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Background: Fibrous dysplasia (FD) is a rare condition in which normal spongy and cortical bone is replaced by non-neoplastic fibrous tissue, leading to weakened bone matrix and increased risk of pathological fractures and deformities. Treating these deformities poses a significant challenge for surgeons. While various cases of surgical stabilization and limb lengthening using intramedullary nails have been reported, there is limited evidence on the use of Motorized Intramedullary Limb-Lengthening Nails (MILLNs) in FD patients. This case report presents the clinical history of a patient with FD who underwent multiple surgical interventions to address severe lower limb length discrepancy (LLD) and angular deformity caused by multiple fractures., Case Presentation: A sixteen-year-old Caucasian girl with polyostotic FD developed a severe post-traumatic LLD of 10 cm on the right side, associated with coxa vara, valgus knee, and patellar instability. The deformity of the proximal femur was addressed with a valgus and derotational femoral osteotomy. However, this procedure exacerbated the knee's valgus deformity and only partially corrected the LLD, leading to the decision to proceed with femoral lengthening. A retrograde magnetic intramedullary nail (PRECICE, NuVasive) was utilized for this purpose. Approximately three months postoperatively, radiographs revealed the loosening of the proximal anchoring screw, while the nail had reached maximum distraction. We then proposed reactivating the previously implanted nail. Nine months after the final surgery, standing long-leg radiographs showed a residual shortening of 1 cm, with excellent healing at the fracture sites and the nail and screws remaining securely in place. The patient was monitored regularly, with the latest follow-up occurring four years and five months after the conclusion of the last lengthening procedure., Conclusions: This case report describes the reactivation of a MILLN in a patient with polyostotic fibrous dysplasia. While nail reactivation has been previously described in the literature, to our knowledge, it has not been reported for treating complications arising from FD. In cases of mechanical complications, this approach can equalize leg length discrepancies and correct deformities, avoiding additional invasive surgeries and reducing healthcare costs. As this is an off-label treatment, preoperative consent from both the patient and the parents is required.

Background: Lower limb length discrepancy (LLD) in children and adolescents, often due to congenital or acquired conditions, is treated to achieve limb equality and alignment, optimizing function and minimizing cosmetic concerns for an active adulthood. This study evaluated the Health-Related Quality of Life (HRQoL) and physical functioning of adults who underwent unilateral limb lengthening with circular external fixators (EFs) in childhood., Methods: Fifty patients treated at a median age of 14.9 years completed the Short Form 36 (SF-36) and Stanmore Limb Reconstruction Score (SLRS) questionnaires in adulthood, with a median follow-up of 8.9 years., Results: Among the 50 patients, 38 underwent a single limb lengthening (21 tibia, 12 femur, 5 both), while 12 required multiple cycles. The median residual LLD was 0.4 cm, with 12 patients (24%) having over 2 cm. Complications occurred in 67% of procedures, mainly due to prolonged healing. Physical and mental health scores were significantly lower than normative data. The mean Physical Component Summary was 52.2 ± 7.2 ( p = 0.20). The mean Mental Component Summary was 43.9 ± 8.6 ( p = 0.001), notably lower in congenital LLD cases. Many SLRS items (Pain, Social, Physical Function, Work, and Emotions) strongly correlated with SF-36 items., Conclusions: Adults treated with distraction osteogenesis for congenital LLD show normal physical but lower mental health scores compared to peers. Lengthening procedure characteristics did not significantly impact mental health. Routine psychological and social assessments are recommended to prevent long-term distress by providing appropriate support.

Background : Infantile tibia vara (ITV) is a rare proximal tibia deformity in infancy, leading to progressive knee varus. High tibial osteotomy is commonly practiced but has high recurrence rates. This study analyzed factors affecting treatment failure and recurrence in children undergoing opening-wedge high tibial osteotomy (OWHTO) for ITV. Methods : We retrospectively studied children with ITV who had OWHTO with a press-fit cancellous bone allograft between 2000 and 2020, with ≥2-year follow-up. Outcomes included recurrence (knee varus with tibiofemoral angle > 10°), complications, and reintervention. Results : We analyzed 39 knees in 29 patients (mean age: 4.8 ± 1.9 years; median follow-up: 7.4 years). Recurrence occurred in 22 cases (56%). Age at surgery significantly influenced recurrence, with rates of 16% before age 5 versus 95% later (hazard ratio: 12.0, p = 0.001). Langenskiöld stage also affected recurrence (β-coefficient: 2.7, 95% C.I. 1.0-4.5, p = 0.002; pseudo-R-squared: 0.50, p = 0.001), with recurrence in all stage IV or higher cases. Conclusions : Early diagnosis and treatment before age 5, ideally with Langenskiöld stage III or lower, are crucial for stable correction with OWHTO alone. Late, high-grade ITV may require combined, acute or gradual, and/or staged correction. Further evidence is needed for optimal management.

Purpose: To evaluate the health-related quality of life and associated risk factors for Multiple Osteochondromas patients., Methods: A cross-sectional, observational study was conducted from May to December 2022 during the routine visit to the referral center for rare skeletal disorders. All patients with Multiple Osteochondromas aged ≥ 3 years were included. EuroQol 5-dimension questionnaires, and demographic, clinical, and surgical history data were collected. Descriptive statistics, Fisher's exact test, One-sample t-test, Spearman's correlation, and multiple linear and logistic regression were performed to analyze the data. Results are reported following STROBE guidelines., Results: A total of 128 patients were included in the study, with a mean age of 14 [SD, 10] years. The mean EQ-5D Index Value was 0.863 [SD, 0.200] and the EQ-VAS was 84 [SD, 19] with a positive correlation between two scores [r = 0.541, p < 0.001]. Patients frequently referred problems in pain/discomfort [78.8%], anxiety/depression [50%], and usual activities [38.8%] dimensions. Increasing age was the common risk factor for health-related quality of life [p < 0.000], as well as Index Value and VAS scores were significantly lower in surgical patients [p = 0.001 and p < 0.001, respectively]., Conclusion: Increasing age and surgical procedures were found highly associated with reduced health-related quality of life in Multiple Osteochondromas patients. Our findings provide relevant information to support the establishment of patient-centered healthcare pathways and pave the way for further research into medical and non-medical therapeutic strategies for these patients., (© 2024. The Author(s).)

(1) Background: Orthopedic surgery has been transformed by 3D-printed personalized instruments (3DP-PSIs), which enhance precision and reduce complications. Hospitals are adopting in-house 3D printing facilities, using cost-effective methods like Fused Deposition Modeling (FDM) with materials like Polylactic acid (PLA) to create 3DP-PSI. PLA's temperature limitations can be overcome by annealing High-Temperature PLA (ann-HTPLA), enabling steam sterilization without compromising properties. Our study examines the in vivo efficacy of ann-HTPLA 3DP-PSI in pediatric orthopedic surgery. (2) Methods: we investigated safety and efficacy using ann-HTPLA 3DP-PSI produced at an "in-office" 3D-printing Point-of-Care (3DP-PoC) aimed at correcting limb deformities in pediatric patients. Data on 3DP-PSI dimensions and printing parameters were collected, along with usability and complications. (3) Results: Eighty-three ann-HTPLA 3DP-PSIs were utilized in 33 patients (47 bone segments). The smallest guide used measured 3.8 cm 3 , and the largest measured 58.8 cm 3 . Seventy-nine PSIs (95.2%; 95% C.I.: 88.1-98.7%) demonstrated effective use without issues. Out of 47 procedures, 11 had complications, including 2 infections (4.3%; 95% CI: 0.5-14.5%). Intraoperative use of 3DP-PSIs did not significantly increase infection rates or other complications. (4) Conclusions: ann-HTPLA has proven satisfactory usability and safety as a suitable material for producing 3DP-PSI in an "in-office" 3DP-PoC.

Introduction: The present study aims to describe a large cohort of Italian patients affected by osteogenesis imperfecta, providing a picture of the clinical bony and non-bony features and the molecular background to improve knowledge of the disease to inform appropriate management in clinical practice., Methods: A total of 568 subjects (from 446 unrelated Italian families) affected by osteogenesis imperfecta who received outpatient care at Istituto Ortopedico Rizzoli from 2006 to 2021 were considered in the present study., Results: Skeletal and extraskeletal features were analyzed showing a lower height (mean z-scores equal to -1.54 for male patients and -1.47 for female patients) compared with the general Italian population. Half of the patient population showed one or more deformities, and most of the patients had suffered a relatively low number of fractures (<10). An alteration in the sclera color was identified in 447 patients. Similarly, several extraskeletal features, like deafness, dental abnormalities, and cardiac problems, were investigated. Additionally, inheritance and genetic background were evaluated, showing that most of the patients have a positive family history and the majority of pathogenic variants detected were on collagen genes, as per literature., Conclusion: This study supports the definition of a clear picture of the heterogeneous clinical manifestations leading to variable severity in terms of skeletal and extra-skeletal traits and of the genetic background of an Italian population of osteogenesis imperfecta patients. In this perspective, this clearly highlights the crucial role of standardized and structured collection of high-quality data in disease registries particularly in rare disease scenarios, helping clinicians in disease monitoring and follow-up to improve clinical practice., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest. The author(s) declared that they were an editorial board member of Frontiers, at the time of submission. This had no impact on the peer review process and the final decision, (Copyright © 2024 Mordenti, Boarini, Banchelli, Antonioli, Corsini, Gnoli, Locatelli, Pedrini, Staals, Trisolino, Lanza and Sangiorgi.)

Background: Guided-growth modulation is a first-line treatment widely adopted to correct lower-limb angular deformities and limb-length discrepancies (LLD) in the paediatric population., Methods: We conducted a retrospective study to evaluate the safety and performance of a new construct (8-Plate Plus or Guided-Growth Plate System Plus, Orthofix S.r.l) used to correct angular deformities and LLD in non-skeletally mature children. The primary endpoint was safety (from plate implantation to removal). The secondary endpoint was performance; patients treated for LLD achieved complete correction if a pre- and post-surgery difference of <0 was observed; angular deformities performance was measured in terms of IMD, ICD, mMPTA, and mLDFA., Results: We performed 69 procedures in 41 patients. A total of 10 patients had an LLD, and 31 had an angular deformity. We observed nine minor complications in the hemiepiphysiodesis group. One patient experienced rebound. All 10 LLD patient treatments were successful. A total of 30/31 patients with an angular deformity had a successful treatment; the remaining patient had a partial correction., Conclusions: Guided-growth by temporary epiphysiodesis or hemiepiphysiodesis was safe and effective for angular deformities and limb-length discrepancies. Further prospective and/or randomized controlled trial studies assessing more significant cohorts of patients and a comparison group could add evidence to our findings., Competing Interests: The authors declare no conflicts of interest.

(1) Background: Virtual reality and 3D printing are transforming orthopedic surgery by enabling personalized three-dimensional (3D) models for surgical planning and Patient-Specific Instruments (PSIs). Hospitals are establishing in-house 3D printing centers to reduce costs and improve patient care. Pediatric orthopedic surgery also benefits from these technologies, enhancing the precision and personalization of treatments. This study presents preliminary results of an In-Office 3D Printing Point of Care (PoC), outlining considerations and challenges in using this program for treating lower limb deformities in pediatric patients through Virtual Surgical Planning (VSP) and 3D-printed Patient-Specific Instruments (PSIs). (2) Materials and Methods: Pediatric patients with congenital or acquired lower limb deformities undergoing surgical correction based on VSP, incorporating 3D-printed PSIs when required, were included in this study. The entire process of VSP and 3D printing at the In-Office PoC was illustrated. Data about deformity characteristics, surgical procedures, and outcomes, including the accuracy of angular correction, surgical times, and complications, were reported. (3) Results: In total, 39 bone correction procedures in 29 patients with a mean age of 11.6 ± 4.7 years (range 3.1-18.5 years) were performed according to VSP. Among them, 23 procedures were accomplished with PSIs. Surgeries with PSIs were 45 min shorter, with fewer fluoroscopy shots. Optimal correction was achieved in 37% of procedures, while the remaining cases showed under-corrections (41%) or over-corrections (22%). Major complications were observed in four patients (13.8%). (4) Conclusions: The In-Office 3D Printing Point of Care is becoming an essential tool for planning and executing complex corrections of lower limb deformities, but additional research is needed for optimizing the prediction and accuracy of the achieved corrections.

(1) Background: The adoption of Virtual Surgical Planning (VSP) and 3D technologies is rapidly growing within the field of orthopedic surgery, opening the door to highly innovative and individually tailored surgical techniques. We present an innovative correction approach successfully used in a child affected by "windswept deformity" of the knees. (2) Methods: We report a case involving a child diagnosed with "windswept deformity" of the knees. This condition was successfully addressed through a one-stage bilateral osteotomy of the distal femur. Notably, the wedge removed from the valgus side was flipped and employed on the varus side to achieve the correction of both knees simultaneously. The surgical technique was entirely conceptualized, simulated, and planned in a virtual environment. Customized cutting guides and bony models were produced at an in-hospital 3D printing point of care and used during the operation. (3) Results: The surgery was carried out according to the VSP, resulting in favorable outcomes. We achieved good corrections of the angular deformity with an absolute difference from the planned correction of 2° on the right side and 1° on the left side. Moreover, this precision not only improved surgical outcomes but also reduced the procedure's duration and overall cost, highlighting the efficiency of our approach. (4) Conclusions: The integration of VSP and 3D printing into the surgical treatment of rare limb anomalies not only deepens our understanding of these deformities but also opens the door to the development of innovative, personalized, and adaptable approaches for addressing these unique conditions.

Background: Blount's disease is a growth disorder of the proximal tibia that causes progressive genu varum in children. Surgical treatment is recommended if the deformity worsens, but which intervention is best remains controversial. This study aims to identify factors influencing outcomes and determine the most effective surgical approach., Methods: A systematic review was conducted of studies published before January 2022., Results: In total, 63 retrospective studies with CEBM IIIb/IV levels were included (1672 knees in 1234 patients). The most commonly reported treatment was acute correction via osteotomy (47%), followed by hemiepiphysiodesis (22%) and gradual correction (18%). Combined procedures were reported in 13% of cases. The overall recurrence rate was 18%, with a significant difference when comparing the recurrence rates after gradual correction with those after hemiepiphysiodesis (7% and 29%, respectively). Major complications beyond recurrence were observed in 5% of cases. A meta-analysis of the available raw data showed a significantly increased recurrence rate (39%) among treated children who were between 4.5 and 11.25 years of age and were followed for a minimum follow-up of 2.5 years., Conclusions: Overall, poor evidence with which to establish an optimal treatment for Blount's disease was found. This study remarked on the need for early diagnosis, classification, and treatment of infantile tibia vara, since a significant rate of recurrence was found in neglected cases.

The use of external fixators (EFs) for lower limb lengthening is common for treating lower limb length discrepancy (LLD) in children. The concern at present revolves around extended treatment times, with some suggesting a healing index (HI) > 45 days/cm as a major complication. The aim of this study is to assess the factors affecting bone healing and treatment duration in children who undergo limb lengthening for LLD using circular EFs. A total of 240 lengthening procedures on 178 children affected by congenital or acquired LLDs (mean age at surgery 13.8 ± 2.8 years) were retrospectively evaluated. Complications according to Lascombes' classification and treatment duration factors were analyzed. Mean HI was 57 ± 25 days/cm for the femur and 55 ± 24 days/cm for the tibia, with an HI > 45 days/cm in 64% of the procedures. A total of 189 procedures (79%) reported complications; 85 had an HI > 45 days/cm as the sole complication. While reducing the frame time is crucial, revising the classifications is necessary to avoid the overestimation of complications.

Over the last decade, evidence has mounted for a prominent etiologic role of femoroacetabular impingement (FAI) in the development of early hip osteoarthritis (OA). The aim of this study was to compare the ultrastructure and tissue composition of the hip labrum in healthy and pathological conditions, as FAI and OA, to provide understanding of structural changes which might be helpful in the future to design targeted therapies and improve treatment indications. We analyzed labral tissue samples from five healthy multi-organ donors (MCDs) (median age, 38 years), five FAI patients (median age, 37 years) and five late-stage OA patients undergoing total hip replacement (median age, 56 years). We evaluated morpho-functional by histology and transmission electron microscopy. Extracellular matrix (ECM) structure changes were similar in specimens from FAI compared to those from patients with OA (more severe in the latter) showing disorganization of collagen fibers and increased proteoglycan content. In FAI and in OA nuclei the chromatin was condensed, organelle degenerated and cytoplasm vacuolized. Areas of calcification were mainly observed in FAI and OA labrum, as well as apoptotic-like features. We showed that labral tissue of patients with FAI had similar pathological alterations of tissue obtained from OA patients, suggesting that FAI patients might have high susceptibility to develop OA., (© 2023. The Author(s).)

(1) Background: The application of computer-aided planning in the surgical treatment of post-traumatic forearm deformities has been increasingly widening the range of techniques over the last two decades. We present the "flipping-wedge osteotomy", a promising geometrical approach to correct uniapical deformities defined during our experience with virtual surgical planning (VSP); (2) Methods: a case of post-traumatic distal radius deformity (magnitude 43°) treated with a flipping-wedge osteotomy in an 11-year-old girl is reported, presenting the planning rationale, its geometrical demonstration, and the outcome of the procedure; (3) Results: surgery achieved correction of both the angular and rotational deformities with a neutral ulnar variance; (4) Conclusions: flipping-wedge osteotomy may be a viable option to achieve correction in forearm deformities, and it deserves further clinical investigation.

Background: Patellar instability is the most common disorder of the knee during childhood and adolescence. Surgical treatment significantly reduces the rate of redislocation, but the underlying pathologies and pattern of instability may affect the results. We aimed to report the clinical and functional outcomes of the three-in-one procedure for patellar realignment in a cohort of skeletally immature patients with or without syndromes and various patterns of chronic patellar instability., Methods: We retrospectively investigated 126 skeletally immature patients (168 knees) affected by idiopathic or syndromic patellar instability, who underwent patella realignment through a three-in-one procedure. We classified the instability according to the score proposed by Parikh and Lykissas., Results: Patellar dislocation was idiopathic in 71 patients (94 knees; 56.0%) and syndromic in 55 (74 knees; 44.0%). The mean age at surgery was 11.5 years (range 4-18) and was significantly lower in syndromic patients. Syndromic patients also exhibited more severe clinical pattern at presentation, based on the Parikh and Lykissas score. The mean follow-up was 5.3 years (range 1.0-15.4). Redislocation occurred in 19 cases, with 10 cases requiring further realignment. The Parikh and Lykissas score and the presence of congenital ligamentous laxity were independent predictors of failure. A total of 22 knees in 18 patients required additional surgical procedures. The post-operative Kujala score was significantly lower in patients with syndromic patellar instability., Conclusions: The type of instability and the presence of underlying syndromes negatively affect the rate of redislocation and the clinical and functional outcome following patellar realignment through the three-in-one procedure. We recommend the consideration of alternative surgical strategies, especially in children with severe syndromic patellar dislocation.

Complex deformities of lower limbs are frequent in children with genetic or metabolic skeletal disorders. Early correction is frequently required, but it is technically difficult and burdened by complications and recurrence. Herein, we described the case of a 7-year-old girl affected by severe bilateral genu varum due to spondyloepiphyseal dysplasia. The patient was treated by patient-specific osteotomies and customized structural wedge allograft using Virtual Surgical Planning (VSP) and 3D-printed patient-specific instrumentation (PSI). The entire process was performed through an in-hospital 3D-printing Point-of-Care (POC). VSP and 3D-printing applied to pediatric orthopedic surgery may allow personalization of corrective osteotomies and customization of structural allografts by using low-cost in-hospital POC. However, optimal and definitive alignment is rarely achieved in such severe deformities in growing skeleton through a single operation.

Osteopetrosis (from the Greek "osteo": bone; "petrosis": stone) is a clinically and genetically heterogeneous group of rare diseases of the skeleton, sharing the same main characteristic of an abnormally increased bone density. Dense bones in radiological studies are considered the hallmark of these diseases, and the reason for the common term used: "Marble bone disease". Interestingly, a radiologist, Dr. Albers-Schonberg, described this disease for the first time in Germany in 1904. Indeed, radiology has a key role in the clinical diagnosis of osteopetrosis and is fundamental in assessing the disease severity and complications, as well as in follow-up controls and the evaluation of the response to treatment. Osteopetrosis includes a broad spectrum of genetic mutations with very different clinical symptoms, age onset, and prognosis (from mild to severe). This diversity translates into different imaging patterns related to specific mutations, and different disease severity. The main recognized types of osteopetrosis are the infantile malignant forms with autosomal recessive transmission (ARO-including the rarer X-linked recessive form); the intermediate autosomal recessive form (IAO); and the autosomal dominant ones ADO, type I, and type II, the latter being called 'Albers-Schonberg' disease. Imaging features may change among those distinct types with different patterns, severities, skeletal segment involvement, and speeds of progression. There are several classical and well-recognized radiological features related to osteopetrosis: increased bone density (all types with different degrees of severity assuming a 'Marble Bone Appearance' especially in the ARO type), different metaphyseal alterations/enlargement including the so-called 'Erlenmeyer flask deformity' (particularly of femoral bones, more frequent in ADO type 2, and less frequent in ARO and IAO), 'bone in bone' appearance (more frequent in ADO type 2, less frequent in ARO and IAO), and 'rugger-jersey spine' appearance (typical of ADO type 2). After conducting an overview of the epidemiological and clinical characteristic of the disease, this review article aims at summarizing the main radiological features found in different forms of osteopetrosis together with their inheritance pattern.

Congenital clubfoot is a common pediatric malformation that affects approximately 0.1% of all births. 80% of the cases appear isolated, while 20% can be secondary or associated with complex syndromes. To date, two genes that appear to play an important role are PTIX1 and TBX4 , but their actual impact is still unclear. Our study aimed to evaluate the prevalence of pathogenic variants in PITX1 and TBX4 in Italian patients with idiopathic clubfoot. PITX1 and TBX4 genes were analyzed by sequence and SNP array in 162 patients. We detected only four nucleotide variants in TBX4 , predicted to be benign or likely benign. CNV analysis did not reveal duplications or deletions involving both genes and intragenic structural variants. Our data proved that the idiopathic form of congenital clubfoot was rarely associated with mutations and CNVs on PITX1 and TBX4 . Although in some patients, the disease was caused by mutations in both genes; they were responsible for only a tiny minority of cases, at least in the Italian population. It was not excluded that other genes belonging to the same TBX4-PITX1 axis were involved, even if genetic complexity at the origin of clubfoot required the involvement of other factors.

Background and Objective: The discoid lateral meniscus (DLM) is a congenital abnormality of the meniscal shape, characterized by a typical central hypertrophy and a diameter larger than a regular meniscus, potentially leading to knee pain and symptoms, especially in children. The present study provides an update and a general review of this uncommon meniscal pathology. The incidence of discoid meniscus is about 0.4-17% for the lateral and 0.1-0.3% for the medial, although, being often asymptomatic, the true prevalence is unknown. We aim to enhance awareness on this subject to medical care provider., Methods: A literature search was performed on PubMed, including articles written in English until October 2021., Key Content and Findings: The articles regarding etiology, diagnosis and management of DLM in children or in patients younger than 18 years were reviewed using the narrative approach., Conclusions: Recent literature has shown that DLM is one of the most frequent congenital anomalies of the knee encountered during childhood. While asymptomatic children with incidental finding can be managed nonoperatively, symptomatic painful DLM should be addressed surgically, restoring typical anatomy using saucerization, tear repair, and stable fixation of the meniscus. The risk of osteoarthritis progression seems to be higher in children with operated DLM, imposing prolonged follow-up and cartilage preserving strategies for these patients., Competing Interests: Conflicts of Interest: All authors have completed the ICMJE uniform disclosure form (available at https://aoj.amegroups.com/article/view/10.21037/aoj-21-31/coif). The series “The Lateral Meniscus” was commissioned by the editorial office without any funding or sponsorship. AG served as the unpaid Guest Editor of the series. SZ served as the unpaid Guest Editor of the series and serves as an unpaid editorial board member of Annals of Joint from March 2021 to February 2023. The authors have no other conflicts of interest to declare., (2022 Annals of Joint. All rights reserved.)

Purpose: The increasing incidence of knee injuries among children is well known by sports physicians. Papers dealing with this topic have often collected patient-reported outcomes measures (PROMs) in a retrospective manner; this limitation could lead to a misinterpretation of the results, because pediatric patients might not remember their preoperative conditions adequately. This study aims to evaluate the reliability and the reproducibility of the IKDC pediatric score when administered retrospectively at a 12-month follow-up., Methods: From September 2018 and June 2019, all patients aged 7-18 scheduled for surgery due to different knee pathologies in a single center were considered eligible. Parents were contacted by phone for consent. An open-source platform was implemented to collect the responses: two surveys were created (Q1, Q2). They included general information and the Pedi-IKDC score. Q1 was completed prospectively, while Q2 was completed 12 months after surgery. The two questionnaires were identical, and patients were carefully advised to complete Q2 recalling their health status before surgery. ICC and the concordance correlation coefficient (ρ c ) were used to assess the reproducibility between the prospective and recalled scores., Results: Sixty-six patients responded to Q1 and Q2, and the mean age was 12.9 ± 2.2 years at Q1 and 14.1 ± 2.2 years at Q2. The mean time between Q1 and Q2 was 14.1 ± 2.1 months. Between prospective-IKDC and recall-IKDC, the ICC coefficient was "poor" at 0.32 (CI 0.09 to 0.5) and the ρ c was "poor" at 0.4 (CI 0.29 to 0.51). Mean prospective-IKDC was 76.8 ± 23.52 mean recalled-IKDC was 60.4 ± 11.5 (P < 0.0001), while mean difference was -16.3 ± 2.09. Simple linear regression models showed that Δ-IKDC is independently associated with age at Q1 (R 2  = 0.2676; P0.0001)  and prospective-IKDC (R 2  = 0.653; P < 0.0001)., Conclusions: Retrospective collection of the Pedi-IKDC score is not reliable and has high recall bias. This should be avoided in children with knee conditions., Level of Evidence: III., (© 2022. The Author(s) under exclusive licence to European Society of Sports Traumatology, Knee Surgery, Arthroscopy (ESSKA).)

Background: The aim of the study was to examine the relationship among patients' characteristics, intraoperative pathology and pre/post-operative symptoms in a cohort of patients undergoing arthroscopic partial meniscectomy for symptomatic meniscal tears., Methods: Clinical data were collected (age, sex, body mass index, time to surgery, trauma). Intraoperative cartilage pathology was assessed with Outerbridge score. Meniscal tears were graded with the ISAKOS classification. Synovial inflammation was scored using the Macro-score. Patient symptoms were assessed pre/post-operatively using the KOOS instrument., Results: In the series of 109 patients (median age 47 years), 50% of the meniscal tears were traumatic; 85% of patients showed mild to moderate synovitis; 52 (47.7%) patients had multiple cartilage defects and 31 (28.4%) exhibited a single focal chondral lesion. Outerbridge scores significantly correlated with patient age, BMI and synovial inflammation. There was a correlation between severity of chondral pathology and high-grade synovial hyperplasia. Pre-operative KOOS correlated with BMI, meniscal degenerative changes and symptom duration. Obesity, time to surgery, presence of high-grade synovial hyperplasia and high-grade cartilage lesions were independent predictors of worse post-operative pain and function., Conclusion: We demonstrated that pre-operative symptoms and post-operative outcomes correlate with synovitis severity and cartilage pathology, particularly in old and obese patients that underwent arthroscopic partial meniscectomy. Importantly, patients with a degenerative meniscal pattern and with longer time to surgery experienced more severe cartilage damage and, consequentially, pain and dysfunction. These findings are fundamental to identify patients suitable for earlier interventions., Competing Interests: The authors declare no conflict of interest.

(1) Background: The Pediatric Outcomes Data Collection Instrument (PODCI) is an English-language questionnaire specifically designed to assess health-related quality of life in children and adolescents with musculoskeletal disorders. This scoring system has been translated into several languages. Given the lack of an Italian version of the PODCI, this study aimed to translate, cross-culturally adapt, and assess the psychometric properties of the PODCI score in the Italian pediatric population. (2) Methods: The PODCI questionnaire was culturally adapted to Italian patients in accordance with the literature guidelines. The study included 59 participants from a single orthopedic institution who underwent orthopedic surgery for various skeletal conditions. The questionnaire was administered to participants at multiple time-points (T0, T1, T2). Internal consistency was evaluated using Cronbach's alpha. Reproducibility was assessed using the intraclass correlation coefficient (ICC) between T0 and T1 assessment. Criterion validity was assessed using Spearman's correlation coefficients between PODCI and the Hospital for Special Surgery Pediatric Functional Activity Brief Scale (HSS Pedi-FABS). Responsiveness was evaluated by the difference between T0 and T2 using the effect size (ES) and the standardized response mean (SRM) calculation. (3) Results: Cronbach's alpha was acceptable in both the self- and parent-reported versions with values of 0.78 (0.68-0.90) and 0.84 (0.60-0.92), respectively. The ICC fluctuated between 0.31 and 0.89 for self-reported and 0.49 to 0.87 for pediatrics. The Spearman's r showed a moderate correlation between HSS Pedi-FABS and the "Sport & Physical Functioning" and "Global Functioning" domains. ES and SRM varied from small to moderate across all the domains. (4) Conclusions: This study demonstrates that the Italian version of the PODCI, translated following the international standardized guidelines, is reliable, valid, and responsive in pediatric patients who underwent orthopedic surgery.

The menisci exert a prominent role in joint stabilization and in the distribution of mechanical loading. Meniscal damage is associated with increased risk of knee OA. The aim of this study was to characterize the synovial membrane and meniscal tissues in patients undergoing arthroscopic partial meniscectomy for meniscal tear and to evaluate association with clinical outcomes. A total of 109 patients were recruited. Demographic and clinical data were collected. Visual Analogic Scale (VAS) measuring pain and Knee injury and Osteoarthritis Outcome Score (KOOS) were recorded at baseline and at 2-years follow-up. Histological and immunohistochemical characterizations were performed on synovial membranes and meniscal tissues. More than half of the patients demonstrated synovial mononuclear cell infiltration and hyperplasia. Synovial fibrosis was present in most of the patients; marked vascularity and CD68 positivity were observed. Inflammation had an impact on both pain and knee symptoms. Patients with synovial inflammation had higher values of pre-operative VAS and inflammation. Higher pre-operative pain was observed in patients with meniscal MMP-13 production. In conclusion, multivariate analysis showed that synovial inflammation was associated with pre-operative total KOOS scores, knee symptoms, and pain. Moreover, meniscal MMP-13 expression was found to be associated with pre-operative pain in multivariate analysis. Thus, targeting inflammation of the synovial membrane and meniscus might reduce clinical symptoms and dysfunction at the time of surgery.

(1) Background: Congenital pseudoarthrosis of the clavicle is a rare condition due to the failure of the union process of the ossification nuclei of the clavicle. The aim of this study was to conduct a systematic review of relevant case series about the argument to find an up-to-date base of evidence for treatment choice. (2) Methods: an electronic literature research of Ovid, MEDLINE and the Cochrane Library databases was conducted, and articles were selected based on inclusion criteria. Demographic data, clinical features, treatment options, outcomes and complications were analyzed. (3) Results: 21 articles met the inclusion criteria, showing a poor overall study quality; 231 pediatric patients (240 clavicles) were analyzed. The condition was typically right sided, showed no sex predominance and no clear predisposing factors. 156 patients underwent surgical treatment, mainly open debridement and refresh of bony ends, fixation with pin or plate and bone graft, with a successful union rate of 87.4%. The nonunion rate was significantly higher in the allograft group (44.4%, p = 0.019). (4) Conclusions: this paper presents an updated systematic review about treatment of congenital pseudoarthrosis of the clavicle. We confirm the generally satisfactory results of surgery, demonstrating that successful union is achievable in 87.4% of cases with a prevalence of 15.7% of major complications. Nonetheless our results should be interpreted with caution due to several limitations.

(1) Background: Sprengel's deformity (SD) is a rare congenital anomaly caused by failure in the descent of the scapula. We aimed to systematically review the current literature reporting data from children undergoing surgery for SD, in order to explore the rate of success and complications of the different surgical techniques, possibly providing recommendations about the management of SD in children. (2) Methods: we electronically searched the literature from Ovid, MEDLINE and the Cochrane Library databases. Demographic data, surgical procedures, outcomes and complications were analyzed. We categorized surgical procedures into five groups. (3) Results: 41 articles met the inclusion criteria, showing a poor overall study quality; 674 patients (711 shoulders) were analyzed. Green's and Woodward's procedures, both aiming the scapular relocation in a more anatomical position, were the most commonly used techniques. We counted 168 adverse events (18 major complications). The best clinical and cosmetic results seem to be achieved when surgery is performed in children aged less than eight years. (4) Conclusions: this paper represents the first systematic review reporting qualitative and quantitative data about the surgical treatment of SD. Surgery for SD seems to be effective in increasing the shoulder's range of motion and improving the cosmetic appearance in almost all cases, with a low rate of major complications.

Improvements in software for image analysis have enabled advances in both medical and engineering industries, including the use of medical analysis tools to recreate internal parts of the human body accurately. A research analysis found that FDM-sourced elements have shown viability for a customized and reliable approach in the orthopedics field. Three-dimensional printing has allowed enhanced accuracy of preoperative planning, leading to reduced surgery times, fewer unnecessary tissue perforations, and fewer healing complications. Furthermore, using custom tools chosen for each procedure has shown the best results. Bone correction-related surgeries require customized cutting guides for a greater outcome. This study aims to assess the biopolymer-based tools for surgical operations and their ability to sustain a regular heat-sterilization cycle without compromising the geometry and fit characteristics for a proper procedure. To achieve this, a DICOM and FDM methodology is proposed for fast prototyping of the cutting guide by means of 3D engineering. A sterilization test was performed on HTPLA, PLA, and nylon polymers. As a result, the unique characteristics within the regular autoclave sterilization process allowed regular supplied PLA to show there were no significant deformations, whilst annealed HTPLA proved this material's capability of sustaining repeated heat cycles due to its crystallization properties. Both of these proved that the sterilization procedures do not compromise the reliability of the part, nor the safety of the procedure. Therefore, prototypes made with a similar process as this proposal could be safely used in actual surgery practices, while nylon performed poorly because of its hygroscopic properties.

Congenital radioulnar synostosis (CRUS) is one of the most common congenital disorders affecting the elbow and forearm, with the forearm being fixed in a range of positions usually varying from neutral rotation to severe pronation. The aim of this study, apart from a systematic review of all surgical procedures described for CRUS, is to derive any correlation between various influencing factors, outcomes and complications. This review was performed in accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses format by an electronic literature search of Ovid, MEDLINE and the Cochrane Library databases. Grading was according to the Newcastle-Ottawa scale and the Modified Coleman Methodology Score. Demographic data, surgical procedures, outcomes and complications were analyzed. Outcome data were pooled to establish means and ranges across all studies. Spearman correlations were performed. A total of 23 articles, showing a poor overall study quality (all Level of Evidence IV), met the inclusion criteria. A total of 374 forearms with a mean age of 6.7 years (2.0-18.8) were analyzed. Derotational surgeries were more commonly performed (91%) than motion-preserving surgeries (9%). The mean deformity improved from 64.8° pronation (-75° to 110°) to a mean of 2.8° pronation (-50° to 80°). In total, 17.9% of patients presented with complications. A significant correlation was noted between age and major complications, proximal osteotomies and complications, and postoperative loss of reduction and double level osteotomies as the primary treatment modality. Most of the complications occurred above the threshold of 65-70° of correction and in children 7 years and above. Surgery is essential to improve the quality of life of children with CRUS. However, each type of surgery is associated with complications, along with the respective hardware being used in rotation osteotomies. Caution is, nevertheless, warranted in interpreting these results in view of the inherent limitations of the included studies., (Copyright © 2020 The Author(s). Published by Wolters Kluwer Health, Inc.)

Multiple osteochondromas (MO) is a rare disorder, characterized by benign osteocartilaginous tumors (osteochondromas), arising from the perichondrium of bones. The osteochondromas increase during growth, frequently causing deformities and limitations. Our study aims to analyze the data captured by the Registry of Multiple Osteochondromas, to refine Istituto Ortopedico Rizzoli (IOR) Classification, providing a representative picture of the phenotypic manifestations throughout the lifespan. We conducted a single-institution cross-sectional study. Patients were categorized according to IOR Classification, which identifies three patients' classes on the presence/absence of deformities and/or limitations. The present dataset was compared with our previously published data, to refine the classification. Nine hundred sixty-eight patients were included: 243 children (<10 years), 136 adolescents (10-15 years), and 589 adults. Of the entire population, half patients presented at least one deformity, and one quarter reported at least one limitation. Compared with our previous study, the amount of children was more than doubled and the percentage of mild/moderate cases was notably increased, giving a better disease overview throughout the lifespan and suggesting a different cut-off for dividing Class II in subclasses. We confirmed that MO is characterized by phenotypic heterogeneity, suggesting that an early classification of the disease may offer a useful tool to follow disease pattern and evolution, to support clinical practice, and to propose timely interventions., (© 2021 The Authors. American Journal of Medical Genetics Part A published by Wiley Periodicals LLC.)

Background : total hip replacement (THR) is a rare surgical option in children and adolescents with disabling hip diseases. The aim of this study is to report results from a retrospective cohort of patients aged 18 years or less who underwent cementless Ceramic-on-Ceramic (CoC) THR at a single institution, investigating clinical and radiographic outcomes, survival rates, and reasons for revision of the implants. Materials and methods : we queried the Registry of Prosthetic Orthopedic Implants (RIPO) to identify all children and adolescents undergoing THR between 2000 and 2019 at a single Institution. Inclusion criteria were patients undergoing cementless CoC THR, aged less than 18 years at surgery, followed for at least 2 years. Sixty-eight patients (74 hips) matched all the inclusion criteria and were enrolled in the study. We assessed the clinical and radiographic outcomes, the rate of complications, the survival rate, and reasons for revision of the implants. Results : The mean follow-up was 6.6 ± 4.4 years (range 2-20). The most frequent reason for THR was post-traumatic or chemotherapy-induced avascular necrosis (38%). The overall survival rate of the cohort was 97.6% (95% CI: 84.9-99.7%) at 5 years of follow-up, 94.4% (95% CI: 79.8-98.6%) at 10 years and 15 years of follow-up. Two THR in two patients (2.7%) required revision. With the numbers available, Cox regression analysis could not detect any significant interaction between preoperative or intraoperative variables and implant survivorship ( p -value 0.242 to 0.989)." The average HOOS was 85 ± 14.3 (range 30.6-100). Overall, 23 patients (48%) reported excellent HOOS scores (>90 points), 21 patients (44%) reported acceptable HOOS scores (60-90 points) while 4 patients (8%) reported poor outcomes (<60 points). Twenty-one patients (43%) were regularly involved into moderate- to high-intensity sport activities (UCLA ≥ 6). Conclusions : Cementless CoC THR is a successful procedure in children and teenagers, having demonstrated high implant survivorship and low rates of complications and failure. A meticulous preoperative planning and implant selection is mandatory, to avoid implant malposition, which is the main reason of failure and revision in these cases. Further studies are needed to assess the impact of the THR on the psychosocial wellbeing of teenagers, as well as risks and benefits and cost-effectiveness in comparison to the hip preserving surgical procedures.

Background: We aimed to investigate the variation of medical and surgical activities in pediatric orthopedics in Italy, during the year of the COVID-19 pandemic, in comparison with data from the previous two years. The differences among the first wave, phase 2 and second wave were also analyzed., Methods: We conducted a retrospective multicenter study regarding the clinical and surgical activities in pediatric orthopedics during the pandemic and pre-pandemic period. The hospital databases of seven tertiary referral centers for pediatric orthopedics and traumatology were queried for events regarding pediatric orthopedic patients from 1 March 2018 to 28 February 2021. Surgical procedures were classified according to the "SITOP Priority Panel". An additional classification in "high-priority" and "low-priority" surgery was also applied., Results: Overall, in 2020, we observed a significant drop in surgical volumes compared to the previous two years. The decrease was different across the different classes of priority, with "high-priority" surgery being less influenced. The decrease in emergency department visits was almost three-fold greater than the decrease in trauma surgery. During the second wave, a lower decline in surgical interventions and a noticeable resumption of "low-priority" surgery and outpatient visits were observed., Conclusion: Our study represents the first nationwide survey quantifying the impact of the COVID-19 pandemic on pediatric orthopedics and traumatology during the first and second wave.

Three-dimensional printed custom cutting guides (CCGs) are becoming more and more investigated in medical literature, as a patient-specific approach is often desired and very much needed in today's surgical practice. Three-dimensional printing applications and computer-aided surgical simulations (CASS) allow for meticulous preoperatory planning and substantial reductions of operating time and risk of human error. However, several limitations seem to slow the large-scale adoption of 3D printed CCGs. CAD designing and 3D printing skills are inevitably needed to develop workflow and address the study; therefore, hospitals are pushed to include third-party collaboration, from highly specialized medical centers to industrial engineering companies, thus increasing the time and cost of labor. The aim of this study was to move towards the feasibility of an in-house, low-cost CCG 3D printing methodology for pediatric orthopedic (PO) surgery. The prototype of a femoral cutting guide was developed for its application at the IOR-Rizzoli Orthopedic Institute of Bologna. The element was printed with an entry-level 3D printer with a high-temperature PLA fiber, whose thermomechanical properties can withstand common steam heat sterilization without bending or losing the original geometry. This methodology allowed for extensive preoperatory planning that would likewise reduce the overall surgery time, whilst reducing the risks related to the intervention.

Background: Multiple osteochondromas is a rare skeletal disorder characterized by the presence of osteocartilaginous protrusions causing bony deformities, especially around the knee. Guided growth by temporary hemiepiphyseal stapling is the treatment of choice to correct the deformity by modulating the residual physeal growth of the lower limbs. Although this procedure is increasingly practiced, inconclusive evidence exists regarding its effectiveness in children with multiple osteochondromas. The study aims to compare the outcomes of temporary hemiepiphyseal stapling for correcting genu valgum in children with multiple osteochondromas vs. idiopathic cases., Methods: In this retrospective cohort study, we included patients admitted at a single institution from 2008 to 2018. A total of 97 children (77 idiopathic, 20 multiple osteochondromas) were enclosed, accounting for 184 limbs treated by temporary hemiepiphyseal stapling. We investigated if children with multiple osteochondromas had a similar successful rate of correction, rate of complications, and correction velocity compared to children with idiopathic genu valgum., Results: Overall, 151 limbs (82%) achieved complete correction or overcorrection, with idiopathic cases having a significantly higher rate of success compared to pathologic cases (88% vs. 55%; p < 0.001). In addition, multiple osteochondromas children sustained a higher rate of major complications ( p = 0.021) and showed significantly lower correction velocity ( p = 0.029)., Conclusion: Temporary hemiepiphyseal stapling is effective in both idiopathic and multiple osteochondromas children, although the latter often achieved incomplete correction, had a higher risk of complications, and required a longer time of stapling. We suggest to anticipate the timing of intervention; otherwise, children with multiple osteochondromas and severe valgus deformity, approaching skeletal maturity, could undergo combined femoral and tibial stapling.

Osteochondrodysplasias are the result of the expression of gene mutations. The phenotypes in osteochondrodysplasias evolve through life, with the possibility that previously unaffected bones may be involved at later stages of growth. Due to the variable time of onset, the diagnosis may be made prenatally, at birth, or later. Certainty in the diagnosis is sometimes only achieved as the patient matures and the disease evolves. Radiographic evaluation is a fundamental part of the diagnostic work-up of congenital skeletal disorders and in most cases the first tool used to arrive at a diagnosis. This review describes the imaging characteristics, specific signs, and evolution of several skeletal dysplasias in which diagnosis may be directly or indirectly suggested by radiologic findings. A definitive accurate diagnosis of a congenital skeletal abnormality is necessary to help provide a prognosis of expected outcomes and to counsel parents and patients., Competing Interests: None declared., (Thieme. All rights reserved.)

The musculoskeletal (MSK) system begins to form in the third week of intrauterine development. Multiple genes are involved in the complex different processes to form the skeleton, muscles and joints. The embryonic period, from the third to the eighth week of development, is critical for normal development and therefore the time when most structural defects are induced. Many of these defects have a genetic origin, but environmental factors may also play a very important role. This review summarizes the embryology of the different components of the MSK system and their configuration as an organ-system, analyzes the clinical implications resulting from failures in the process of organogenesis, and describes the first approach to diagnosis of skeletal abnormalities using prenatal ultrasound., Competing Interests: None declared., (Thieme. All rights reserved.)

Background: Neglected fractures of the lateral humeral condyle (LHC) are misdiagnosed or insufficiently treated fractures, presenting later than 3 weeks after injury. The management of neglected LHC fractures in children remains controversial., Methods: Twenty-seven children were included in this retrospective study. Charts and medical records were investigated for demographics, time interval between injury and treatment, and type of treatment. Baseline radiographs were assessed for fracture grading and displacement. Final radiographs were investigated for bone healing, avascular necrosis, elbow deformities and growth disturbances. Complications were classified by the Clavien-Dindo-Sink (CDS) system. Outcomes were assessed according to the Dhillon Score (DhiS) and Mayo Elbow Performance Score (MEPS)., Results: The mean time from injury to presentation was 27 months. Treatments included nonoperative management (6 patients), "in-situ" fixation (7 patients), open reduction and internal fixation (11 patients) and corrective osteotomy (3 patients). The mean follow-up was 7 years (range: 2-16). Overall, we observed complications in 16 patients (59%); six complications were considered major (22%) and occurred in Weiss Grade 3 fractures, with lateral displacement ≥5 mm. At the latest follow-up, pain and functional scores improved in 23 of 27 patients (85%). Mean MEPS increased from an average of 62 points preoperatively to 98 points postoperatively, while mean DhiS improved on average from 5 to 8 points. CDS score and time interval between injury and treatment were independent predictors of MEPS and DhiS., Conclusion: Our study describes outcomes from a cohort of children undergoing different treatments for neglected LHC fractures. Prolonged time interval between injury and treatment and perioperative major complications negatively impacted the treatment outcomes. Our findings strengthen the requirement for widely agreed guidelines of surgical management in neglected LHC fractures.

Avulsion fracture of the tibial spine (TSA) is uncommon in children, although its incidence is increasing with the earlier practice of competitive sport activities. This study aims to report mid to long term outcomes in children who sustained a TSA, with a special focus on a return to sport activities. Skeletally immature patients with a TSA, treated in two orthopedic hospitals, were evaluated for range of motion and knee laxity using KT1000, KiRA and Rolimeter. The pediatric International Knee Documentation Committee score (Pedi-IKDC) and the Hospital for Special Surgery pediatric Functional Activity Brief Scale (Pedi-FABS) questionnaires were recorded during the latest visit. Forty-two children were included. Twenty-six were treated nonoperatively and 16 underwent surgery. At a mean follow-up of 6.9 ± 3.6 years, 36 patients completed the questionnaires and 23 patients were tested with arthrometers. Among them, 96% had normal knee laxity. The Pedi-IKDC score averaged 96.4 ± 5.7 points, while the mean Pedi-FABS was 22.2 ± 5.9 points, without statistically significant differences between groups. Twenty-eight patients (78%) returned to their previous level of sport activity (eight amateur, 13 competitive, seven elite athletes). Eight patients (22%) quit sport, mostly because of re-injury fear. If properly treated, pediatric TSAs achieve a high rate of successful healing, with complete restoration of knee stability and an early return to sport activities.

Purpose: The aim of the present study was to translate, cross-culturally adapt, and assess the psychometric properties of the Pedi-IKDC and Pedi-FABS scores in the Italian paediatric population with various knee pathologies., Methods: In accordance with the Paediatric Anterior Cruciate Ligament Monitoring Initiative (PAMI) research protocol, the original English versions of the questionnaires were translated into Italian. All patients aged 8-16 and scheduled for knee surgery were considered eligible in the study. An open-source platform was implemented to collect responses to the surveys which included general patient information, the questionnaires Pedi-IKDC, and Pedi-FABS. Two surveys were sent under stable clinical conditions before surgery (Q 1 and Q 2 ); a third survey was sent 3-4 months after surgery (Q 3 ). The following properties were calculated: reliability, internal consistency, criterion validity, responsiveness, and floor/ceiling effects., Results: Eighty-nine patients completed Q 1 , 81 patients completed Q 2 , and 49 patients completed Q 3 . Both questionnaires demonstrated acceptable properties. Pedi-IKDC: standard error of measurement (SEM) = 4.4, smallest detectable change (SDC) = 12.3, interclass correlation coefficient (ICC) = 0.96, Cronbach alpha (α) = 0.92, moderate-to-low correlation to Pedi-FABS, effect size (ES) = 0.79, standardized response mean (SRM) = 0.86, floor = 0%, ceiling = 22%. Pedi-FABS: SEM = 2.1, SDC = 5.8, ICC = 0.94, Cronback alpha (α) = 0.93, moderate-to-low correlation to Pedi-IKDK, ES = 0.60, SRM = 0.51, floor = 19%, ceiling = 0%., Conclusions: The Italian version of Pedi-IKDC and Pedi-FABS is valuable tools for patient assessment, by demonstrating good psychometric properties. In clinical setting, these questionnaires can be used to properly evaluate outcomes in Italian pediatric patients with knee pathologies., Level of Evidence: II.

Background: congenital posteromedial bowing of tibia (CPMBT) is a very rare birth defect, characterized by shortened bowed leg and ankle deformity. We described a single institution experience in the management of CPMBT., Methods: we identified 44 CPMBT in 44 children. The age at presentation was 5.5 ± 5.6 years and the mean age at the final review was 10.1 ± 4.8 years. Radiographic evaluation included the antero-posterior and lateral inter-physeal angle (AP-IPA and L-IPA), the limb length discrepancy (LLD), the morphology of the distal tibia and the lateral distal tibial angle (LDTA). During the study period, 26 children underwent surgical treatment., Results: the estimated curves showed a progressive spontaneous correction of both AP-IPA and L-IPA during growth, but a progressive increase of the LLD. The L-IPA showed a more predictable behaviour while the AP-IPA showed a scattered correction, with a wider variation of the estimated final angle. The final LDTA was 85.3° ± 4.2° and was correlated with the L-IPA (r = 0.5; p = 0.02). Among the 26 children who underwent surgical treatment, 23 cases had limb lengthening, 1 case had contralateral epiphysiodesis, 1 child underwent tibial osteotomy, 1 patient was treated by hemiepiphysiodesis of the distal tibia to correct ankle valgus deformity., Conclusions: our study described the largest case series of CPMBT. A combination of surgical treatments, in a staged surgical process, should be tailored to the developmental characteristics of this abnormality. An experience-based algorithm of treatment is also proposed. Further studies are needed to understand which is the best strategy to correct this deformity during childhood., Level of Evidence: level IV prognostic study.

Background: The management of painful rigid flatfoot (RFF) with talocalcaneal coalition (TCC) is controversial. We aimed to compare operative and nonoperative treatment in children with RFF and TCC., Methods: We retrospectively reviewed medical records and radiographs of children with RFF and TTC treated between 2005 and 2015. The nonoperative treatment consisted of manipulation under anesthesia, cast immobilization and shoe insert after cast removal. The operative treatment consisted of combined TCC resection, graft interposition and subtalar arthroereisis., Results: Thirty-four children (47 ft) in the nonoperative group and twenty-one children (34 ft) in the operative group were included. No differences were found between groups, concerning baseline characteristics. The mean age at treatment was 11.8 years (9-17): 11.6 (9-17) for the nonoperative group, 12.2 (10-15) for the operative group. The mean follow-up averaged 6.6 (3-12) years and was significantly longer in the nonoperative group (7.8 versus 4.7 years; p < 0.0005), since the operative procedure was increasingly practiced in the latest years. There were no complications in either groups, but 6 patients (7 ft) in the nonoperative group were unsatisfied and required surgery. At the latest follow-up, the AOFAS-AHS improved in both groups, although the operative group showed significantly better improvement. The operative group reported also significantly better FADI score, after adjustment for follow-up and baseline variables., Conclusion: The operative treatment showed better results compared to the nonoperative treatment. Symptomatic RFF with TCC in children can be effectively treated in one step with resection, graft interposition and subtalar arthroereisis. Further prospective randomized studies are needed to confirm our findings and to identify the best operative strategy in this condition.

Background: Hip osteoarthritis (HOA) is the most common hip disorder and a major cause of disability in the adult population, with an estimated prevalence of end-stage disease and total hip replacement. Thus, the diagnosis, prevention, and treatment of the early stages of the disease in young adults are crucial to reduce the incidence of end-stage HOA. The purpose of this study was to determine whether (1) a relationship among the inflammatory status of labrum and synovium collected from patients with femoroacetabular impingement (FAI) would exist; and (2) to investigate the associations among the histopathological features of joint tissues, the pre-operative symptoms and the post-operative outcomes after arthroscopic surgery., Methods: Joint tissues from 21 patients undergoing hip arthroscopy for FAI were collected and their histological and immunohistochemical features were correlated with clinical parameters., Results: Synovial mononuclear cell infiltration was observed in 25% of FAI patients, inversely correlated with the hip disability and osteoarthritis outcome score (HOOS) pain and function subscales and with the absolute and relative change in total HOOS. All the labral samples showed some pattern of degeneration and 67% of the samples showed calcium deposits. The total labral score was associated with increased CD68 positive cells in the synovium. The presence of labral calcifications, along with the chondral damage worsened the HOOS post-op symptoms (adjusted R-square = 0.76 p = 0.0001)., Conclusions: Our study reveals a relationship between the histologic labral features, the synovial inflammation, and the cartilage condition at the time of FAI. The presence of labral calcifications, along with the cartilage damage and the synovitis negatively affects the post-operative outcomes in patients with FAI.

According to previous research, natural polyamines exert a role in regulating cell committment and differentiation from stemness during skeletal development. In order to assess whether distinct polyamine patterns are associated with different skeletal cell types, primary cultures of stem cells, chondrocytes or osteoblasts were dedicated for HPLC analysis of intracellular polyamines. Spermine (SPM) and Spermidine (SPD) levels were higher in adipose derived stem cells (ASC) compared to mature skeletal cells, i.e. chondrocytes and osteoblasts, confirming the connection of polyamine content with stemness. To establish whether polyamines can protect ASC against oxidative DNA damage in a 3-D differentiation model, the level of γH2AX was measured by western blot, and found to correlate with age and BMI of patients. Addition of either polyamine to ASC was able to hinder DNA damage in the low micromolecular range, with marked reduction of γH2AX level at 10 µM SPM and 5 µM SPD. Molecular analysis of the mechanisms that might underlie the protective effect of polyamine supplementation evidences a possible involvement of autophagy. Altogether, these results support the idea that polyamines are able to manage both stem cell differentiation and cell oxidative damage, and therefore represent appealing tools for regenerative and cell based applications.

The aim of this study was to identify the molecules and pathways involved in the cross-talk between meniscus and synovium that may play a critical role in osteoarthritis (OA) pathophysiology. Samples of synovium and meniscus were collected from patients with early and end-stage OA and cultured alone or cocultured. Cytokines, chemokines, metalloproteases, and their inhibitors were evaluated at the gene and protein levels. The extracellular matrix (ECM) changes were also investigated. In early OA cultures, higher levels of interleukin-6 (IL-6) and IL-8 messenger RNA were expressed by synovium and meniscus in coculture compared with meniscus cultured alone. RANTES release was significantly increased when the two tissues were cocultured compared with meniscus cultured alone. Increased levels of matrix metalloproteinase-3 (MMP-3) and MMP-10 proteins, as well as increased release of glycosaminoglycans and aggrecan CS846 epitope, were observed when synovium was cocultured with meniscus. In end-stage OA cultures, increased levels of IL-8 and monocyte chemoattractant protein-1 (MCP-1) proteins were released in cocultures compared with cultures of meniscus alone. Chemokine (C-C motif) ligand 21 (CCL21) protein release was higher in meniscus cultured alone and in coculture compared with synovium cultured alone. Increased levels of MMP-3 and 10 proteins were observed when tissues were cocultured compared with meniscus cultured alone. Aggrecan CS846 epitope release was increased in cocultures compared with cultures of either tissue cultured alone. Our study showed the production of inflammatory molecules by synovium and meniscus which could trigger inflammatory signals in early OA patients, and induce ECM loss in the progressive and final stages of OA pathology., (© 2018 Wiley Periodicals, Inc.)

The treatment of a missed Monteggia (MM) fracture dislocation is still controversial. We describe our initial experience with ulnar osteotomy and progressive correction with unilateral external fixator in MM. We retrospectively evaluated 20 children undergoing ulnar osteotomy and progressive distraction angulation by unilateral external fixator to treat MM. Nine patients had closed reduction, whereas 11 patients had simultaneous open reduction, repair, or reconstruction of the annular ligament and K-wire stabilization of the radiocapitellar joint. Patients were followed for an average of 3 (1-11) years. Three children developed aseptic nonunion and one child had delayed union. A distal level of the osteotomy significantly increased the rate of nonunion or delayed union. At the final follow-up, eight children had complete reduction of the radial head, six children had partial reduction, whereas in six cases, the radial head remained dislocated. The angulation and the level of the osteotomy significantly influenced the relocation, whereas the open reduction had no significant effect on the final position of the radial head. At the final follow-up, the Kim's score averaged 93.25. The flexion-extension arc significantly improved postoperatively, and it was positively correlated with the angulation. The ulnar osteotomy and progressive traction-angulation by unilateral external fixator can achieve satisfactory results in MM, if a meticulous surgical technique is applied; care must be taken regarding the level of osteotomy and the progressive traction-angulation.

Aim: Osteoarthritis (OA) is a whole joint pathology involving cartilage, synovial membrane, meniscus, subchondral bone, and infrapatellar fat pad (IFP). Synovitis has been widely documented in OA suggesting its important role in pathogenesis. The aim of this study was to investigate the role of different joint tissues in promoting synovitis., Materials and Methods: Conditioned media (CM) from cartilage, synovial membrane, meniscus, and IFP were generated from tissues of five patients undergoing total knee replacement and used to stimulate a human fibroblast-like synoviocytes cell line (K4IM). Cytokines, chemokines, and metalloproteases release was analyzed in all CM by Bio-Plex Assay and sulfated glycosaminoglycan (GAG) content by dimethylmethylene blue assay. Gene expression of several markers was evaluated by real-time PCR in K4IM cells stimulated with the CM obtained from joint tissues., Results: CM from all tissues produced high levels of IL-6, IL-8, and CCL2. CCL21, MMP-3, and -13 levels were detected in all CM except IFP. MMP-10 was present only in CM of cartilage and synovial tissues. IL-1β, IL-15, TNF-α, CCL5, and CCL19 were undetectable. However, only K4IM cells stimulated by the CM from OA synovium showed an increase of IL-6, CXCL-8, CCL21, MMP10, and IL-1β expression., Conclusion: Our study showed that K4IM might be a suitable in vitro model for evaluating different cellular pathways in OA studies. Importantly, we demonstrated that in OA, all joint tissues might be involved in the progression of synovitis with a predominant role of synovial membrane itself compared to the other joint tissues.

The human meniscus plays a crucial role for transmission and distribution of load across the knee, as well as shock absorption, joint stability, lubrication, and congruity. The aim of this study was to compare the complex geometry, and unique ultrastructure and tissue composition of the meniscus in healthy (control) and pathological conditions to provide understanding of structural changes that could be helpful in the future design of targetted therapies and improvement of treatment indications. We analyzed meniscus samples collected from 3 healthy multi-organ donors (median age, 66 years), 5 patients with traumatic meniscal tear (median age, 41 years) and 3 patients undergoing total knee replacement (TKR) for end-stage osteoarthritis (OA) (median age, 72 years). We evaluated the extracellular matrix (ECM) organization, the appearance and distribution of areas of calcification, and modifications of cellular organization and structure by electron microscopy and histology. The ECM structure was similar in specimens from traumatic meniscus tears compared to those from patients with late-stage OA, showing disorganization of collagen fibers and increased proteoglycan content. Cells of healthy menisci showed mainly diffuse chromatin and well preserved organelles. Both in traumatic and in OA menisci, we observed increased chromatin condensation, organelle degeneration, and cytoplasmic vacuolization, a portion of which contained markers of autophagic vacuoles. Areas of calcification were also observed in both traumatic and OA menisci, as well as apoptotic-like features that were particularly prominent in traumatic meniscal tear samples. We conclude that meniscal tissue from patients with traumatic meniscal injury demonstrate pathological alterations characteristic of tissue from older patients undergoing TKR, suggesting that they have high susceptibility to develop OA.

The aim of this study was to analyze the magnetic resonance imaging (MRI) volumetric and morphometric characteristics of the infrapatellar fat pad (IFP) and the suprapatellar fat pad (SFP) in normal controls, moderate and end-stage osteoarthritis (OA) patients. Forty-four MRI images of the three groups were collected: a) 17 patients undergoing meniscectomy with Outerbridge score 0 (control group); b) 15 patients undergoing meniscectomy with Outerbridge score 3/4 (moderate OA group); and c) 12 patients undergoing total knee replacement (end-stage OA group). Volume, depth, femoral and tibial arch lengths of IFP were quantified. The hypointense IFP signals were also scored. The SFP volume, oblique, antero-posterior and cranio-caudal lengths were determined. IFP and SFP characteristics were compared between groups. A decrease of IFP volume, depth, femoral, and tibial arch lengths in moderate and end-stage OA compared to controls were observed. A difference in IFP hypointense signal was found between groups. No differences were found in SFP characteristics between the groups. In controls and moderate OA patients, correlations were found among the different MRI characteristics of both IFP and SFP, while in the end-stage OA group correlations were found only in SFP. We evidenced differences of the IFP MRI morphometric characteristics between the groups analyzed, supporting an important role of IFP in OA pathology and progression. On the contrary, no differences were highlighted in SFP analysis suggesting that this fat pad is not clearly involved in OA, probably due to its peculiar localization and different function., (Copyright © 2018 Elsevier GmbH. All rights reserved.)