Dynamic Magnetic Resonance Imaging Protocol: An Effective and Useful Tool to Assess Discoid Lateral Meniscus Instability in Children.

OBJECTIVES: One of the most common symptoms in cases of discoid lateral meniscus (DLM) in children is a "snapping" knee. The clock in extension, followed by a pop in flexion, perceived by the clinician, reflects the meniscal displacement caused by the peripheral meniscocapsular detachment. Standard magnetic resonance imaging (MRI) results in a 40% false-negative rate for detecting this instability. The hypothesis was that a dynamic MRI protocol could reduce the false negative rate and improve the efficiency of the MRI in detecting the direction of instability. METHODS: Eight DLM knees (8 patients) with snapping knees (grade 2 of Lyon's classification) were included in this monocentric prospective preliminary study in a referral center of pediatric orthopaedic surgery. Every patient underwent a dynamic MRI protocol with both T2-Fat-Sat sagittal and coronal slices, performed "after the clock" and again "after the pop" in a knee with standard 20 degrees of flexion during acquisition. All the MRI data were correlated with an arthroscopic description of the peripheral tear of the DLM according to Ahn's classification to assess for diagnostic accuracy. RESULTS: The standard MRI protocol resulted in a false-negative rate of 50% for detecting the direction of instability. The dynamic MRI protocol allowed the identification of, and classification of the meniscal instability, meniscal shift, and meniscocapsular tear in 8 of 8 patients (0% false-negative rate), perfectly correlated with arthroscopic findings. CONCLUSION: This preliminary series, although short, allowed us to understand all the types of movements and lesions associated with the child's discoid meniscus. The detailed case analysis showed a strong benefit of such a protocol for planning the surgical suture procedure. The functionality and reliability of the dynamic MRI protocol is a good and method relatively simple method which does not require specific equipment, minimizing any additional cost compared with standard MRI. LEVEL OF EVIDENCE: Level IV.

Absorbable Sutures Repair Without Meniscopexy, Results in Superior Outcomes for Discoid Lateral Meniscus With Meniscocapsular Anterior Detachment in Children.

BACKGROUND: Despite the fact that meniscocapsular anterior detachment is the most common location of instability in children with a Discoid Lateral Meniscus (DLM), there is a lack of consensus about the type of repair that should be utilized for stabilization. The aim of this study was to determine the best fixation method for anterior detachment of DLM in children. Our hypothesis was that excessive rigidity with fixation would restrict meniscal mobility and increase the rate of failure or prevent full knee flexion. METHODS: This study was a retrospective single-center study consisting of 51 consecutive knees (45 children) with menico-capsular anterior detachment that underwent stabilization and minimal saucerization of the meniscus between 2007 and 2018. We aimed to compare the need for revision surgery and knee flexion between the different types of fixations utilized; namely we compared meniscopexy using anchors on the tibia (n=30) with outside-in arthroscopic soft tissue fixation (n=21), and absorbable (n=18) and nonabsorbable sutures (n=33) were compared. RESULTS: The mean age at surgery was 10.4 years (3 to 17) with a mean follow-up of 52 months (18 to 148). The group of knees treated with absorbable sutures had a significantly better rate of full knee flexion (15/18) compared with the nonabsorbable group (17/33) ( P =0.03). Despite the absence of significance ( P =007), there was a lower rate of revision surgery due to suture failure in the soft tissue fixation group (0 revision operations) compared with the meniscopexy group (5 revision operations). CONCLUSIONS: For anterior meniscocapsular detachment of DLM, it is recommended to perform soft tissue fixation with absorbable sutures, as this technique resulted in better knee flexion and a lower rate of revision surgery when compared with meniscopexy and nonabsorbable suture fixation. LEVEL OF EVIDENCE: Level III-retrospective case studies.

Asymmetric Deficit of Knee Flexion: An Occasional Symptom of Discoid Lateral Meniscus in Children.

PURPOSE: A knee flexion deficit can be the unique symptom and sign of discoid lateral meniscus (DLM) in children. The aim of this study was to investigate the accuracy of this clinical sign. The hypothesis was that deficit of knee flexion was associated with anterior rim disinsertion and posteriorly fixed DLM. METHODS: A retrospective, single-center study was conducted in a larger series of 114 operated knees for symptomatic DLM from 2007 to 2018. A focus was done on 9 knees with a deficit of knee flexion without snapping. History and clinical examination with grading of instability, preoperative magnetic resonance imaging, and arthroscopic findings were studied. RESULTS: Among 35 knees with DLM grade 3 of instability, 26 had an isolated extension deficit. Nine knees in 5 boys and 4 girls with a mean age of 9 years (range 6 to 12) had a flexion deficit. It was the only symptom of DLM in 6, and it was associated to extension deficit in 3. In the history, 8 knees were grade 2 (snapping knee) before evolving toward a flexion deficit. All had a complete DLM with posterocentral (n=7) or central (n=2) shift at magnetic resonance imaging analysis. All had DLM with posterior shift fixed during arthroscopic evaluation. Moreover, looking at the entire series, 1 knee was a false negative and had a full flexion despite a posterior and fixed DLM during arthroscopic evaluation. The sensitivity of asymmetrical knee flexion to predict posterior fixed DLM was 90%. CONCLUSIONS: An asymmetric deficit in knee flexion is highly suggestive of DLM with anterior detachment and fixed posterior meniscal shift (specificity and positive predictive value of 100%). Given this could be the only clinical sign of DLM, specific attention therefore must be paid in the assessment of knee flexion by measuring the distance between heel and bottom on both sides, especially in a knee without snapping currently but with a history of snapping (grade 3). LEVEL OF EVIDENCE: Level III.

Growth progression of anterior cruciate ligament bone grafts in a pediatric population: magnetic resonance imaging analysis.

BACKGROUND: In children, the incidence of anterior cruciate ligament (ACL) ruptures and reconstructions has significantly risen. Unfortunately, re-rupture rates following surgery are substantially higher in children than adults. Previous research suggests that smaller graft diameters are predictive of re-rupture. OBJECTIVE: This study aimed to investigate the growth progression of the ACL bone graft, specifically in terms of width and length, within the intra-articular portion and tunnels, using successive magnetic resonance imaging (MRI) scans. The hypothesis was that the ACL grafts would undergo thinning during growth. MATERIALS AND METHODS: The cohort comprised 100 patients who underwent ACL reconstruction. Among them, 37 patients with significant residual growth were selected for analysis. Of these, 4 patients experienced graft rupture, 5 had "over-the-top" techniques, 12 had missing MRI scans and 5 were lost to follow-up. Each included patient underwent two MRI scans; the analyses of which were conducted in a double-masked manner. RESULTS: A total of 13 knees (and patients) were analyzed, with a mean ± SD (range) delay of residual growth between the two MRI scans of 3.3 + / - 1.4 (1.2-5.2) years. The graft exhibited elongation, thinning and eventual integration with the surrounding bone in the tunnels. Within the intra-articular portion, the mean [95% CI] increase in graft size between the two MRI scans was 30.8% in length and 14.8% in width. The width/length ratio in the intra-articular part was 20.4% on the first MRI and 20.8% on the second MRI. Since this difference in the ratio (+ 0.4%) was not statistically significant (P=0.425), our results indicate that the grafts remained stable in terms of proportions without thinning or thickening. Therefore, the initial hypothesis was validated for the tunnel portion but not the intra-articular portion of the grafts. CONCLUSION: In children with open physes, ACL grafts demonstrate smooth growth progression in all dimensions. However, this finding does not fully explain the high rate of re-rupture observed in children. Further research is needed to elucidate the underlying factors contributing to re-rupture in this population.

Discoid lateral meniscus instability in children: part II.: Repair first to minimise the saucerisation.

PURPOSE: Despite an improved understanding of discoid lateral meniscus (DLM), the treatment of symptomatic discoid lateral meniscus remains controversial. METHODS: The aim of this retrospective, single-centred, consecutive-case study was to evaluate the clinical outcome of 60 DLM treated arthroscopically by the "meniscoplasty or saucerisation-suture" technique in children and adolescents [median (range) age 11 (4-17) years], and to investigate surgical failures. The instability was assessed before any saucerisation. The hypotheses were that: (i) the management of instability with suture first was effective and that (ii) a combined classification with clinical and MRI data had a prognostic value. RESULTS: In 57 knees (95%), the DLM was unstable, and a suture fixation was performed. After a median follow-up of 41.5 months, the median (range) IKDC score was improved from 55 (10-70) preoperatively to 90 (37.5-100) postoperatively. The median (range) Lysholm score at last follow-up was 93.5 (45-100). The procedure was effective in 49 knees (81.6%) after a single procedure. Eleven patients had a failure with a new meniscal tear after a median (range) delay of 42 months (24-60) after the initial procedure. The patterns of discoid lateral meniscus instability were not found to have a prognostic value for surgical failure since they mainly occurred after sport-related injuries. All the patients with initial repair failures but one achieved a good clinical outcome after revision repair without any further meniscectomy. CONCLUSION: In contrast to adult knees, symptomatic discoid lateral meniscus is rarely stable in children (5%). Meniscal repair is effective to preserve the meniscus tissue, but revision repair became necessary in 18% of the cases and was finally successful. Level of evidence Level III.

Hyperphosphatemic tumoral calcinosis caused by FGF23 compound heterozygous mutations: what are the therapeutic options for a better control of phosphatemia?

BACKGROUND: Hyperphosphatemic familial tumoral calcinosis (HFTC) is a rare autosomal recessive disease caused by mutations in genes encoding FGF23 or its regulators, and leading to functional deficiency or resistance to fibroblast growth factor 23 (FGF23). Subsequent biochemical features include hyperphosphatemia due to increased renal phosphate reabsorption, and increased or inappropriately normal 1,25-dihydroxyvitamin D (1,25-D) levels. CASE-DIAGNOSIS/TREATMENT: A 15-year-old girl was referred for a 1.2-kg-calcified mass of the thigh, with hyperphosphatemia (2.8 mmol/L); vascular impairment and soft tissue calcifications were already present. DNA sequencing identified compound heterozygous mutations in the FGF23 gene. Management with phosphate dietary restriction, phosphate binders (sevelamer, aluminum, nicotinamide), and acetazolamide moderately decreased serum phosphate levels; oral ketoconazole was secondary administered, leading to significantly decreased 1,25-D levels albeit only moderate additionally decreased phosphate levels. However, therapeutic compliance was questionable. Serum phosphate levels always remained far above the upper normal limit for age. The patient presented with two relapses of the thigh mass, requiring further surgery. CONCLUSIONS: We suggest that control of phosphate metabolism is crucial to prevent recurrences and vascular complications in HFTC; however, the medical management remains challenging.

2018 International Olympic Committee consensus statement on prevention, diagnosis and management of paediatric anterior cruciate ligament (ACL) injuries.

In October 2017, the International Olympic Committee hosted an international expert group of physiotherapists and orthopaedic surgeons who specialise in treating and researching paediatric ACL injuries. Representatives from the American Orthopaedic Society for Sports Medicine, European Paediatric Orthopaedic Society, European Society for Sports Traumatology, Knee Surgery & Arthroscopy, International Society of Arthroscopy Knee Surgery and Orthopaedic Sports Medicine, Pediatric Orthopaedic Society of North America and Sociedad Latinoamericana de Artroscopia, Rodilla y Deporte attended. Physiotherapists and orthopaedic surgeons with clinical and research experience in the field, and an ethics expert with substantial experience in the area of sports injuries also participated. Injury management is challenging in the current landscape of clinical uncertainty and limited scientific knowledge. Injury management decisions also occur against the backdrop of the complexity of shared decision-making with children and the potential long-term ramifications of the injury. This consensus statement addresses six fundamental clinical questions regarding the prevention, diagnosis and management of paediatric ACL injuries. The aim of this consensus statement is to provide a comprehensive, evidence-informed summary to support the clinician, and help children with ACL injury and their parents/guardians make the best possible decisions.

Ramp lesions in ACL deficient knees in children and adolescent population: a high prevalence confirmed in intercondylar and posteromedial exploration.

PURPOSE: Ramp lesions are common in ACL deficient knees. Their diagnosis is difficult and, therefore, they may be underestimated. So far, no study analyzed their prevalence in a pediatric population. The diagnosis of these Ramp lesions is of major clinical relevance because of a frequent misestimating and technic difficulties. Ramp lesions might be associated with residual knee pain and instability after ACL reconstruction. The aim of this study was to evaluate the prevalence of ramp lesions explored through a systematic intercondylar and posteromedial arthroscopic approach during an ACL reconstruction in a pediatric and adolescent population. METHODS: Children and adolescents who underwent an ACL reconstruction were screened prospectively between October 2014 and 2016. The presence or absence of a ramp lesion was evaluated after each of three arthroscopic steps: (1) an anterior approach, (2) an intercondylar inspection, and (3) a posteromedial approach. Ramp lesions were screened at each step and their prevalence was evaluated. Furthermore, their presence was correlated to age, weight, size, sex, and state of the physis (open or closed). Finally, the meniscal status on MRI and arthroscopic findings were compared. RESULTS: Fifty-six patients were analyzed. The median age was 14.0 ± 1.3 years (12-17). The median interval between injury and surgery was 11.5 months (1-108). During step 1 (anterior approach), only 1 ramp lesion (2%) was diagnosed. 13 (23%) ramp lesions were found after inspection through the intercondylar notch. No additional lesions were found with a direct view through the posteromedial approach. No correlation between ramp lesions and side, sex, weight, size, or state of physis was found. 10 ramp lesions out of 13 could not be diagnosed on MRI. CONCLUSIONS: The prevalence of ACL-associated ramp lesions in children and adolescents is similar to adult populations. A systematic inspection through the intercondylar notch is recommended during ACL reconstruction to make a precise diagnosis. The posteromedial approach is essentially useful for meniscal repair LEVEL OF EVIDENCE: Testing, previously developed diagnostic criteria in a consecutive series of patients and a universally applied "gold" standard, Level I.

2018 International Olympic Committee consensus statement on prevention, diagnosis and management of paediatric anterior cruciate ligament (ACL) injuries.

In October 2017, the International Olympic Committee hosted an international expert group of physiotherapists and orthopaedic surgeons who specialise in treating and researching paediatric anterior cruciate ligament (ACL) injuries. Representatives from the American Orthopaedic Society for Sports Medicine, European Paediatric Orthopaedic Society, European Society for Sports Traumatology, Knee Surgery and Arthroscopy, International Society of Arthroscopy Knee Surgery and Orthopaedic Sports Medicine, Pediatric Orthopaedic Society of North America, and Sociedad Latinoamericana de Artroscopia, Rodilla y Deporte attended. Physiotherapists and orthopaedic surgeons with clinical and research experience in the field, and an ethics expert with substantial experience in the area of sports injuries also participated. Injury management is challenging in the current landscape of clinical uncertainty and limited scientific knowledge. Injury management decisions also occur against the backdrop of the complexity of shared decision-making with children and the potential long-term ramifications of the injury. This consensus statement addresses six fundamental clinical questions regarding the prevention, diagnosis, and management of paediatric ACL injuries. The aim of this consensus statement is to provide a comprehensive, evidence-informed summary to support the clinician, and help children with ACL injury and their parents/guardians make the best possible decisions.

Percutaneous guided biopsy for diagnosing suspected primary malignant bone tumors in pediatric patients: a safe, accurate, and cost-saving procedure.

BACKGROUND: Percutaneous biopsy is the reference diagnostic procedure for adult musculoskeletal tumors. Its place in pediatrics is controversial and open biopsy remains recommended. OBJECTIVE: To assess diagnostic performance and feasibility of percutaneous biopsy performed on children and young adults for suspected malignant bone tumors. MATERIALS AND METHODS: We conducted a 5-year retrospective study including patients ≤21 years who underwent a bone biopsy for a suspected malignant bone tumor. We assessed diagnostic yield (percentage of analyzable biopsies), accuracy (percentage of accurate diagnoses among all analyzable biopsies) and efficacy (percentage of accurate diagnoses among all biopsies), costs, anesthetic requirements and sample availability for biomedical research. Patients diagnosed with an open biopsy were used to compare diagnostic performances, anesthetic requirements and costs. RESULTS: We included 90 percutaneous and 27 open biopsies in 117 patients. For percutaneous biopsy, diagnostic yield was 95.5% (95% confidence interval [CI] 88.8-98.7%), accuracy was 96.2% (95% CI 86.8-99.5%) and efficacy was 89.3% (95% CI 78.1-96.0%). There was no statistical difference with open biopsy (Fisher exact test, P > 0.05). Mean costs were reduced with percutaneous biopsy: €1,937 (standard deviation [SD] €2,408) versus €6,362 (SD €5,033; Mann-Whitney, P < 0.0001). Thirty-two of the 48 (67%) patients included in clinical trials and diagnosed with percutaneous biopsy had suitable samples for ancillary analyses. CONCLUSION: Percutaneous biopsy is a valid alternative to open biopsy for diagnosing pediatric and young adult primary malignant bone tumors.

Cartilaginous tibial eminence fractures in children: which recommendations for management of this new entity?

PURPOSE: Cartilaginous tibial eminence fracture (CTEF) is a new pattern of ACL rupture in children under the age of nine. MRI signs have been recently reported, but no series gave information about outcomes. It was hypothesized that primary treatment gave better results than delayed management due to frequent misdiagnosis. METHOD: This retrospective study focused on 15 patients, managed acutely (n = 7) or delayed (n = 8). The patients' median age at the time of initial injury was 6.5 years (range 5-9). Lysholm, IKDC 2000 subjective scores, and the measurement of the residual laxity by a side-to-side difference with a KT-1000 junior arthrometer were used at the time of revision. RESULTS: After a mean follow-up of 9.8 years (range 1-18.5), the mean Lysholm and IKDC subjective scores were, respectively, 97.7 ± 2.6 and 97 ± 3.4. The median residual laxity was 2 mm (range 0-4). Non-operative treatment lead to 2 failures: intermeniscal ligament entrapment and combined avulsion fracture at the femoral site. Suture fixation of the avulsed fragment allows regularly good results when performed acutely or even 4 years after the injury. The hypothesis that primary treatment gives better result than delayed treatment tends to be wrong as 2 failures were reported in each group. An ACL reconstruction was performed in 3 out of the 4 treatment failures. Progressive resorption of the avulsed fragment was noticed in 3 of the 4 failures suggesting an associated ACL resorption. CONCLUSION: CTEF has a good prognosis even after misdiagnosis and treatment at the time of non-union; this could be due to low-energy mechanism of injury and low rate of associated lesion. Orthopaedic treatment for acute minimally displaced fractures is only indicated under strict MRI control, and suture fixation is the recommended strategy in other situations. Conservative management of non-union could expose to ACL involution and cannot be recommended. LEVEL OF EVIDENCE: Retrospective case series, Level IV.

The difficult diagnosis of cartilaginous tibial eminence fractures in young children.

PURPOSE: Anterior tibial eminence fracture is the main mode of ACL failure in patients with open physes. In young children, purely cartilaginous avulsions of the tibial ACL insertion are possible. The aim of this study was to focus on patients referred for misdiagnosed cartilaginous tibial eminence fractures. METHODS: Ten young patients with cartilaginous tibial eminence fractures were identified in the hospital database. Six of them were misdiagnosed and included in this retrospective case series. Clinical data at the time of injury, radiographs and MRI were analysed in order to evaluate the causes which could have led to inappropriate management. RESULTS: The patients' median age at the time of injury was 7 years (5-8.5). The main cause of injury was a low-energy domestic accident (n = 4). Radiographs at the time of injury were normal (n = 4) or showed a very thin ossification (n = 2). The traditional MRI findings of ACL injuries were all negative. On T2 sequences, an epiphyseal fluid signal allowed for a retrospective diagnosis. Cartilaginous tibial eminence fractures were regularly prolonged posteriorly giving a 'double-PCL sign' in 4 of the 6 patients. On a median of 6 months (2.5-48) after the injury, patients were referred for repeat giving ways (n = 5) and/or limitation of extension or hyperextension (n = 4). Symptoms were related to non-union, ossification and secondary enlargement of the avulsed fragment. CONCLUSIONS: Post-traumatic knee joint effusions in children aged 9 or younger, even occurring after a low energy trauma and with normal radiographs, should suggest a cartilaginous tibial eminence fracture. Systematic MRI examinations should be mandatory in these patients in whom the avulsed fragment may appear as a double-PCL sign. During follow-up, new radiographs are recommended. A better knowledge of this rare entity should allow us to avoid misdiagnosis and to perform an early refixation of the avulsed fragment.

Reconstruction with antibiotic loaded single-side gore-tex "Tartine" methyl-methacrylate cementoplasty for pediatric chest wall reconstruction: A 10-case series.

INTRODUCTION: Chest wall reconstruction in children after large resection of tumors may be performed with rigid or soft materials. Cementoplasty is commonly used with the "Sandwich" method i.e. gore-tex meshes surrounding both faces of the cement. HYPOTHESIS: Is antibiotic loaded single-side gore-tex "Tartine" methyl-methacrylate cementoplasty an interesting alternative to the double-side "sandwich" method for chest wall reconstruction? MATERIAL AND METHODS: Consecutive patients who were treated from 2011 to 2023 in our hospital were included. RESULTS: Among the ten children treated with a median 5.6 years follow-up, there were no surgical complications related to the reconstruction, loss of function, infections, post operative complications (versus 22.7% in meta-analysis encompassing the 50 rigid reconstructions reported worldwide) nor scoliosis (versus 25%). Three patients have an asymmetric chest wall appearance. DISCUSSION: "Tartine" cementoplasty is a simple, low-cost technique for pediatric chest wall reconstruction. It is well tolerated and checks key demands for chest wall reconstructions. LEVEL OF EVIDENCE: IV; retrospective case series.

Septic arthritis caused by noncapsulated Haemophilus influenzae.

Since the introduction of type b Haemophilus influenzae vaccination, noncapsulated H. influenzae has become responsible for most cases of invasive H. influenzae diseases. In our two cases of septic arthritis, we isolated strains with ß-lactamase-positive amoxicillin-clavulanate resistance (BLPACR). Thus, the increasing prevalence of BLPACR should be taken into account when empirical therapy is chosen for septic arthritis.

Arthroscopic Suture-Saucerization of Discoid Meniscus Allows Volume Conservation but Does Not Fully Restore Coverage.

Purpose: The purpose of this study was to quantify the effect of meniscoplasty suture-saucerization on volume and surface coverage of lateral discoid menisci. Methods: This retrospective study included all consecutive 10 patients treated between 2014 and 2019 who had magnetic resonance imaging before and after surgery and 15 controls. The MITK 3M3 semiautomatic software was used to segment the meniscus and cartilage before and after surgery to measure the percentage of meniscus coverage on the tibial cartilage. Results are compared to control patients without knee pathology matched on sex and age with Student t test. Results: Discoid meniscus surface and volume before surgery were respectively 597 mm2 (range, 550-887 mm2) and 2,822 mm³ (1,571-3,407 mm³), representing 74.5% (56%-89%) of the tibial cartilage surface. After surgery, it decreased to 422 mm2 (229-569 mm2) and 1,235 mm³ (680-1,738 mm³), leading to 45.7% (22.5%-68.6%) coverage. In the control group, median surface was 457 mm2 (314-641 mm2), volume was 1,321 mm3 (641-2,240 mm3), and tibial coverage was 55% (41%-77%). Altogether, meniscus volume after surgery was similar to normal, while coverage was significantly lower than controls (P = .04). Conclusions: Meniscoplasty suture-saucerization procedure may allow meniscus sparing and restauration of a similar to normal meniscus volume. Meniscus surface and coverage are diminished compared to controls. Both surface and volume normalization is usually not achievable without decreasing the thickness of the rather thick discoid meniscus. Clinical Relevance: Both surface and volume normalization is usually not achievable without decreasing the thickness of thick discoid menisci.

Epiphyseal-sparing biological reconstruction of bone sarcoma in children: Focus on articular function and growth.

INTRODUCTION: Epiphyseal preservation surgery and biological reconstruction after resection of metaphyseal bone sarcoma in children is a surgical challenge which can only be justified if future joint function is maintained. HYPOTHESIS: The main hypothesis of this work was that long-term function was maintained. The secondary hypotheses were that local control of the disease and growth restoration were achieved, at the cost of an acceptable number of complications. MATERIAL AND METHOD: This was a retrospective study of 14 children with a median age of 8 years [2-14] at the time of surgery. The tumors (Ewing's sarcoma or osteosarcoma) were mostly situated at the knee (n=9) and hip (n=3). The reconstruction used an induced membrane (n=7) or an allograft (n=7). We studied joint function, mechanisms contributing to loss of growth, surgical complications and survival at the last follow-up. RESULTS: At the median follow-up of 76 months [24-130], 9 out of 14 patients required revision for non-union, and 4 of them required a second revision. At the last follow-up, 82% of the length had been restored, due to 3 bone lengthenings and 7 contralateral epiphysiodeses. Preserved joint function was excellent with an average modified MSTS score of 28.3/30 [24-30]. No local recurrence was reported. DISCUSSION: Our experience of epiphyseal preservation allows local control of the disease and very good function but at the cost of a cumbersome surgical program (12 out of 14 patients were reoperated on, with an average of 1.2 interventions per patient). The main difficulty is the growth management, most often by complex programs of alternating bone lengthening and shortening. LEVEL OF EVIDENCE: IV, retrospective study.

Isolated MPFL reconstruction with soft tissue femoral fixation technique in 54 skeletally immature patients: Clinical outcomes at 2 years follow-up. A French multicenter retrospective study.

BACKGROUND: Medial patello-femoral ligament (MPFL) reconstruction is one of the therapeutic options to treat patellofemoral instability. Classically, a à la carte treatment of skeletal and ligament abnormalities is described. This option is difficult to achieve in children because bony procedures can damage the femoral and/or tibial growth plate. The objective was to evaluate a strategy for isolated reconstruction of the MPFL in the treatment of objective patellar instabilities in children, in a large cohort. The return to sport, knee function and pain or discomfort were studied as secondary endpoints. METHODS: This French multicenter retrospective study included 54 pediatric patients with objective patellofemoral instability. Patients were included if they had presented at least 2 episodes of objective patella dislocation. A Deie-like technique with gracilis tendon graft, soft tissue femoral fixation and patellar bone tunnels for patellar fixation was used. Recurrence of dislocation was studied as the primary endpoint, and the recurrence rate was compared with the literature. A comparison of functional scores (Kujala, Lille femoro-patellar instability score or LFPI Score and Tegner activity score) and NRS between pre- and postoperative was studied as a secondary objective. RESULTS: A recurrence of femoro-patellar instability was observed for five patients within 2 years follow up (9%). A significant improvement of the Kujala, LFPI score, Tegner and NRS scores was observed (p<0.001). CONCLUSION: Isolated reconstruction of the MPFL presents a risk of recurrence of 9% at 2years follow-up. This technique significantly improves the functional scores of the knee. This modified Deie technique provides good clinical and functional results, allowing return to sports with an acceptable risk of recurrence of patellar dislocation, similar to those observed in the literature. Isolated MPFL reconstruction as a first-line treatment appears to be a reliable and effective technique in terms of recurrence of dislocation and functional scores. It allows early recovery and rehabilitation and has lower morbidity than procedures requiring bone gestures. LEVEL OF EVIDENCE: III, retrospective comparative study.

Identification of a novel translocation producing an in-frame fusion of TAF15 and ETV4 in a case of extraosseous Ewing sarcoma revealed in the prenatal period.

Ewing sarcoma (ES) is a highly malignant round cell sarcoma, characterized by gene fusion involving FET (FUS, EWSR1, TAF15) and ETS family genes, respectively. The involvement of the EWSR1 gene has been reported in approximately 90% of cases of ES, with the EWSR1::FLI1 fusion being the most frequent. We report the case of a newborn with a localized soft tissue paravertebral neoplasm diagnosed prenatally. Histopathology and immunophenotype were consistent with a CD99 + , NKX2.2 + undifferentiated round cell sarcoma (URSC); whole-exome RNA-sequencing demonstrated an undescribed in-frame TAF15::ETV4 fusion transcript, while consensus clustering analysis showed high transcriptomic proximity to the ES group. Given clinical context, high tumor chemosensitivity to ES conventional drugs, morphological characteristics, nature of the fusion partners involved, and high transcriptomic proximity to bona fide ESs, this case may represent a new genetic variant of ES.

Locally aggressive rarely metastazing tumors and low-grade sarcoma in children, adolescents and young adults: The benefits of a national network.

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.