsevier Masson SAS. All rights reserved.)

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., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Objectives: We aimed to evaluate the efficacy of fascia iliaca nerve block (FINB), routinely used for children with femoral fractures, in a pediatric emergency department (PED)., Methods: This retrospective, single-center, observational study examined FINB using ropivacaine and a 1% lidocaine hydrochloride solution, in all patients under 18 years of age admitted with a femoral fracture from January 2012 to December 2016. Pain was assessed using two validated pediatric pain scales: EVENDOL or a visual analog scale. A level of ≥ 4 on either scale indicates the need for an analgesic. The primary outcome was the percentage of patients who were pain free after the FINB procedure defined by a pain score of < 4. Secondary outcomes were the time spent between PED admission and FINB, the need of additional analgesics, side effects, and the success rate of FINB., Results: Of 161 patients screened, 144 were included. The median age was 3.2 years (range 2 months to 16 years) and 74% were boys. The number of children determined to be pain free (pain score < 4) increased from 36 (25%) before the FINB to 123 (85%) after the FINB (absolute risk difference 60%, 95% CI: 51%-70%). Overall, 21 children (15%) required a second analgesic after the FINB., Conclusion: The routine use of FINB with ropivacaine and lidocaine by pediatric ED physicians provided effective pain relief for children admitted for a femoral fracture in the emergency department. Our data support the efficiency and feasibility of FINB for the antalgic management of children with femoral fracture., (Copyright © 2021. Published by Elsevier Masson SAS.)

X-linked hypophosphatemia (XLH) is due to mutations in the PHEX gene leading to unregulated production of FGF23 and uncontrollable hypophosphatemia. XLH is characterized in children by rickets, short stature, waddling gait, and leg bowing of variable morphology and severity. Phosphate supplements and oral vitamin D analogs partially or, in some cases, fully restore the limb straightness. XLH patients may also be affected by premature, complete, or partial ossification of sutures between cranial bone, which could eventually result in cranial dysmorphia, decreased intracranial volume, and secondary abnormally high intracranial pressure with a cerebral compression. Our goal is to address the criteria and the management of the skeletal complications associated with XLH, mainly orthopedic and neurosurgical care, and reflect on decision-making and follow-up complexities., (Copyright © 2021. Published by Elsevier Masson SAS.)

Background: Treatment of extremity rhabdomyosarcomas (RMS) includes chemotherapy, surgery, and radiotherapy. Lymph node irradiation is recommended in the presence of regional node involvement at diagnosis. The aim of this study was to analyze the correlation between the pattern of relapse of non-metastatic extremity RMS and the initial therapies delivered., Methods: All patients with localized extremity RMS prospectively treated in France in the MMT-95 and RMS-05 protocols were selected. Extent of disease and pattern of relapse were evaluated by clinical examination and imaging., Results: We identified 59 patients with clinical characteristics corresponding to unfavorable prognostic factors. Twenty patients (34%) were considered to have lymph node involvement at diagnosis. Regional node biopsy was performed in 32 patients (54%) and modified the lymph node stage in 8 of the 59 patients (14%). Seventy-three percent of patients received radiotherapy. Fifty-two patients achieved first remission. Overall, 26 patients underwent complete tumor resection, 17 had R1 margins, and 5 were not operated due to early tumor progression. With a median follow-up of 82 months (range: 5-287), 18 relapses had occurred, at least locoregional in 12 cases. The 5‑year local and nodal control rates were 73% (63-86%) and 86% (77-95%), respectively. Five-year progression-free and overall survival were 57% (95%CI [45-72%]) and 70% (95%CI [58-84%]), respectively., Conclusion: The main sites of extremity RMS relapse are locoregional. Nodal failures in non-irradiated fields are not uncommon. We recommend systematic biopsy of in-transit nodes, especially in alveolar RMS and/or RMS with regional positive nodes at diagnosis to ensure their negativity., (© 2021. Springer-Verlag GmbH Germany, part of Springer Nature.)

Severe infant osteogenesis imperfecta requires osteosynthesis. Intramedullary tibia's osteosynthesis is a technical challenge given the deformity and the medullar canal's narrowness. We describe an extramedullary technique: 'In-Out-In' K-wires sliding. We performed an anteromedial diaphysis approach. The periosteum was released while preserving its posterior vascular attachments. To obtain a straight leg, we did numerous osteotomies as many times as necessary. K-wires ('In') were introduced into the proximal epiphysis, and the medial malleolus ('Out') bordered the cortical and ('In') reach their opposite metaphysis. K-wires were cut, curved and impacted at their respective epiphysis ends to allow a telescopic effect. All tibial fragments are strapped on K-wires, and the periosteum was sutured over it. Our inclusion criteria were children with osteogenesis imperfecta operated before 6 years old whose verticalization was impossible. Seven patients (11 tibias) are included (2006-2016) with a mean surgery's age of 3.3 ± 1.1 years old. All patients received intravenous bisphosphonates preoperatively. The follow-up was 6.1 ± 2.7 years. All patients could stand up with supports, and the flexion deformity correction was 46.7 ± 14.2°. Osteosynthesis was changed in nine tibias for the arrest of telescoping with flexion deformity recurrence and meantime first session-revision was 3.8 ± 1.7 years. At revision, K-wires overlap had decreased by 55 ± 23%. Including all surgeries, three distal K-wires migrations were observed, and the number of surgical procedures was 2.5/tibia. No growth arrest and other complications reported. 'In-Out-In' K-wires sliding can be considered in select cases where the absence of a medullary canal prevents the insertion of intramedullary rod or as a salvage or alternative procedure mode of fixation. It can perform in severe infant osteogenesis imperfecta under 6 years old with few complications and good survival time., (Copyright © 2020 Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: Vascularized fibular proximal epiphyseal transfer associated to a diaphyseal segment is used to treat childhood epiphyseal defect. The aim of the present study was to analyze surgical technique and long-term clinical and radiological results., Material and Method: Between 1997 and 2008, 7 patients with a mean age of 5.7 years (range, 2-8 years) were operated on for bone malignancy with vascularized fibular epiphyseal transfer: 5 Ewing sarcomas and 2 osteosarcomas, located in the proximal femur (n=3), proximal humerus (n=3) or distal radius (n=1). Mean transplant size was 13.8cm. Vascularization involved a single artery in 5 cases (3 peroneal, 2 anterior tibial) and both in 2 cases. Internal fixation used intramedullary nailing in 6 cases and screwed plate in 1. All patients underwent pre- and post-operative chemotherapy following French Pediatric Oncology Society (SFOP) protocols., Results: Mean follow-up was 11 years (range, 3 years 11 months to >17 years). All patients were alive and in tumor remission. Reconstructed joint function was satisfactory in 85% of cases. Graft thickening indicated integration in all cases. The transferred cartilage had recovered growth in 4 cases. Complications comprised postoperative infection (n =1), consolidation defects (n = 2), fractures (n = 8), malalignment requiring surgical revision (n = 1), and spontaneously resolving common peroneal nerve palsies (n = 2)., Discussion: In young children, vascularized fibular epiphyseal transfer fills bone defect, reconstructs a functional joint and allows continued growth in the resected segment. Growth prostheses, in the authors' experience, always give poor results in this age-group, and fusion fails to address the growth problem. Patients should be informed about the risk of fracture, persisting over the long term., Conclusion: Vascularized fibular epiphyseal transfer is a difficult technique, subject to complications, but enables reconstruction of a bone segment involving the epiphysis in young children, conserving function and growth., Level of Evidence: III, retrospective clinical study., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Introduction: Aneurysmal bone cyst (ABC) is a benign bone lesion of childhood and adolescence. It can be locally aggressive, with risk of fracture. Management is controversial. The aim of the present study was to assess the efficacy, simplicity and tolerance of percutaneous alcohol-bases sclerotherapy in ABC., Hypothesis: Alcohol-based sclerotherapy for ABC under radiographic control is safe and effective., Material and Methods: A single-center retrospective study for the period 2008-2016 included all of the 55 ABCs, in 54 patients, confirmed on pathology and treated by alcohol-based sclerotherapy under radiographic control. Mean age at diagnosis was 9.6 years. ABC involved the humerus in 30 cases (54%), tibia in 7 (13%) and femur in 5 (9%). Mean follow-up was 50.9 months (range, 16-117 months). Mean number of applications was 1.7 (range, 1-4). Results were assessed clinically (pain, return to sport, limb length and alignment, revision surgery) and radiologically. The main endpoint was lesion volume reduction. The secondary endpoint was failure, defined by open revision surgery or pain preventing return to a sports activity., Results: Clinical progression was favorable in 36 patients (67%), and radiological progression in 45 (85%). Only 1 cyst required secondary resection. One patient experienced spontaneously resolving intraoperative bradycardia. Male gender and young age emerged as factors for poorer response., Discussion: ABC management in children can be made difficult by lesion size, aggressiveness, location, proximity to the growth plate and small bone stock. Alcohol-based sclerotherapy is simple, reliable and effective in childhood ABC, and may be a first-line attitude, avoiding recourse to invasive surgery., Level of Evidence: IV, retrospective study., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

The aim of the present study was to report results of direct circular suture after 1-stage circumferential resection of limb ring constriction in amniotic band syndrome. A multicentre retrospective study included 14 patients with amniotic band syndrome (mean age, 13.3 months) operated on between 2004 and 2019 by circumferential release of ≥1 ring constriction. Assessment was based on limb function and clinical scar aspect on the POSAS and Vancouver scales. Mean follow-up was 3.9 years. There were no scar-related, vascular or neurologic complications, postoperatively or at last follow-up. POSAS and Vancouver scores were satisfactory. One-stage circumferential release with direct closure is a simple technique that provides satisfactory functional and esthetic results., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Introduction: Madelung's deformity is rare. Treatment often consists in radial or ulnar osteotomy, Vickers' ligament resection or ulnar epiphysiodesis. The present multicenter retrospective study reports clinical and radiographic results in various surgeries. The study hypothesis was that early surgery improves outcome., Material and Method: Files were analyzed for 36 children (47 wrists) operated on between 1998 and 2015. Mean age at diagnosis was 12 years (range, 6-15 years). Mean follow-up was 7.2 years (range, 2-17.4 years). Two age groups were distinguished: 6-11 and 12-17 years. Endpoints were esthetic, functional (Mayo Wrist Score: MWS) and radiographic [lunate fossa angle (LFA), radial height (RH), lunatum cover rate (LCR), ulnar head translation (UHT), lunatum ascension (LA)]. Techniques mainly comprised distal radius osteotomy (DRO) with ulnar shortening osteotomy (USO), and Vickers' ligament resection (VR), either isolated or associated to osteotomy., Results: Age at surgery guided choice of technique, but did not impact results. VR gave better functional results when performed in young children; it was mainly performed in the 6-11 years age group, providing very satisfactory results (7 out of 8 wrists) with excellent function (7 out of 8), without improving radiographic parameters; in the 12-17 years age group, even when esthetic results were excellent, functional results were only good-to-acceptable (3 out of 5 wrists) and 1 result was poor. DRO+USO was mainly performed in the 12-17 years age group, with very satisfactory or satisfactory results (8 out of 10 wrists) and excellent function (6 out of 10). DRO+USO appeared to be an option of choice, significantly improving radiographic parameters and correcting the deformity (24.5° improvement in LFA (p=0.0033) and 36.2% improvement in LCR (p=0.0103)). DRO+USO+VR was mainly implemented in the 12-17 years age group, with very satisfactory results (3 out of 4 wrists) and excellent-to-good MWS in most cases (3 out of 4), but without significant radiographic improvement., Discussion: Esthetic and functional results were comparable regardless of age, except for VR, which gave better functional results in the 6-11 years age group, but without significant difference., Conclusion: The present study showed that, regardless of children's age, surgery provided esthetic, functional and radiographic benefit, although no decision-tree could be drawn up., Level of Evidence: IV., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

Introduction: In the pediatric population, fractures of the radial neck account for approximately 1% of all fractures and can have substantial consequences. Given the growing popularity of horse riding and the high-energy traumas that this can entail, we sought to assess prognosis for radial neck fracture sustained in horse riding., Hypothesis: Radial neck fracture sustained in horse riding is more severe and more frequently associated with other ipsilateral osteoarticular lesions which worsen prognosis., Material and Method: We performed a multicenter retrospective comparative study in a continuous series of 39 patients. The inclusion criteria consisted of radius neck fracture in children under the age of 15 years. Endpoints comprised associated lesions at time of trauma, and ranges of elbow motion in pronation/supination and flexion/extension at last follow-up., Results: Compared to other etiologies, radial neck fractures following horse-riding accidents were more frequently associated with other ipsilateral osteoarticular lesions (p=0.0002), and more often required open reduction (p=0.0409) and postoperative rehabilitation (p=0.041). However, there were no significant differences in any ranges of motion at last follow-up (p>0.05)., Conclusion: Radial neck fractures following horse-riding accidents in children were more severe than those caused by other mechanisms. Awareness campaigns in riding clubs and development of specific protective equipment should be considered to reduce occurrence., Level of Evidence: Therapeutic III - Retrospective comparative study., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

In Rubinstein-Taybi syndrome, patients may have a particularly severe clinodactyly of the thumb. We evaluated a new method for correction of these severe clinodactylies using non-vascularized toe phalanx transfer as a replacement for the abnormal delta phalanx. Results of the new technique are presented, together with those of an osteotomy technique. We retrospectively recorded the angle of the clinodactyly before and after surgery and at long-term follow-up of 11 osteotomies and five transfers in nine patients from 1990 to 2017. The pre-operative angle of clinodactyli was similar between the two groups with a mean of 59°. After surgery, the correction was equivalent (7° and 11°). At the last follow-up (7 and 18 years), the relapse of clinodactyly was 17° for osteotomies and 1° for phalanx transfers. We noticed growth of the transferred phalanx, resulting in an excellent thumb length. We conclude that non-vascularized toe transfer can be an effective correction of severe clinodactyly and may be more stable than osteotomy in the long-term. Level of evidence: IV.

Background: Outcomes for adults with soft tissue sarcoma are better when managed at referral centers. Care guidelines advise for 5 main criteria: 1-Imaging before biopsy; 2-Tumor biopsy before surgery; 3-Multidiscipinary team discussion (MTD) before biopsy; 4-Biopsy in "expert centers"; 5-Somatic molecular biology feasible. The aim is to describe and assess the prognostic impact of initial management of STS according to the type of referring centers and the number of optimal criteria., Methods: Monocentric retrospective analysis of the management of 127 youths (0-25 years) with localized STS treated from 2006 to 2015., Results: Median age at diagnosis was 9.6 years (range: 025). Overall, only 41% patients had 5/5, 28% 3-4, 31% ≤2. No adequate imaging was performed before surgery/biopsy for 18% patients, no biopsy before treatment for 29%. Patients referred by "expert centers" had higher compliance to guidelines (P = 0.025). Upfront surgery was performed in 59/127 patients. Immediate re-operation was inversely related to the number of criteria (0% when 5 criteria vs. 14% for 3-4, 46% if ≤ 2; P < 0.001). For malignant tumors, outcome was better when 5 criteria were reached: 5 year EFS 90.8% (81.4-100.0%) vs. 71.6 for (60.4-84.9%; ≤4 criteria; p = 0.033), OS 93.6% (85.5-100%) vs. 79.5% (68.9-91.8%; p = 0.11), and LRFFS 90.6% (81.0-100.0) vs. 73.1% (62.0-86.3%; p = 0.047)., Conclusion: Less than half of the youths with STS are initially managed according to international guidelines, highlighting the need for better information about optimal management. These results plead for immediate management in reference centers to reduce initial burden of therapy., Competing Interests: Declaration of competing interest The authors declare that there is no conflict of interest., (Copyright © 2019 Elsevier Ltd, BASO ~ The Association for Cancer Surgery, and the European Society of Surgical Oncology. All rights reserved.)

Aim: Angiomatosis of soft tissue (AST) is a rare, high-flow, intramuscular vascular anomaly. In the context of PTEN hamartoma tumour syndrome (PHTS), this AST is referred to as PTEN hamartoma of soft tissue. Given that AST is observed in patients with no history of PHTS, we hypothesised that non-syndromic AST arises as a consequence of a somatic mutation., Methods and Results: Thirteen patients with histologically confirmed AST were retrospectively studied. Details of the patients' personal and family medical histories and symptoms were retrieved from their medical records. The histological analyses were reviewed and a tissue sample was used for genetic testing. Somatic mutations in the PIK3CA gene (p.Glu542Lys; p.Glu545Lys; p.His1047Arg) were identified in the tissue samples from seven patients, all of whom had unremarkable medical histories and had presented with a single lesion located in the lower limb. Five pathogenic variations in the PTEN gene (mutations: p.Lys263Arg; c.1026+2T>A; p.Ala126Thr; p.Leu108Arg; deletion, log ratio -0.55) were identified in the lesions of four patients; two of the latter had multifocal lesions. All four patients displayed macrocephaly, three boys presented with penile freckles, but none had a family history of PHTS. There were no histological differences between the PIK3CA and PTEN groups., Conclusions: AST can be related to either PTEN or PIK3CA mutations and may be multifocal in PHTS. AST appears to be a manifestation of PHTS that occurs in early childhood. The patient's medical history and clinical presentation should prompt the physician to perform specific genetic testing., (© 2019 John Wiley & Sons Ltd.)

Forearm deformities are often observed in patients with hereditary multiple osteochondroma, resulting in functional disability and cosmetic impairment. The aim of this study was to assess clinical and radiological outcomes after corrective osteotomy of the radius (COR). We performed a retrospective analysis of clinical and radiologic data from patients with forearm deformities who underwent COR combined with osteochondroma resection between 1978 and 2015. Seventeen patients (17 forearms) were included. The mean (range) age at surgery was 11.8 years (3.2-14.4), and the mean interval between surgery and last follow-up was 8.2 years (2-34.2). Range of motion was moderately increased and postoperative radiological assessments found significant improvements in ulnar variance, radial articular angle, bowing of the radius, and carpal slip. At last follow-up, a loss of ulnar variance correction was noted in 11 cases (mean loss: 4mm). The mean score on the Quick Disabilities of the Arm, Shoulder and Hand self-administered questionnaire was 13.9. Our results show that a forearm deformity in a patient with hereditary multiple osteochondroma is an appropriate indication for COR combined with osteochondroma resection and should be performed at the end of growth. This simple, safe technique corrects bowing of the radius and radius-ulna length discrepancy and could limit the risk of radial head dislocation. LEVEL OF EVIDENCE: IV., (Copyright © 2019 SFCM. Published by Elsevier Masson SAS. All rights reserved.)

Introduction: Mucopolysaccharidoses (MPS) are rare and clinically heterogeneous lysosomal storage disorders. Carpal tunnel syndrome (CTS) is a frequent complication in MPS types I, II, VI, and VII. CTS symptoms are difficult to recognize in these children, and often there is a lack of appropriate investigations., Patients and Methods: In this retrospective study, all MPS patients were referred to the electrodiagnostic (EDX) laboratory of a single academic center during a 10-year period. Forty-eight children underwent serial EDX studies for CTS diagnosis and follow-up after surgery. Forty-two patients were diagnosed with CTS. Sensory nerve conduction velocity (SNCV), distal motor latency (DML), and motor nerve conduction velocity through the wrist (MNCV-W) of the median nerve were reviewed and analyzed., Results: One-hundred-three EDX examinations were performed on 48 patients. The median age at disease diagnosis was 2.1 years versus 4.9 years for CTS diagnosis. Analysis of the series revealed that electrophysiological abnormalities of CTS could have started much earlier (before the age of 2 years or at diagnosis of MPS). Diagnosis was based on SNCV and DML results, and MNCV-W was taken into consideration. Bilateral CTS was frequent (88%) in the types of MPS studied in our population and was observed from the first year of life, and may not have be associated with obvious clinical symptoms. EDX studies also helped in the follow-up and detection of CTS relapses, thus leading to an early intervention allowing a better recovery., Conclusion: EDX studies should be performed promptly and regularly in these patients. Prospective studies are required in order to understand the effect of disease-specific therapies in preventing the development of CTS in these patients., Synopsis: EDX studies should be performed in MPS patients soon after diagnosis and during routine follow-up, before and after surgical decompression.

Purpose: The objective of the present study was to evaluate the outcome of surgical treatment of hand lesions in Ollier disease (OD) carried out during childhood., Methods: A retrospective review was carried out of 10 pediatric patients with hand involvement of OD, who had undergone surgery for metacarpal or phalangeal enchondromas. The technique comprised curettage and cortical bone reconstruction with corticoplasty, to restore a near-normal phalangeal volume. The range of finger motion (pulp-to-palm distance), the shortened Disabilities of the Arm, Shoulder, and Hand (QuickDASH) questionnaire score, cosmetic improvement, radiological findings (according to Tordai's classification), and recurrence were recorded after a mean follow-up of 7.5 years (range, 4-11.3 years)., Results: The mean age at surgery was 10.7 years (range, 6-14 years). Curettage was performed on 35 enchondromas, and 9 cavities were filled with a bone graft. The mean pulp-to-palm distance was significantly lower after surgery (from 1.5 cm to 0.25 cm; P < .05). The mean QuickDASH score was 3.84 (range, 0-11.4). A marked cosmetic improvement was noted for 83% of the hands. Three enchondromas recurred in 1 patient, requiring a second curettage. Fifty-seven percent of the cavities were completely filled with bone (Tordai stage 1) at last follow-up. The outcome did not depend on the presence or absence of a bone graft., Conclusions: Our results suggest that early surgical treatment comprising curettage and corticoplasty leads to good clinical, cosmetic, and radiological outcomes. Early surgical treatment of well-developed and/or symptomatic enchondromas of the hand in OD should be considered., Type of Study/level of Evidence: Therapeutic V., (Copyright © 2018 American Society for Surgery of the Hand. Published by Elsevier Inc. All rights reserved.)