INTRODUCTION:Total hip arthroplasty (THA) is commonly performed in adults with excellent outcomes. Its use in the paediatric population remains rare due to concerns regarding potential damage to the growth plate, pelvic biomechanical changes during growth, leg-length discrepancies, and implant longevity. Although modern prostheses have an estimated lifespan of approximately 30 years, durability remains a major concern in young patients. However, in selected cases, particularly in spastic patients suffering from chronic painful hip dislocation, medical options may be exhausted, and conservative surgical procedures may no longer be effective. The aim of this study was to evaluate the mid- to long-term outcomes of paediatric patients with cerebral palsy who underwent THA. METHODS:This retrospective single-centre study included 22 paediatric patients with cerebral palsy who underwent THA. Clinical outcomes were analysed according to patient characteristics and prosthetic factors. The primary outcome was chronic pain relief, defined as absence of pain at the last follow-up. Secondary outcomes included postoperative complications and rehospitalisation within 90 days of surgery. RESULTS:The mean age at surgery was 15.6 years. The mean follow-up duration was 11.1 ± 3.7 years. Preoperative chronic pain was present in all patients except 1, who was non-communicative; in this case, the surgical indication was based on radiographic evidence of hip dislocation. The overall complication rate was 22% (n = 5), including 2 dislocations, 1 trochanteric nonunion, 1 intraoperative distal femoral fracture requiring plate fixation, and 1 femoral component loosening at 10-year follow-up. No postoperative infections were observed. Rehospitalisation within 3 months occurred in 13.6% of patients (n = 3). DISCUSSION:THA in paediatric patients with cerebral palsy appears to provide substantial pain relief with acceptable complication rates. Mid- to long-term outcomes are encouraging in this challenging population.

OBJECTIVE:Severe scoliosis, defined by a Cobb angle exceeding 80°, requires surgical correction and is associated with a high risk of postoperative neurological complications. These complications may be reduced by halo gravity traction (HGT) and neuromonitoring. This study evaluated the outcomes of preoperative and intraoperative multimodal neuromonitoring by somatosensory evoked potential (SSEP) and motor evoked potential (MEP) in patients undergoing HGT before and during spinal surgery for severe idiopathic scoliosis. The secondary objective was to investigate any changes in SSEP associated with traction. METHODS:A single-center retrospective review was conducted including all patients who underwent surgery for severe idiopathic scoliosis following HGT. Demographic data were collected. Pre-traction, post-traction, and postoperative Cobb angle corrections were reported. Complications, preoperative and intraoperative neuromonitoring data were recorded. RESULTS:70 patients with an average age at surgery of 14.4 years were included. The mean postoperative correction rate was 68.79%±7.57. Postoperatively, five deficits were observed, with one being permanent. There were 19 neuromonitoring alerts during surgery (27.14%). One false negative occurred, while 15 alerts did not result in postoperative deficits. Abnormal SSEP before traction or at the beginning of the intervention was associated with an intraoperative alert. SSEP latencies increased, and amplitude decreased between the period before traction was applied and the beginning of the surgery. CONCLUSIONS:Multimodal neuromonitoring by MEP and SSEP presents a high number of alerts with low postoperative deficit. Traction appeared to increase SSEP latencies and decrease amplitudes. However, multimodal monitoring remains a valuable tool for reducing the risk of neurological deficits in severe scoliosis undergoing preoperative HGT.

Continuous local oxygenation monitoring during spinal surgery lacks a feedback modality that is direct, local, and implementable in real time. Based on multi-wavelength transmissive vertebral photoplethysmography data acquired in a porcine model, this paper compares various data processing methods under hypoxia and tachycardia conditions. Over the full recording, the subspace filter achieves the lowest error (RMSE 3.21%) and outperforms static modified Beer-Lambert Law (9.66%). Under strictly causal real-time evaluation, however, the best result is obtained with the legacy pipeline combined with automatic reset and one-dimensional Kalman filtering (RMSE 2.13 %). These results indicate that the subspace filter is better suited to offline robust analysis, whereas the Kalman-filter-stabilized legacy pipeline is closer to intraoperative real-time deployment.

Les infections du site opératoire (ISO) constituent une complication majeure en orthopédie pédiatrique et surviennent principalement après chirurgie rachidienne pour scoliose. Le diagnostic repose sur les signes cliniques, les marqueurs biologiques de l’inflammation et les prélèvements microbiologiques profonds peropératoires, réalisés si possible avant toute antibiothérapie. En cas de cultures négatives, notamment après antibiothérapie préalable, les techniques de biologie moléculaire telles que la PCR ARN 16S peuvent améliorer l’identification des pathogènes. Le profil microbiologique varie selon le terrain. Les scolioses idiopathiques sont principalement associées à des bactéries à Gram positif, notamment Staphylococcus aureus et Cutibacterium acnes, tandis que les scolioses neuromusculaires sont plus fréquemment associées à des bacilles à Gram négatif. Les facteurs de risque incluent la malnutrition, les pathologies neurologiques, le diabète, la durée opératoire prolongée, l’instrumentation étendue et la présence de dispositifs médicaux. La prévention repose sur des mesures multimodales incluant une préparation cutanée adaptée avec décolonisation du S. aureus lorsque indiquée, une douche antiseptique préopératoire, l’optimisation de l’état nutritionnel et une antibioprophylaxie peropératoire adaptée, le plus souvent par céfazoline avec ou sans couverture des bacilles à Gram négatif chez les patients à risque. La prolongation postopératoire de l’antibioprophylaxie n’est pas recommandée. Le maintien de la normothermie et le retrait précoce des drains contribuent également à réduire le risque infectieux. La prise en charge repose sur une approche multidisciplinaire. L’antibiothérapie est initiée après les prélèvements microbiologiques, adaptée aux résultats, et poursuivie pendant 6 à 12 semaines. La conservation du matériel est privilégiée chaque fois que possible jusqu’à obtention de la fusion osseuse. La mise en place de registres nationaux et de réseaux spécialisés apparaît nécessaire afin d’améliorer la prévention et la prise en charge des ISO en orthopédie pédiatrique.

OBJECTIVE:Shear wave elastography (SWE) is a recent method for evaluating soft tissue stiffness. Its application to the spinal cord is limited by the ultrasound bone barrier. Spinal dysraphism can cause neurological symptoms impairing bladder, bowel, and lower limb function due to a mechanical stress known as tethered cord syndrome. Detethering of the spinal cord is a part of surgical treatment. This study aimed to assess the feasibility of intraoperative elastography to guide surgery in patients with spinal dysraphism involving traction of the spinal cord. METHODS:This single-center retrospective study included all patients who underwent surgery for spinal dysraphism between November 2023 and July 2024. SWE was performed under general anesthesia with the patient in the prone position before and after spinal cord detethering. All SWE measurements were performed using the Aixplorer system. Demographic, clinical, and radiological data (age at surgery, neurological status, and MRI findings) were collected. RESULTS:Twenty-one patients with a mean ± SD (range) age of 3.7 ± 5.3 (0-17.5) years were included. Etiologies included open saccular dysraphism (n = 3), spinal cord conus lipoma (n = 6), limited dorsal myeloschisis (n = 3), and fibrolipomatous filum (n = 9). In 85% of patients, at least 1 clip was of sufficient quality for analysis both before and after release. The mean ± SD (range) spinal cord stiffness at the beginning of surgery was 25.2 ± 9.2 (12.3-39.1) kPa and 11.2 ± 3.8 (6.2-16.5) kPa at the end of release, with a significant stiffness decrease (p = 0.018). No complications attributable to SWE were observed. CONCLUSIONS:SWE of the spinal cord in patients with spinal dysraphism is a safe and feasible imaging method. This technique may assist in assessing the adequacy of untethering during surgery. However, no established normative SWE values for the pediatric spinal cord currently exist. Until it does, the intraoperative interpretability and clinical utility of this technique remain limited.

BAC KGROUND:Total hip arthroplasty (THA) is a rare procedure in pediatric patients. Although it shows excellent results in adults, the presence of growth plates, the limited longevity of implants, and the hip-spine biomechanics involved during growth make the outcomes of THA in children uncertain. OBJECTIVE:We hypothesized that THA yields satisfactory results for children suffering from unmanageable pain due to hip joint osteoarthrosis. METHODS AND SETTINGS:27 patients aged <18 years who underwent THA for hip joint osteoarthritis at our hospital between 2015 and 2022 were retrospectively included. Primary outcomes included two clinical scales: the Postel Merle d'Aubigné (PMA) and Oxford scale. Secondary outcomes included chronic pain relief and complications. RESULTS:Mean age at surgery was 13.7± 1.7 years (min11, max 17). The mean follow-up was 5.4±2.4 years (min 3, max 10). Etiology of osteoarthrosis was slipped capital femoral epiphysis (n=13), idiopathic hip chondrolysis (n=4), femoral neck fracture (n=3), developmental hip dysplasia (n=3), hip joint infection (n=2), aseptic osteonecrosis secondary to drepanocytosis (n=1), and Legg-Calvé-Perthes disease (n=1). The average Oxford score was 43.2±4.8, and the mean PMA score was 15.9±0.6. Pain relief was achieved in 96.3% of patients. Six patients had major complications (22.2%), with three (11.1%) requiring unplanned surgeries. LIMITATIONS:The main limitations were a small sample size and the absence of a control group. CONCLUSION:To date, no study has specifically evaluated THA clinical outcomes in children with non-inflammatory hip joint osteoarthritis. This study demonstrates satisfactory outcomes following THA in children with hip joint osteoarthritis. THA should be considered a viable option for selected pediatric patients.

Introduction: Hand injuries are a major public health issue and are very common in pediatrics, explained by children's world discovery. The aim of this study was to produce a short, animated video to raise awareness and educate children and their parents, by proxy, about common hand injuries, and to assess its effect.Material and Methods: The video was shown to children at school. They were asked to complete an anonymous questionnaire before the projection and 21 days after, to analyze its impact and its spreading.Results: Eight hundred and thirty five children, with an average age of 9.57 years old, filled the pre-projection questionnaire. Upon those children, 88% have already hurt their hands, 86% knew someone who had hurt their hands and 30% did not know the dangerous situations for the hands. Out of the 565 children who filled the post-projection form, 97% answered that they understood the dangerous situations for the hands, with a significative difference compared to the pre-projection answers. 45% watched the video with their parents, 33% advised their families to watch it and 57% thought they would change their habits.Conclusion: Hand injuries should be a priority, to reduce their incidence and consequences. This short video sets out the various possible accidents and risk situations, targeting children between 6 and 12 years old, and indirectly adults. Even if this study has encouraging results, spreading must be improved and association with incidence of hand injuries followed to evaluate its impact and benefits.

Early-onset scoliosis (EOS) comprises a heterogeneous group of spinal deformities diagnosed before the age of ten years, including idiopathic, congenital, neuromuscular, and syndromic etiologies. Management remains particularly demanding because treatment must simultaneously address deformity progression while preserving spinal growth, thoracic development, and pulmonary function. Despite major advances over the past two decades, treatment strategies continue to vary considerably and no universally accepted algorithm has been established.This review examines the current role of conservative and surgical treatment modalities for EOS and discusses their respective indications, benefits, and limitations. Nonoperative management remains the preferred initial approach for most progressive deformities. Bracing may delay curve progression, particularly in idiopathic EOS, although outcomes are influenced by curve magnitude, patient age, brace compliance, and family support. In congenital, neuromuscular, and syndromic scoliosis, its effectiveness is generally more limited. Serial casting represents a valuable option for younger children and for more severe deformities, especially in idiopathic EOS, where substantial correction may occasionally be achieved. However, repeated cast applications may be associated with local complications and, when performed under general anesthesia, expose patients to cumulative anesthetic risks.When conservative treatment fails or when progressive deformity threatens thoracic growth and respiratory function, surgical intervention becomes necessary. Contemporary management favors growth-preserving techniques rather than early definitive fusion, which has been shown to compromise spinal and thoracic development. Magnetically controlled growing rods currently represent the most widely adopted growth-friendly strategy, although complication and reoperation rates remain substantial. Emerging technologies, including modern Luque trolley constructs, one-way self-expanding rods, and spring-based distraction systems, may further reduce the burden of repeated surgical procedures.Successful management of EOS requires individualized decision-making based on etiology, deformity severity, growth potential, and overall patient condition. Regardless of the selected treatment pathway, families should be counselled regarding the prolonged nature of care and the persistent risk of complications throughout childhood.

Neuromuscular scoliosis (NMS) typically develops at an early age, leading to postural instability and cardiopulmonary complications due to spinal deformity and pelvic obliquity. Long-term comfort requires surgical intervention to restore spino-pelvic balance. This study aimed to report the long-term outcomes of a reverse T-construct pelvic fixation technique combined with magnetically controlled growing rods for the pediatric NMS treatment. Between 2016 and 2023, 34 patients with NMS underwent surgery using the reverse T-construct using two sacral screws and two iliac screws connected by a horizontal rod, linked to magnetically controlled growing rods. Clinical and radiographic outcomes were evaluated with a minimum follow-up of two years. The mean age at surgery was 10 ± 1.7 years. The mean preoperative Cobb angle of 69.9° ± 20.3, was decreased to 35.6° ± 21.4 postoperatively. At the last follow-up, the average correction loss was 8.9° ± 17 (p < 0.05). The mean preoperative pelvic obliquity of 14.8° ± 11.1, improved to 3.3° ± 3.7 postoperatively, with an average correction loss of 1.5° ± 3.5° at the last follow-up (p < 0.05). Two postoperative infections and two cutaneous complications were reported. Eight mechanical complications were recorded: one rod fracture, one migration of a rib hook, and six related to pelvic fixation. The reverse T-construct with magnetically controlled growing rods demonstrated satisfying long-term correction of spinal alignment and pelvic obliquity. This technique offers stable pelvic balance, early mobilization, with a low complication rate, making it a viable alternative for managing patients NMS.

Background Severe scoliosis, defined by a major curve exceeding 80°, requires surgical correction and is particularly prone to post-operative mechanical and neurological complications. Objective This study aimed to evaluate the corrections and complications obtained by halo-gravity traction (HGT) followed by single posterior approach surgery for severe scoliosis. The secondary objective was to identify any risk factors associated with neurological complications. Methods A single-center retrospective review of all patients operated on with severe scoliosis or kyphosis of any cause, by a single posterior approach after a period of HGT. Demographic data including age at surgery, sex, and body mass index were collected. Pre-traction, post-traction, postoperative major curve, and kyphosis angles were reported. Perioperative complications both during the traction period and postoperatively were recorded. Results 117 patients with a mean age at surgery of 14.0 ± 2.0 years were included. The mean preoperative major curve was 90.7°±22.1° (from 80.1 to 147.2°) and the mean kyphosis was 60.8°± 31.70 (from 3.3 to 150.2°). The mean major curve after traction was 66.1°±15.2° (from 30.2 to 101.5°) and 33.7°±17.0° (from 0.2° to 104.4°) postoperatively, with a total correction of 69 %. During the HGT period, 8 (6.8 %) complications occurred: 3 superficial infections, 4 pin displacement, and 1 case of encopresis. Postoperatively, eight deficits were observed, with two being permanent. No risk factors for postoperative neurological complications were identified. Conclusions A 6-week period of HGT is associated with a low complication rate, and is effective to prepare severe scoliosis surgery, even for curvatures exceeding 120°

Introduction L’ostéogenèse imparfaite (OI) est une maladie génétique rare du tissu osseux, causée principalement par une mutation autosomique dominante des gènes COL1A1 ou COL1A2, codant pour les chaînes alpha du collagène de type 1. Dans les formes sévères et chez les patients non marchants, chez qui la pratique d’exercice physique est difficile, l’exposition de l’os aux stimulations mécaniques, en favorisant les mouvements, notamment par la physiothérapie et les aides à la mobilité, est un point essentiel de la pratique clinique. Cependant, les effets de la stimulation mécanique au niveau cellulaire restent inconnus pour cette pathologie. Hypothèse L’hypothèse de cette étude est que les cellules souches mésenchymateuses humaines (hMSCs) de patients atteints d’OI sont aussi sensibles à la stimulation mécanique que celles de patients sains, justifiant la pratique clinique actuelle. Patients et méthode Des hMSCs ont été extraites chez trois témoins sains et trois patients atteints d’OI lors d’une ostéotomie programmée d’un os long du membre inférieur. Les CSM saines et celles souffrant d’OI ont été exposées à des contraintes de cisaillement de 0, 0,7, 1,5 et 3Pa à une fréquence de 2,8Hz pendant 30minutes à l’aide d’un système commercial Ibidi. L’expression génétique de la prostaglandine endoperoxyde synthase 2 (PTGS2), précoce et mécanosensible, a été examinée une heure après la stimulation afin de déterminer la meilleure valeur de la stimulation mécanique. Pour ce niveau de stimulation mécanique, l’expression de sept autres gènes mécanosensibles a également été examinée après exposition à une contrainte de cisaillement intermittente de 1,5Pa. Résultats Dans toutes les hMSCs, la stimulation mécanique a induit la surexpression du gène PTGS2 avec un maximum après l’exposition à une contrainte de cisaillement intermittente de 1,5Pa et sans différence significative entre les donneurs OI et les donneurs sains. À l’exception du facteur de croissance des fibroblastes 2, l’expression des gènes chez les donneurs OI s’est avérée significativement différente de celle des hMSCs non exposées à la contrainte de cisaillement. De plus, l’expression relative associée à la stimulation mécanique n’était pas significativement différente entre les donneurs sains et les donneurs OI pour la plupart des autres gènes. Discussion Cette étude est la première à décrire que les hMSCs de patients atteints d’OI sont aussi sensibles à la contrainte mécanique de cisaillement que les hMSCs de patients sains. La valeur de la contrainte mécanique qui entraîne le plus grand changement dans l’expression de PTGS2 chez les patients atteints d’OI est similaire à celle rapportée dans la littérature pour les patients sains. Ces résultats constituent une étape importante vers d’autres études fondamentales visant à confirmer l’effet du stress mécanique au niveau cellulaire à long terme et, plus important encore, vers le développement de protocoles cliniques pour la stimulation mécanique des patients. Niveau de preuve III ; étude comparative avec groupe témoin.

Background Many techniques have been described for lateral ankle ligament reconstruction. Although the biomechanical properties of gracilis tendons are different from those of ligaments, the use of a gracilis tendon autograft is a popular option for anatomical reconstruction. Graft maturation and the biomechanical processes over time remain unclear. This study describes changes in graft stiffness following anterior talofibular ligament (ATFL) reconstruction and graft reaction to varus stress. Hypothesis The reconstruction would be stiffer than the native ATFL, but would decrease during follow-up. Methods Twenty patients were prospectively included after arthroscopic reconstruction of the ATFL and calcaneofibular ligament for ankle stabilization. All patients were followed up 3, 6, and 12 months after surgery to assess graft stiffness by shear wave elastography (SWE) at different angles of varus in the ankle. At one year the EFAS and AOFAS functional scores were obtained. A control group of twenty healthy subjects were included to compare graft stiffness to that of a native ATFL. Results The stiffness of the native ATFL in the control group was 12.8 +/- 2.4 kPa in neutral position, 18.4 +/- 4.8 kPa at 15 ° of varus, 31.9 +/- 6.6 kPa at 30 ° of varus. One year after surgery, graft stiffness was statistically higher and averaged 56 +/- 9 kPa, 70.2 +/- 11.6 kPa and 84.9 +/- 10.5 kPa, respectively. Postoperative graft stiffness at three, six, and twelve months was not correlated with any of these scores, reflecting patient satisfaction and good function at one year. Conclusion Graft stiffness decreases over time but remains four times stiffer than that of a native ATFL at one year in the neutral position. ATFL graft stiffness at one year during varus stress appears to be different from that of a native ATFL. Level of evidence III.

BACKGROUND:The surgical treatment of scoliosis in type 2 spinal muscular atrophy (SMA2) is challenging and little described in the literature due to its rarity and fragility of the patients. The aim of this study was to review the surgical strategies and outcomes in patients with SMA2 who underwent surgery for scoliosis at a French reference neuromuscular center. METHODS:All consecutive patients with genetically confirmed SMA2 who underwent spinal surgery between 2009 and 2022 at our French reference center were retrospectively analyzed. They were divided into 2 groups, according to their primary surgery: either magnetically controlled growing rods (MCGR) or posterior spinal fusion (PSF). Demographic, respiratory, and radiologic parameters were collected preoperatively and at the latest follow-up. All complications were reported. Patients and/or caregiver-reported outcome questionnaires were also used to assess the improvement of sitting posture. RESULTS:Seventeen patients underwent MCGR, and 9 patients underwent PSF during the inclusion period. Mean follow-up was 5.3±1.8 years in the MGCR group, and 8.0±4.5 years in the PSF group. The average age at surgery was 9.7±1.6 years in the MCGR group and 12.6±1.7 years in the PSF group. Pelvic fixation was performed using a Tconstruct (2 sacral and 2 iliac screws). PSF was performed with all levelled pedicle screws. In the MGCR group, upper thoracic fixation was lateral ribs (n=4), vertebral on three levels (n=9), or hybrid costo-vertebral (n=4). No blood transfusion was required. No differences were found between preoperative and postoperative lung function tests for the 2 surgical procedures. The major curve correction rate was 44% in the MCGR group and 55% in the PSF group. The pelvic obliquity at last follow-up was <5 degrees in all patients. Three unplanned surgeries occurred: 1 MGCR change after lengthening and 2 PSF-one for proximal hook migration and one for infection. All patients improved their ability to sit. CONCLUSION:In this series, PSF and MGCR allowed stable radiographic and respiratory results, with a reduced rate of global complications. Pelvic fixation with T-construct was a reliable and effective technique to correct pelvic obliquity in this population of patients.

Achieving Return to Sport (RTS) is crucial in managing ankle fractures for athletes. This study aimed to identify RTS factors post-surgical fixation of ankle fractures. A retrospective analysis was conducted on 93 active patients with surgically treated displaced or unstable ankle fractures from January 2020 to January 2021. The median follow-up was 2.12 years. Clinical, functional, and radiographic aspects were evaluated. Among the athletes, 82.8 % resumed sports post-surgery, with 26.9 % returning within 3 months and 75.3 % at 1 year. At 1 year, 40.9 % regained their pre-injury activity level. There was a significant association between RTS and AO/OTA fracture subtype (p = 0.038). Unimalleolar fractures had the best outcomes, with 100 % achieving RTS in a median of 4 months. Bimalleolar fractures had 80.77 % RTS in 6 months, and trimalleolar fractures had 65.22 % RTS in 8 months. AO/OTA subtypes B and C predicted lower RTS to pre-injury levels, with delayed recovery for bimalleolar and trimalleolar fractures. These findings underscore the impact of fracture severity on RTS, with more complex fractures leading to poorer and delayed recovery outcomes.

BACKGROUNDS:Magnetic controlled growing rods (MCGRs) have been proven to be effective in controlling early onset neuromuscular scoliosis but no study has evaluated the combination with a sacro-bi-iliac construct. The aim of our study is to report surgical management of early onset non-walkers neuromuscular scoliosis correction using MCGRs associated with a sacro-bi-iliac "T-construct" and its mid-term outcomes. Our hypothesis was that this set-up provided well correction of the pelvic obliquity and that this correction was maintained over time. METHODS:A retrospective single-center study was conducted including all consecutive neuromuscular early onset scoliosis who underwent spinopelvic fixation using "T-construct" with two MCGRS. Four millimeters lengthening was performed every 4 months during outpatient clinics sessions. All children had a low-dose biplanar stereoradiography in EOS-Chair at pre/postoperative phase, each outpatient clinic appointment and last follow-up. RESULTS:Eighteen patients were included and 17 analyzed at the last follow-up. The mean age at surgery was 9.5 (range from 5 to 12 years), the mean follow-up was 4,7 years (range from 2.5 to 6.6 years) and 8 patients had a Risser stage above four. The global complication rate was 35% (N = 6/17 patients) including three medical and three mechanical complications related to "T-construct", while the reoperation rate was 18% (N = 2 patients for wound debridement and one for iterative pelvic fixation). Cobb angle and pelvic obliquity were significantly improved by surgery (mean correction was 33.2 ° (55%) and 11 ° (77%) respectively; p < 0.001). At the last follow-up, we noted a loss of frontal Cobb angle correction (p < 0.01) whereas we did not observe any significant loss of pelvic obliquity (p > 0.9). CONCLUSIONS:Although the global complication rate was 35% (half of which are mechanical complications), the treatment combining pelvic T-construct and MCGRs provides satisfactory correction of pelvic obliquity correction, good maintenance in the medium term and may be a procedure to consider for the surgical treatment of early onset neuromuscular scoliosis. LEVEL OF EVIDENCE:IV; Retrospective cohort prognostic study.

BACKGROUND:Spinal muscular atrophy (SMA) affects the motor neurons of the anterior spinal cord, causing progressive muscle weakness and atrophy. SMN restoring therapies have led to the emergence of new phenotypes in spinal muscular atrophy (SMA), including early-onset scoliosis. Guidelines regarding the optimal treatment for scoliosis and its consequences are lacking. OBJECTIVE:To provide guidelines to help clinical decision-making and to standardise the management of neuromuscular scoliosis (NMS) in children with SMA. METHODS:We conducted a Delphi study with 31 experts between July 2023 and February 2024. Three rounds were conducted using anonymous electronic questionnaires to determine consensus on items grouped into 5 main domains: Respiratory monitoring (7 items), Trunk orthoses (4 items), Surgical approaches (13 items), Preoperative care (17), post-operative care (10 items). Experts were asked to rate their agreement with each item on a scale from 1 (strongly disagree) to 9 (strongly agree). Consensus was considered to have been achieved if the median value of the responses was ≥ 7. Items that did not reach consensus in one round were reformulated in the following round using comments provided by the experts. RESULTS:The experts included 12 orthopaedic surgeons, 9 physical medicine and rehabilitation physicians and 10 paediatricians/child neurologists. Fourty-seven items achieved consensus. In the first round, consensus was achieved for 33 of the 51 items. The second round included 15 items, and a consensus was achieved for 11 items. In the third round, 3 of the 4 items included achieved consensus and the fourth item was dropped due to a lack of agreement. CONCLUSIONS:The recommendations generated from the questionnaire provide a relevant consensus-based guidance for the multidisciplinary management of spinal deformities in children with SMA.

INTRODUCTION:Over the past decade, the use of patient-specific instrumentation (PSI) has increased exponentially as a means of improving surgical precision. In pediatric orthopedics, its adoption is more recent and differs from adult applications due to the unique characteristics of pediatric deformities and anatomical constraints, particularly the need to preserve the physis. This study aimed to assess the reliability and accuracy of PSI in performing lower limb long bone osteotomies in children. HYPOTHESIS:Planned corrections are comparable to the achieved corrections. PATIENTS AND METHODS:This bicentric retrospective study included all PSI-assisted lower limb osteotomies performed since 2021. PSI guides were developed using bilateral CT scans. Demographic data, clinical outcomes at the final follow-up, and any complications were recorded. In all cases, postoperative three-dimensional (3D) reconstruction was performed using low-dose biplanar stereo radiography (EOS) and compared to the preoperative CT-based surgical plan. For each osteotomy, 3D measurements of the primary correction in all three planes were analyzed. Postoperative reliability was defined as the difference between the preoperative plan and the achieved correction (Δ angle). A threshold of ±3 ° was used to assess the accuracy of PSI. RESULTS:Eighteen patients (21 osteotomies, mean age 14.6 ± 3.4 years) were included: 52% femoral (n = 11) and 48% tibial (n = 10). At a mean follow-up of 10 ± 5 months, one intraoperative and three postoperative complications were observed. Two patients reported residual pain at the final follow-up. Planned corrections ranged from 9 ° to 85 °, with 62% exceeding 15 °. The mean Δ angle was 11 °, with no significant difference overall (p = 0.06), but a trend toward undercorrection was observed in cases with planned corrections >15 ° (p = 0.03). DISCUSSION:The use of PSI in pediatric lower limb osteotomies tends to result in undercorrection compared to the planification, particularly in case of severe deformity. This may be explained by the greater magnitude of corrections typically required in pediatric patients compared to adults. Although these findings require confirmation through larger cohorts and 3D postoperative analysis, they should be considered when using PSI in pediatric lower limb osteotomies. LEVEL OF EVIDENCE:III; Retrospective case-control study.

Les « syndromes et dysplasies squelettiques » regroupent plus de 150 entités le plus souvent de causes génétiques. Certaines d’entre-elles entraînent des anomalies du rachis cervical, avec ou sans instabilité, déformation ou compression de la moelle épinière. Ces anomalies doivent être détectées et traitées si nécessaires car elles peuvent avoir des conséquences graves comme une tétraplégie. Jusqu’à 30 % des patients atteints de trisomie 21 sont touchés par une instabilité atloïdo-axoïdienne ou atloïdo-occipitale. La radiographie dynamique du rachis cervical en est l’outil de dépistage le plus courant. Les mucopolysaccharidoses (MPS) sont un groupe de maladies de surcharge lysosomal qui entraînent l’accumulation de glycosaminoglycanes parfois responsables d’une instabilité cranio-cervicale et d’une sténose du canal rachidien cervical. Leur surveillance nécessite la réalisation d’une IRM tous les deux ans. La neurofibromatose de type 1 ainsi que les syndromes comportant des anomalies du tissu conjonctif (syndrome de Marfan, syndrome de Loeys-Dietz, syndrome d’Ehlers-Danlos) peuvent causer des déformations sévères et instables du rachis cervical qui peuvent rester longtemps asymptomatiques. Des radiographies cervicales doivent donc être réalisées au moindre doute. Certaines chondrodysplasies rares (chondrodysplasie ponctuée, syndrome de Larsen, dysplasie métatropique) ou des anomalies de segmentation (syndrome de Klippel Feil, maladie de Sprengel) comportent des anomalies du rachis cervical qui doivent être recherchées. En cas de progression d’une déformation (habituellement en cyphose) ou d’une sténose du rachis cervical, il est important d’envisager un traitement chirurgical comportant une correction et une stabilisation. Parfois précédée d’une période de traction par halo crânien, l’instrumentation doit avoir des limites « larges » et dépasser de 2 à 3 niveaux au minimum les seules limites anatomiques de la déformation rachidienne pour éviter la survenue d’une déformation adjacente. L’utilisation de plus en plus courante des techniques de navigation chirurgicale permet de pratiquer des corrections plus importantes et des stabilisations plus performantes y compris sur des déformations sévères du rachis cervical. La vigilance et le dépistage de ces anomalies restent la clé d’un traitement précoce et préventif des complications de ces anomalies rachidiennes sur des terrains souvent difficiles. Niveau de preuve V (avis d’expert).