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Unlabelled: Leg lengthening by external fixation is associated with various difficulties. We evaluated eight adolescent patients who underwent leg lengthening with a motorized intramedullary lengthening device. We asked whether this method could reduce the time of hospitalization and rehabilitation and whether the incidence of complications commonly associated with external fixators could be reduced. We compared our preliminary results with those from other reports, with a focus on leg length achieved, time of rehabilitation, and rate of complications. The average leg-length discrepancy was 3.8 cm (range, 3-5 cm). The average lengthening distance was 3.8 cm (range, 2.9-4.7 cm). In six patients, leg lengthening was combined with successful correction of the mechanical axis alignment. The consolidation index averaged 26 days/cm (range, 19-41 days/cm). The average hospital stay was 9.6 days. No bone or soft tissue infections were observed. In comparison to other studies (1.0-2.8 complications/patient), our results suggest that the difficulties commonly associated with external fixators can be reduced with this method. It also allows good angular correction in patients with mechanical axis deviation. These features combined with a short time of hospitalization and rehabilitation make it a promising procedure for limb lengthening. Level Of Evidence: Level IV Therapeutic study. [ABSTRACT FROM AUTHOR]

Background: Electronic patient-reported outcome measures (ePROMs) are useful tools to assess care needs of patients diagnosed with cancer and to monitor their symptoms along the illness trajectory. Studies regarding the application of ePROMs by advanced practice nurses (APNs) specialized in sarcoma care and the use of such electronic measures for care planning and assessing quality of care are lacking., Objective: To explore the potential of ePROMs in clinical practice for assessing the patient's quality of life, physical functionality, needs, and fear of progression, as well as distress and the quality of care in sarcoma centers., Methods: A multicenter longitudinal pilot study design was chosen. Three sarcoma centers with and without APN service located in Switzerland were included. The instruments EQ-5D-5L, Pearman Mayo Survey of Needs, the National Comprehensive Cancer Network Distress Thermometer, PA-F12, and Toronto Extremity Salvage Score were used as ePROMs. Data were analyzed descriptively., Results: Overall, 55 patients participated in the pilot study; 33 (60%) received an intervention by an APN, and 22 (40%) did not. Patients in sarcoma centers with APN service reported overall higher scores in quality of life and functional outcome. The number of needs and distress level were lower in sarcoma centers with APN service. No differences were found with respect to patients' fear of progression., Conclusions: Most of the ePROMs proved to be reasonable in clinical practice. PA-F12 has shown low clinical relevance., Implications for Practice: Using ePROMs appears to be reasonable to obtain clinically relevant patient information and to evaluate the quality of care in sarcoma centers., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Synovial sarcoma is a rare and highly malignant soft tissue sarcoma. The inconspicuous and diversity of its early symptoms make it a highly misdiagnosed disease. The management of synovial sarcomas is challenging as they are rare and have a poor prognosis. Early and correct diagnosis and treatment are critical for clinical outcomes. Misdiagnosis or delayed diagnosis can have devastating consequences for the patient. The detection of SS18 gene rearrangement is considered a powerful tool in establishing the diagnosis of synovial sarcomas. Biopsies and testing for gene rearrangements are recommended for all patients in whom SS cannot be excluded. Surgery is the mainstay of treatment for synovial sarcomas. Neoadjuvant/adjuvant radiotherapy is recommended for patients with big tumors (>5 cm) or positive resection margins, and neoadjuvant/adjuvant chemotherapy is recommended for patients with high-risk tumors or advanced diseases. This article reviews synovial sarcomas from the perspectives of clinical and radiological presentation, histological and cytogenetic analysis, differential diagnosis, treatment, and prognosis.

Background: Congenital pseudarthrosis of the tibia is a limb deformity, which can be distressing for the affected patients and the pediatric orthopedic surgeons involved. We hypothesized that the modified McFarland procedure would avoid fractures and even have a corrective effect on the affected tibia in congenital pseudarthrosis of the tibia patients. Toward this end, we evaluated the midterm results of treating congenital pseudarthrosis of the tibia patients of Crawford Type I and II with allograft bypass combined with long-term bracing., Methods: This study retrospectively evaluated 7 patients with congenital pseudarthrosis of the tibia who were treated with allograft bypass combined with long-term bracing between 2009 and 2018. The median follow-up was 7.0 years (range 3.8-10.0 years). The medical records and radiographs were reviewed for demographic data, clinical characteristics, outcomes, and complications., Results: At the time of the last follow-up, all allografts revealed complete consolidation in the patients' tibiae at both ends. All patients presented no functional restriction of the lower limbs and no amputation or non-union has occurred. Most of the obvious deformities of the tibia diaphysis or ankle joint were corrected. Two complications occurred that required successful revision surgery., Conclusion: In this series of seven congenital pseudarthrosis of the tibia patients, the allograft bypass technique showed satisfactory midterm results and validated our hypothesis. For congenital pseudarthrosis of the tibia patients of Crawford Type I and II, this procedure combined with long-term bracing, which involves the affected leg only, can delay or possibly prevent fractures, decrease tibial malalignment, and preserve leg length., Level of Evidence: level IV., Competing Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article., (© The Author(s) 2024.)

Prevention of rotatory impairment and radial head dislocation in the forearm is an important aspect when treating children with osteochondromas. Various studies tried to determine the best treatment, describing different surgical techniques. No consensus has been reached yet. This retrospective study compares the treatment outcome of patients with osteochondroma of the radius and ulna after surgical or conservative treatment. Seventeen forearms treated over a period of 20 years were analysed. Outcome parameters were the prospectively collected clinical data and the radiological findings: "relative shortening" of ulna/radius, the "radial articular angle" (RAA) and the "carpal slip" (CS). Our study shows an improvement of the range of motion and cosmetic appearance of the forearm after an operative procedure, with or without bone lengthening. We observed an increase in wrist and elbow mobility with a decrease in pain scores and a confirmed high cosmetic satisfaction in almost 70% of the patients after bone lengthening and up to 85% after simple excision. For patients suffering from functional impairment or pain, an operative approach is beneficial. Multiple and repetitive osteochondroma excisions are recommended during growth to prevent deformity and rotatory motion restriction. Lengthening procedures require a careful indication.

Background and Purpose: The retrograde femoral approach is an established technique for implantation of nails for leg lengthening and correction and in cases of distal femoral fractures. The purpose of this study was to determine the 10-year outcome of this technique by analyzing the clinical long-term effects and radiological status of the knee after leg lengthening via a retrograde femoral approach., Patients and Methods: This retrospective single-center study included 13 patients (median age at surgery 17 [range 15-20] years) who underwent unilateral, retrograde, femoral lengthening with a motorized nail. Outcome measurements were graded variables of the SF-36, ISKD score, and Lysholm score. MRI of both knees was performed in all patients. MRI was evaluated for the presence of degenerative changes and compared with the healthy contralateral knee. Cartilage condition was graded according to the International Cartilage Repair Society (ICRS) scoring system., Results: All patients were pain-free and had a full range of motion 10 (range 10.0-12.2) years after surgery. All postoperative knees showed fibrosis of Hoffa's fat pad and moderate to severe cartilage defects (ICRS Grade 2-4) of the trochlear groove (nail entry site). 6 out of 13 operated knees exhibited retropatellar cartilage defects., Conclusion: Our study showed that patients were pain-free, but cartilage defects at the entry point and arthrofibrosis at Hoffa's fat pad were observed without causing clinical impairment.

Bone sarcomas of the lower extremities are rare malignancies occurring mostly amongst adolescents and young adults. Necessarily, the therapy conducted in sarcoma centers is multimodal and multidisciplinary. In certain cases, in a metastasis free situation with adequate therapy, an overall survival rate of 90% can be achieved. Two principal surgical procedures exit for the local control of the malignancy: 1. Limb salvage with biological with/or endoprosthetic reconstruction; and, 2. amputation with restoration of the function with exoprosthesis or endo-exoprosthesis. Currently, limb salvage procedures are performed in up to 95% of cases. In contrast, amputation is performed when the disease has reached an advanced stage or limb salvage has failed. Both of the surgical options have their risks and possible complications. According to the literature, there should be no significant difference between limb salvage and amputation with respect to long-term overall survival, overall quality of life, psycho-socio-economic outcomes, or patient satisfaction. An important advantage of limb salvage is greater everyday functionality. With the expanded indication of limb salvage and great survival rates, the cases of late complications in patients expecting to maintain their own leg continues to increase. In some cases, it requires multiple interventions, ranging from minor up to the most complex revisions, to maintain the functionality of the extremity. Despite the great costs, personal effort, and the possible complications, limb salvage could be a suitable method to achieve functionally beneficial outcomes and patient satisfaction in bone sarcomas of the lower extremities over the long-term even in cases involving complications., Competing Interests: Declaration of competing interest The authors declare no conflict of interest., (Copyright © 2022 Elsevier Ltd. All rights reserved.)

Introduction: Different factors can lead to inconsistencies in measurement for the acetabular version using 2D axial CT-cuts. We have defined a "true" anteversion angle (AV angle) in the physiological position of the pelvis in 3D with the largest European population measured to our knowledge., Material and Methods: We analyzed 258 hemipelvises and created 3D models. We compared the results of our AV angle 3D method with the cross-sectional cuts of the same acetabula. We included factors like side, sex, body mass index, and patient positioning., Results: Overall, the mean (SD) AV angle was 16.1 (5.9)° as measured with the 3D method and 22.0 (6.0)° as measured with the 2D method (p < 0.0001). Measured with both the 3D and the 2D method, the AV angle was significantly larger in female than in male individuals (p < 0.0001). In the 2D method, the AV angle estimation was influenced by the pelvic tilt., Conclusion: We propose a more accurate method for the measurement of the AV angle of the acetabulum in a 3D model that is not influenced by patient positioning or pelvic tilt. We provide a computational model that will facilitate operative decisions and improve preoperative planning. We confirm that 3D measurement should be the gold standard in measuring the acetabular anteversion., (© 2022. The Author(s).)

Background/aim: Vascularized (VFG) and non-vascularized fibula grafts (NVFG) are used in reconstruction of bone defects after tumour resection. This study compared both autografts and their results, risk factors, and complications., Patients and Methods: Tumour resection and reconstruction by using VFG (n=17) and NVFG (n=36) were performed in 53 patients at our institute (range=3-65 years of age, mean: 21.2 ± 13.2 years) of which 24 were female. Malignant tumours were diagnosed in 26 patients (VFG=16 patients-94%). The mean follow-up was 14.9 years (range=1.5-43 years). Factors like consolidation, functional and oncologic outcomes, and complications were analysed., Results: In total, 75 struts of fibula were obtained. The mean length of the fibula was 16.3 cm (16 in NVFG and 16.5 in VFG). The mean union time was 13 months (6 to 25 months) overall. Hypertrophy was found in 65 of 75 grafts (86.7%) and consolidation was found in 69 (92%). Hypertrophy was similar in VFG (85.3%) and NVFG (87.1%). Complication rate in VFG was 41% and in NVFG 25%. Fractures were found in 7 (13%), infections in 4 (7.5%), and non-union in 5 (9.4%) patients. Chemotherapy was the only negative prognostic factor for union time (p=0.021)., Conclusion: Both VFG and NVFG are used with successful results in the reconstruction of segmental bone tumour defects. With lower complication rates, NVFG showed comparable results to VFG but is limited in indication by size for greater defects, and malignant tumours. Chemotherapy is an adverse factor leading to prolonged union time in both techniques., (Copyright © 2022 International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Rearrangements of the transcription factors FOS and FOSB have recently been identified as the genetic driver event underlying osteoid osteoma and osteoblastoma. Nuclear overexpression of FOS and FOSB have since then emerged as a reliable surrogate marker despite limitations in specificity and sensitivity. Indeed, osteosarcoma can infrequently show nuclear FOS expression and a small fraction of osteoblastomas seem to arise independent of FOS/FOSB rearrangements. Acid decalcification and tissue preservation are additional factors that can negatively influence immunohistochemical testing and make diagnostic decision-making challenging in individual cases. Particularly aggressive appearing osteoblastomas, also referred to as epithelioid osteoblastomas, and osteoblastoma-like osteosarcoma can be difficult to distinguish, underlining the need for additional markers to support the diagnosis. Methylation and copy number profiling, a technique well established for the classification of brain tumors, might fill this gap. Here, we set out to comprehensively characterize a series of 77 osteoblastomas by immunohistochemistry, fluorescence in-situ hybridization as well as copy number and methylation profiling and compared our findings to histologic mimics. Our results show that osteoblastomas are uniformly characterized by flat copy number profiles that can add certainty in reaching the correct diagnosis. The methylation cluster formed by osteoblastomas, however, so far lacks specificity and can be misleading in individual cases., (© 2022. The Author(s).)

Introduction: Customized accurate tumor resection and individualized reconstruction is a challenging in treatment of malignant bone tumor. Three-dimensional (3D)-printing technique is now widely used in the resection and following reconstruction of malignant bone tumor, which included but not limited to tumor model, osteotomy guide and customized implant., Methods: We retrospectively reviewed 17 patients, who underwent limb salvage surgery by using 3D-printed guide at a single center between August 2014 and October 2019. The median duration of follow-up was 26.5 months. Osteosarcoma (41.2%) were the predominant diagnoses. The functional outcomes were assessed by Musculoskeletal Tumor Society (MSTS) functional score. We also analyzed survival status, intraoperative data (blood loss, operation time and resection length), reconstruction method, margin outcomes and complications., Results: We totally performed 93 guided osteotomies on affected bone and allograft bone in 17 patients. Reconstruction in 12 cases was performed with biological technique: allograft combined with autograft was used in 7 cases. 11 of 12 (91.7%) cases showed a good bone healing in both allograft and autograft. 1 of 12 (8.3%) cases had allograft necrosis. Additional intra-operative extracorporeal radiation was performed in 3 pelvic cases for reconstruction. 63 of 64 (98%) osteotomies achieved wide resection and negative margin. All the cases had successful limb salvage result without amputation. At the latest follow up, the mean MSTS Score was 24 (range: 13-30), 12 patients alive with no evidence of disease, 1 patient alive with disease, 5 patients had died of disease and 5 years overall survival is 73.3%. The most common complications are wound healing disorder in 4 cases (23.5%) and infection in 3 cases (17.6%)., Conclusion: The 3D-printed resection guide was easy to use and showed promise in the field of orthopedic oncology. It can not only used in primary malignant bone tumor personalized resection but also in shaping structural bone allograft in biological reconstruction, which can achieve a safety surgical margin and individualized resection at the same time., (Copyright © 2022 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background/aim: Limb-sparing procedures are frequently applied to improve patient outcomes. The use of vascularized bone grafts is associated with significant improvements in oncologic safety and functional satisfaction. This study highlights the clinical outcomes following tumor resection combined with vascularized bone graft reconstructions., Patients and Methods: Twenty-five free vascularized bone grafts (17 fibulas, 5 iliac crests, 3 medial femoral condyles) were assessed with respect to consolidation and hypertrophy, functional and oncologic outcomes, and local complications., Results: The rate of healing of fibular grafts after a median of 5 months was 86%. The rate of achieved unions of iliac crest grafts after a median of 5 months was 80%. In medial femoral condyle bone grafts, union occurred after a median of 4 months. Significant hypertrophy was observed in 13 patients. We identified six complications with highest rates in the fibula-group. Despite the high complications, functional results were highly satisfactory., Conclusion: Vascularized bone grafts represent a reconstructive approach, maintaining long-term functionality and cosmetic satisfaction without compromising tumor recurrence outcomes., (Copyright © 2021 International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Purpose: Axial malignant bone tumours are rare in children and adolescents, and their prognosis is still relatively poor due to non-specific symptoms, such as back or groin pain, which may result in late hospital presentation. Therefore, it is very important to raise awareness regarding this pathology., Methods: We performed a narrative review, including scientific publications published in English. We searched Medline and Google Scholar databases for information on the incidence and prognosis of axial malignant bone tumours in children and adolescents (< 18 years). Outcomes of different surgical management strategies and reconstruction options were assessed., Results: The incidence of primary malignant bone tumours before the age of 18 years is approximately five per one million population; around 25% of these tumours are located in the axial skeleton. With a five-year survival rate of 50%, tumours in an axial location (chest cage, spine, pelvis) are associated with a poorer prognosis than tumours in more peripheral locations. En bloc excision with clear margins has been shown to improve local control and overall survival, even though obtaining adequate surgical margins is difficult due to the close location of large neurovascular structures and other major organs. Spinal reconstruction options include instrumented fusion with allograft or expandable cage. Pelvic reconstruction is needed in internal hemipelvectomy, and the options include biological, endoprosthetic reconstructions, hip transposition, arthrodesis or creation of pseudoarthrosis and lumbopelvic instrumentation., Conclusion: Early diagnosis, a timely adequate multidisciplinary management, appropriate en bloc excision, and reconstruction improve survival and quality of life in these patients., Level of Evidence: V., (Copyright © 2021, The author(s).)

An 8-year-old girl developed severe acute lung injury after irrigation of a pelvic aneurysmal bone cyst with H2O2 and filling with bone cement. Sudden profound oxygen desaturation occurred on the operating table when the patient was turned from the prone to the supine position. After a brief improvement in her oxygenation, the girl developed rapidly progressing severe respiratory failure necessitating reintubation and hour-long manual ventilation, while copious amounts of hemorrhagic frothy fluids were aspirated through the endotracheal tube. The patient started to improve after 24 hours and eventually made a full recovery. We hypothesize that the incident was caused by gas embolization and pulmonary endothelial damage by H2O2., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2021 International Anesthesia Research Society.)

Osteosarcoma is the most common primary malignant bone tumour in children and adolescents. More than a third of patients do not respond to standard therapy and urgently require alternative treatment options. Due to a high degree of inter- and intra-tumoural genomic heterogeneity and complexity, recurrent molecular alterations that could serve as prognostic predictors or therapeutic targets are still lacking in osteosarcoma. Copy number (CN) gains involving the IGF1R gene, however, have been suggested as a potential surrogate marker for treating a subset of patients with IGF1R inhibitors. In this study, we screened a large set of osteosarcomas and found specific CN gains of the IGF1R gene in 18 of 253 (7.1%) cases with corresponding IGF1R overexpression. Despite the discouraging results observed in clinical trials in other tumours so far, focusing only on selected patients with osteosarcoma that show evidence of IGF pathway activation might represent a promising new and innovative treatment approach., (© 2020 The Authors. The Journal of Pathology: Clinical Research published by The Pathological Society of Great Britain and Ireland & John Wiley & Sons, Ltd.)

Background: Acute hematogenous osteomyelitis (OM) and septic arthritis require immediate diagnosis and treatment by an interdisciplinary team of pediatric infectious disease specialists and pediatric orthopedic surgeons. Adverse outcomes such as growth disturbance, bone deformity, and chronic infections have been described in older studies. However, there is only little known about long-term follow-up of patients of the last two decades. Therefore, we aimed to evaluate subjective and objective long-term outcomes of these children with osteoarticular infections treated in the millennial years. Methods: Cross-sectional study performed in two pediatric centers including patients admitted for OM and/or SA between 2005 and 2014 and follow-up consultations in 2019. Patients with symptoms of ≤2 weeks duration at initial presentation were contacted. Subjective outcomes were assessed by standardized interview, objective outcomes by clinical examination. Medical charts were used to extract data from the initial presentations. Statistical analysis was performed by non-parametric tests and Fisher's exact test. Results: Of 147 eligible patients 77 (52%) agreed to participate, of which 68 (88%) had an interview and physical examination and 9 (12%) an interview only. Thirty-three (39%) had OM, 26 (34%) SA, and 21 (27%) combined OM/SA. Median (IQR) age at follow-up was 13.3 (10.5-18.0) years with a median (IQR) follow-up of 7.1 (6.1-8.6) years. Persistent complaints including pain, functional differences and scar paresthesia, reported by 21 (28%) patients, were generally mild and only 3 (5%) required ongoing medical care. Objective sequelae including pain, limited range of motion, unilateral axis deformity or asymmetric gait were found in 8 (12%) participants. Older age, female sex, joint involvement, surgical intervention, persistent fever, and C-reactive protein elevation were associated with adverse clinical outcome. Conclusions: Adverse outcomes were observed in a considerable number of patients, most of which were minor, and only few required ongoing medical care. Long-term follow up is advisable for patients with risk factors identified during the initial presentation. This study was registered on ClinicalTrials.gov (NCT03827980)., (Copyright © 2020 Manz, Krieg, Buettcher, Ritz and Heininger.)

Purpose: To establish simple quantitative variables at short-tau inversion recovery (STIR) magnetic resonance imaging (MRI) to identify lipomas with high specificity in patients with indeterminate subfascial lipomatous tumors., Materials and Methods: The MRI examinations of 26 patients (14 men, 12 women; mean age 63±12.5 [SD] years; range: 40-84years) with histopathologically proven subfascial atypical lipomatous tumors/well-differentiated liposarcomas (ALT/WDLs) and those of 68 patients (32 men, 36 women; mean age, 56±13.5 [SD] years; range: 21-83years) with lipomas were retrospectively reviewed. Ratios derived from region of interest based signal intensity (SI) measurements of tumors and adjacent fat on STIR images were calculated and maximum tumor diameters were noted. Diagnostic parameter capabilities were assessed using ROC curve analysis. Interreader agreement was evaluated by calculation of intraclass correlation coefficients (ICC)., Results: Using a cut-off value of 1.18, STIR-SI ratios allowed discriminating between lipoma and ALT/WDL (AUC=0.88; P<0.001) yielding 93% specificity (95% CI: 77-99%) and 74% sensitivity (95% CI: 61-84%) for the diagnosis of lipoma. Interreader agreement was excellent (ICC=0.93). A significant difference in maximum tumor diameter was found between ALT/WDLs (mean: 18.1±6.0 [SD] cm; range: 5.6-33.1cm) and lipomas (mean: 9.7±5.0 [SD] cm; range: 2.9-29.1cm) (P<0.001). Using a cut-off of 11cm, maximum tumor diameter allowed discriminating between lipoma and ALT/WDLs with 92% specificity (95% CI: 75-99%) and 69% sensitivity (95% CI: 57-80%). The combination of a STIR-SI ratio<1.4 and maximum tumor diameter<11cm yielded 100% specificity (95% CI: 87-100%) and 65% sensitivity (95% CI: 54-77%) for the diagnosis of lipoma., Conclusion: The combination of STIR-SI ratio and maximum diameter allows discriminating between lipoma and ALT/WDL in initially indeterminate lipomatous tumors., (Copyright © 2020 Société française de radiologie. Published by Elsevier Masson SAS. All rights reserved.)

Introduction: Simple bone cysts (SBCs) are common in children and adolescents. The risk of refracture and the probability of spontaneous healing in SBCs are mainly dependent on the activity of the cyst and can be quantified with the Cyst-Index. Avoiding pathological fractures is the primary goal. Our study presents a comparison of two different bioresorbable bone graft substitutes (BGSs) in the minimally-invasive treatment of SBC in the active stage by percutaneous cyst aspiration and injection., Methods: Between 2006 and 2017, 38 patients (aged two to 37 years; mean age 12.4 (sd 5.6)) were treated with percutaneous cyst aspiration and refilled with bioresorbable BGSs in three hospitals. The cysts of 21 patients (11 humerus, five femur, four calcaneus, one fibula) were refilled with porous beta-tricalcium phosphate (PB-TP group) (ChronOS Inject) and of 17 patients (nine humerus, six femur, one calcaneus, one fibula) with hydroxyapatite/calcium sulphate (H/CS group) (CERAMENT|BONE VOID FILLER). There were 13 (62%) preceding fractures in the PB-TP group and eight (47%) in the H/CS group. The follow-up of all patients was at least two years. During follow-up, radiological healing (modified Neer classification), activity level, refracture rates, recurrence rates, resorption period and complications were analyzed., Results: In all, 21 patients treated with PB-TP group experienced 27 operations (one refracture, three recurrences and one persistent cyst). A total of 17 patients treated with H/CS experienced 20 operations (one refracture and one recurrence). After six weeks, 95% in the PB-TP group returned to unrestricted activity with one refracture in the femur due to insufficient biomechanical stability and all returned to unrestricted activity in the H/CS group. Partial or complete radiological response was observed in 81% after 13 months (sd 3.4). Three recurrences (14%) occurred in the PB-TP group and one recurrence (6%) occurred in the H/CS group. The refracture rates were similar in both groups; one (5%) in the PB-TP group and one (6%) in the H/CS group. All H/CS treated cysts showed completed resorption after two years, whilst in PB-TP treated cysts no resorption occurred in five cases (25%) (p = 0.031). Two (10%) wound infections occurred in the PB-TP group and no infections occurred in the H/CS group., Conclusion: Both PB-TP and H/CS can provide stability and prevent refracture in patients with single bone cysts at the upper extremity or the foot. For the proximal femur, additional stabilization is necessary, due to the weight-bearing and associated high refracture rate. The H/CS bone graft substitute has a better resorption rate than the PB-TP graft., Level of Evidence: III., (Copyright © 2020, The author(s).)

Purpose: Extra-abdominal desmoid tumours are benign and rare, and lead to a persistent treatment dilemma because of their high recurrence rate and their heterogeneous behaviour. The goal of this retrospective study was to evaluate the results of different treatment modalities for extra-abdominal desmoid tumours at four sarcoma treatment centres., Methods: The mean follow-up time for the 96 patients included in the study (63.5% female; mean age 38.9 years) was 8.4 years (2.0&ndash;40.5 years). The initial treatments were surgery (n = 44), surgery with radiation (n = 16), watchful waiting (n = 15), radiation only (n = 9), or systemic treatment (n = 12). Patient demographics, tumour sites, and the follow-up status of all patients were reviewed and evaluated for each of the treatment modalities., Results: The local recurrence rate was 45.5% in patients with primary surgical treatment and 37.5% following surgery combined with irradiation. Patients who were treated with radiation alone showed regressive (33.3%) or stable disease (66.6%). Systemic treatment alone resulted in disease progression in 41.7% of our patients. In the watchful waiting group, 73.3% showed stable disease, 20.0% showed spontaneous regression, and 6.7% showed progression after a mean follow-up of 4.1 years (2.0&ndash;11.5 years)., Conclusions: Our results suggest that a watchful waiting approach should be the first line treatment in asymptomatic desmoid tumours. However, radiation can help improve local control rates in patients who have undergone surgery. Progression and local recurrence rates following systemic treatment were comparable to those observed in surgery combined with radiation.

Non-ossifying fibroma (NOF), which occasionally results in pathologic fracture, is considered the most common benign and self-limiting lesion of the growing skeleton. By DNA sequencing we have identified hotspot KRAS, FGFR1 and NF1 mutations in 48 of 59 patients (81.4%) with NOF, at allele frequencies ranging from 0.04 to 0.61. Our findings define NOF as a genetically driven neoplasm caused in most cases by activated MAP-kinase signalling. Interestingly, this driving force either diminishes over time or at least is not sufficient to prevent autonomous regression and resolution. Beyond its contribution to a better understanding of the molecular pathogenesis of NOF, this study adds another benign lesion to the spectrum of KRAS- and MAP-kinase signalling-driven tumours. Copyright © 2018 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd., (Copyright © 2018 Pathological Society of Great Britain and Ireland. Published by John Wiley & Sons, Ltd.)

Objective: To report on a CT scan virtual periacetabular osteotomy (PAO) process to evaluate the potential risk of different PAO cutting planes., Methods: A total of 123 patients (64 men and 59 women) underwent virtual PAO. We defined two retroacetabular cutting (RC) planes: the RC plane and the RC + plane (10 mm posteriorly as compared to the RC plane). Subsequently, we measured the anatomical minimum distance between the acetabulum and the sciatic notch, the minimum distance between the acetabulum and the retroacetabular cutting plane (osteotomy of the posterior column), and the osteotomy length in the cranio-caudal direction., Results: The mean (standard deviation [SD]) minimum distance between the acetabulum and the sciatic notch was 25.82 ± 3.52 mm (95% confidence intervals [CI], 25.36-26.25 mm). In men, the mean (SD) minimum distance between the acetabulum and sciatic notch (27.18 ± 3.47 mm; 95% CI, 26.56-27.78 mm) was significantly (3 mm) larger than in women (24.34 ± 2.92 mm; 95% CI, 23.82-24.89 mm; P < 0.001). The mean (SD) minimum distance between the acetabulum and the retroacetabular plane was significantly larger for the RC + plane (6.97 ± 0.91 mm) than for the RC plane (P < 0.001). In men, this distance (10.23 ± 3.84 mm) was significantly (2.3 mm) larger than in women (7.94 ± 3.45 mm; P < 0.001). The mean (SD) osteotomy length was significantly larger for the RC + plane (61.78 ± 6.75 mm) than for the RC plane (68.48 ± 6.65) mm; P < 0.001). All three evaluated parameters had significantly shorter lengths in women than in men., Conclusion: The safety space for PAO in women was narrower than in men. By shifting the RC plane 10 mm into the posterior direction, the RC + plane provides a safer cutting distance and shorter osteotomy line in the PAO than the RC plan, which is important to avoid intraarticular penetration., (© 2019 The Authors. Orthopaedic Surgery published by Chinese Orthopaedic Association and John Wiley & Sons Australia, Ltd.)

Background/aim: Reconstruction of diaphyseal tibial sarcomas with extracorporeal irradiated autograft is a rarely applied technique and is analyzed in this study., Patients and Methods: Eight patients with malignant sarcomas received local treatment by means of a wide resection and reimplantation of an extracorporeally-irradiated autograft. The graft was combined with an ipsilateral vascularized fibula when a full-thickness segment of the tibia had to be resected and no cortex could be preserved (n=5). Oncological and functional results were recorded., Results: All patients had clear margins after resection, and with no local recurrence 72 months after treatment. Full weight-bearing was allowed at the time of radiological consolidation of the irradiated grafts (after a median of five months). The functional results were good and excellent in 7 of 8 patients, respectively., Conclusion: Extracorporeal irradiation grafting is a suitable method for the treatment of localised and resectable tibial sarcomas., (Copyright© 2019, International Institute of Anticancer Research (Dr. George J. Delinasios), All rights reserved.)

Background: Structural leg length discrepancy (LLD) is a common phenomenon. However, its effect on spinal gait kinematics remains unclear., Research Question: How does LLD affect spinal gait kinematics in patients with structural LLD and what is the immediate effect of a shoe lift?., Methods: 10 adolescents with structural LLD (20-60 mm) and 14 healthy controls were included. All of whom were fitted with a trunk marker set and requested to walk barefoot as well as with an orthotic shoe lift (only patients). Data were collected using a 12-camera motion capture system. Group comparisons were conducted using one-dimensional Statistical Parametric Mapping (SPM)., Results: Patients with LLD showed statistically significant increased frontal plane lumbar bending angles to the longer side (p = 0.007), increased pelvic drop on the shorter side (p < 0.001) and increased hip adduction angles on the longer leg (p < 0.001) compared to the healthy controls. In the sagittal plane, patients demonstrated changed knee (shorter leg) and ankle joint (longer leg) motion. All gait deviations observed in patients with LLD could immediately be altered by correcting the LLD using a shoe lift., Significance: Due to the LLD, patients showed a lateral pelvic drop on the shorter side, which appeared to be compensated for by a contralateral bending in the lumbar spine and a lateral shift of the pelvis towards the longer side. In addition, the use of an orthotic correction seems to be a suitable option to instantly normalize gait kinematics in patients with mild to moderate LLD., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Purpose: Intramedullary lengthening nails are an accepted alternative to external fixators but are limited by anatomical preconditions. Therefore, to date the use of external fixators is sometimes inevitable. We report on a new technique for correction of combined limb length discrepancies and complex axis deformities using solely internal devices - a lengthening nail and a locking plate., Methods: Between October 2008 and November 2011 five patients (two femora, three tibias) with a mean leg length discrepancy of 36 mm (25 to 50) and a complex angular deformity were treated with a fully implantable motorized lengthening nail (Fitbone) and a locking plate. All patients were evaluated with regards to the pre- and postoperative leg length as well as axis alignment, functional outcome, lengthening indices and complications., Results: A successful leg length equalization was achieved in all cases and physiological joint orientation angles in all but one case. The mean distraction index was 1.2 mm/day, the maturation index 24 days/cm and the consolidation index 35 days/cm. The functional outcome was very encouraging in all cases with bilateral free range of movement. In total, two complications were observed, one nonunion and one loss of leg length after an early locking bolt removal in a peripheral hospital., Conclusion: The combination of a fully implantable motorized lengthening nail and a locking plate is a valuable alternative option for treating selected cases with limb length discrepancies in combination with a complex deformity of the lower leg. However, the reported technique puts high demands on the preoperative planning, operative technique as well as surgeon's skills., Level of Evidence: IV (retrospective series).

This study aimed to determine the alteration in maximum isokinetic torque in patients after intramedullary femoral leg lengthening. Thirty patients with a median leg-length discrepancy of 3.0 cm underwent femoral limb lengthening with an intramedullary motorized device. Maximum isokinetic, concentric torque of the extensors, and flexors of the knee was measured before (n=30) and 2 years after surgery (n=21). Postoperatively, a significant difference remained for the maximum isokinetic torque of the extensors (22%) between the lengthened and the normal leg, which might have been caused by muscle response to the distraction procedure itself in the form of higher stiffness, less immediate displacement, and inconsistent force relaxation properties. However, we provide evidence that physiotherapy after limb lengthening should focus on extensors to prevent loss of strength.

Diagnostic tools for the management of acute osteomyelitis (OM) and septic arthritis (SA) have improved over the last decade. To investigate the influence and availability of magnetic resonance imaging (MRI) and nucleic acid testing (NAT), a retrospective cohort study was done. Patients admitted with acute OM or SA between 2005 and 2014 were identified using ICD-10 discharge codes. Ninety-six children were identified: OM, n = 45; SA, n = 42; and OM + SA, n = 9. Diagnostic imaging was performed in 100% of OM or OM + SA and 95% of SA patients. MRI was performed in 85% of OM patients, 26% of SA patients and 100% OM + SA patients. In patients with OM or SA, concomitant joint/bone involvement was detected in 24 and 36% of patients, respectively. In 58% of patients, a pathogen was detected (Staphylococcus aureus, Streptococcus pyogenes and Streptococcus pneumoniae being most common). Blood and tissue culture were positive in 41 and 86% for OM patients and in 14 and 41%, respectively, for SA patients. In 42% of patients, no pathogen was identified, of which 40% had no material for blood or tissue culture/NAT taken., Conclusion: Optimal use of imaging modalities including MRI and systematic pathogen detection including NAT should be advocated to limit use of broad spectrum antibiotics and treatment duration. What is Known: • Magnetic resonance imaging and sonography have the best sensitivity for detection of acute osteomyelitis and septic arthritis in children. • Systematic use of blood cultures, tissue cultures and nucleic acid testing improves pathogen detection in children with acute osteomyelitis and septic arthritis. What is New: • The added value of imaging modalities other than magnetic resonance and sonography for detection of osteomyelitis and septic arthritis is limited, and their routine use should be questioned. • Despite availability of optimal pathogen detection methods, missed opportunities to improve pathogen detection are frequent.

Background: The reconstruction of meta-/diaphyseal bone defects following bone tumour resection is challenging, and biological treatment options should be applied whenever possible, especially in benign lesions and early stage sarcomas. We aimed to evaluate the results of segmental (SR) and hemicortical reconstructions (HR) at the extremities using non-vascularised fibula grafts., Methods: We retrospectively enrolled 36 patients who were treated with non-vascularised fibula reconstructions (15 SR, 21 HR) after bone tumour resection (15 malignant, 21 benign). All cases were evaluated regarding consolidation, hypertrophy at the graft-host junctions, and complications; moreover, the functional and oncological results were assessed. The mean follow-up was 8.3 years (2.1-26.6 years)., Results: Primary union was achieved in 94% (SR 87%, HR 100%) of patients, and 85% (SR 81%, HR 88%) showed hypertrophy at the graft-host junction. The overall complication rate was 36% with 4 patients (11%) developing local recurrence. There was a significant correlation between the development of mechanical complications (fracture, delayed-/non-union) and a defect size of ≥12 cm (p = 0.013), segmental defects (p = 0.013) and additional required treatment (p = 0.008). The functional outcome was highly satisfactory (mean MSTS score 86%)., Conclusions: Due to encouraging results and advantages (such as their remodelling capacity at the donor site), non-vascularised fibula reconstructions should be considered a valuable alternative treatment option for patients with hemicortical defects or segmental reconstructions of less than 12 cm in which no additional neo-/adjuvant treatment is necessary.

Congenital pseudarthrosis of the clavicle (CPC) is a very rare condition with a predilection for the right clavicle. Young children are often only symptomatic with activities and as they grow. Operative management in an asymptomatic child is debatable, with various techniques reported in the literature. This is a retrospective, single-centre case series study. All children with CPC treated surgically by resection, bone grafting (nonvascular iliac crest) and plate fixation between 2004 and 2012 at our centre were included. Excluded conditions were traumatic or obstetric fractures of the clavicle, children with musculoskeletal or neurological disorders and children lost to follow-up. Clinical and radiological examination was performed at 6 weeks, 3 months and between 6 and 12 months postoperatively. A total of eight clavicles in seven children (four girls and three boys), with a mean age of 7.1 years (5-8 years), were operated with a mean follow-up of 7 years (4-10 years). In six children, the right (dominant) side was affected, with one child affected bilaterally. Overall, 85% of our patients showed good functional results 6 weeks postoperatively and complete radiographic consolidation at a mean of 8.5 months (3-25 months) postoperatively. One female child showed delayed union, but without clinical deficit. There was low donor-site morbidity. We recommend early operative treatment of CPC with a nonvascularized bone graft from the iliac crest and plate fixation., Level of Evidence: IV.

Purpose: Prognosis of extraskeletal osteosarcoma (ESOS) is reported to be poorer than that of skeletal osteosarcoma. This multicenter retrospective study aimed to evaluate factors influencing ESOS prognosis., Patients and Methods: Members of the European Musculoskeletal Oncology Society (EMSOS) submitted institutional data on patients with ESOS., Results: Data from 274 patients treated from 1981 to 2014 were collected from 16 EMSOS centres; 266 patients were eligible. Fifty (18.7%) had metastases at diagnosis. Of 216 patients with localised disease, 211 (98%) underwent surgery (R0 = 70.6%, R1 = 27%). Five-year overall survival (OS) for all 266 patients was 47% (95% CI 40-54%). Five-year OS for metastatic patients was 27% (95% CI 13-41%). In the analysis restricted to the 211 localised patients who achieved complete remission after surgery 5-year OS was 51.4% (95% CI 44-59%) and 5-year disease-free survival (DFS) was 43% (95% CI 35-51%). One hundred twenty-one patients (57.3%) received adjuvant or neoadjuvant chemotherapy and 80 patients (37.9%) received radiotherapy. A favourable trend was seen for osteosarcoma-type chemotherapy versus soft tissue sarcoma-type (doxorubicin ± ifosfamide) regimens. For the 211 patients in complete remission after surgery, patient age, tumour size, margins and chemotherapy were positive prognostic factors for DFS and OS by univariate analysis. At multivariate analysis, patient age (≤40 years versus >40 years) (P = 0.05), tumour size (P = 0.0001) and receipt of chemotherapy (P = 0.006) were statistically significant prognostic factors for survival., Conclusion: Patient age and tumour size are factors influencing ESOS prognosis. Higher survival was observed in patients who received perioperative chemotherapy with a trend in favour of multiagent osteosarcoma-type regimen which included doxorubicin, ifosfamide and cisplatin., (Copyright © 2017 Elsevier Ltd. All rights reserved.)

Aims: The severe form of coxa vara, the 'shepherd's crook deformity', is always a consequence of a locally extensive form of polyostotic fibrous dysplasia (or McCune-Albright syndrome). Treatment of this deformity is a challenge. The soft bone does not tolerate any implant that depends on the stability of the cortical bone (like plates or external fixators). Intramedullary nails are the most appropriate implants for stabilisation, but if they are inserted from the greater trochanter, they cannot correct the varus deformity enough., Patients and Methods: We have developed a special intramedullary nail that can be inserted from the osteotomy site and can be driven retrograde into the femoral neck in an appropriate valgus position. We have operated 15 legs in 13 patients. The average age at surgery was 14 years and 5 months (6 to 28.9). In all, 11 femora had been operated before (unsuccessfully) with various implants., Results: The average follow-up was 54.2 months (7 to 132). The average correction of the neck/(distal) shaft angle was 57.5° (10° to 80°) ( = 72.8%). While pre-operatively none of the patients was able to walk without aid, at follow-up only one patient was unable to walk, three used the aid of crutches because of tibial lesions and one patient had an increased external rotation of the leg. At follow-up, most patients were free of pain. One implant broke and had to be replaced., Conclusion: This new operative method offers the possibility of efficient correction and stabilisation of this severe and difficult deformation.

PURPOSE OF THE STUDY Ewing sarcomas (ES) are the second most common solid malignant bone tumors in both, children and adolescents, and systemic chemotherapy protocols were established during the last 3 decades which proved to be a successful approach in addition to local treatment. The purpose of the present study is (i) to provide survival rates and prognostic factors for patients with ES which received treatment in a single center and (ii) to compare data with results of multicenter studies. MATERIALS AND METHODS Patients (n = 38) were treated by the same surgeon whereas surgery was combined with radiotherapy in 55.3% of the patients (n = 21). Median age at diagnosis was 17.5 years (4.7-60) and the median follow-up time for all patients was 8.2 years (9.8 years for survivors, 3.2 years for non-survivors). RESULTS The survival rate for metastasis free sarcoma decreases from 90.5% to 50% for patients diagnosed with disseminated disease stage. Patients with a good response to chemotherapy survived in 83.3% of the cases. In addition, a higher OS was found for patients younger than 15 years (82.4%) when compared to patients older than 15 years (73.3%). In contrast, multicenter studies reported lower survival rates for metastasis free (~60%) and metastasis stages (< 40%). DISCUSSION The survival rates in the present single center study are higher than the rates reported from multi-center studies although same chemotherapy protocols were used and no substantially difference are apparent for patient population. CONCLUSIONS Based on the present data we re-emphasize that patients with Ewing sarcoma receive appropriate treatment in a large and qualified center particularly considering the survival rates. In addition, our data underline that a close collaboration between the oncological team and the experienced surgeon is crucial for patient's care. Key words: Ewing sarcoma, survival rate, single center, prognostic factors, chemotherapy, surgery, multi center, single center.

Unlike external fixators, the use of solid intramedullary lengthening nails is restricted to defined anatomical preconditions, such as an adequate bone length. Furthermore, all deformity corrections except the lengthening procedure have to be implemented intraoperatively and cannot be adjusted postoperatively. Conversely, even complex deformity corrections can be performed using intramedullary devices after a thorough preoperative planning. For preparation of the intramedullary cavity as well as positioning of the lengthening nail according to the preoperative planning, reaming the medullary canal with rigid reamers which don't follow the line of least resistance is inevitable. However, the application of solid lengthening nails might be limited, especially in children with ongoing epiphyseal growth, although a central perforation of the growth plate was shown to have no adverse effects on the growth potential. In cases with complex or multilevel deformities, an additional osteotomy and locking plate fixation could sometimes be a valuable solution in order to avoid external fixation. The low complication rate as well as the reduced compromising of soft tissues and periosteum render intramedullary lengthening nails the state-of-the-art procedure for limb lengthening in combination with deformity correction in patients who meet the anatomical preconditions.

Background: Tumor-associated fibroblast growth factor 23 (FGF-23)-induced hypophosphatemic rickets is a rare but known pediatric entity first described in 1959. It results from local production of phosphatonins by benign and malignant mesenchymal tumors., Case-Diagnosis/treatment: We report an 8-year-old boy with tumor-associated hypophosphatemic rickets due to paraneoplastic FGF-23 secretion from a benign mesenchymal pelvic-bone tumor. Excessive FGF-23 production was visualized by immunohistochemistry in the resected tumor. Phosphate wasting stopped immediately after tumor resection. We reviewed 26 reports of pediatric patients with tumor-induced hypophosphatemic rickets; paraneoplastic FGF-23 secretion was documented in only three of them. All tumors developed inside bone, were benign in 21/26 cases, and were localized in femur/tibia (13/26), radius/ulna/humerus (7/26), pelvis (4/26), rib (1/26), and craniofacial (1/26) bones. Mean interval between onset of signs and/or symptoms and diagnosis was 34 months., Conclusions: In patients with hypophosphatemic rickets acquired beyond infancy, radiologic investigations for bone tumors need to be performed rapidly. In contrast to biochemical screening for increased circulating FGF-23 levels, immunohistochemical confirmation of FGF-23 production in resected tumor tissue can be regarded as being well established.

Purpose: The eight-plate system for angular deformity correction is well known, reliable and effective at any age during growth. Due to high implant costs, we sought to evaluate the effectiveness and safety of a less expensive alternative., Methods: Between 2006 and 2011, 41 children with angular deformities were managed using a two-hole one-third tubular plate in cases where an eight plate would normally be indicated. Inclusion criteria in this retrospective study were: genu valgum and genu varum. X-ray documentation was performed before and after surgery and patients were followed clinically every 3 months after surgery. The cost per implant was 361.40 Sfr (Swiss Francs) compared to the eight plate at 737 Sfr., Results: Mean time for correction was 13 months. A mean LDFA/MPTA after correction of 89.9°/86.8° was recorded, as well as a mean correction angle of 6.8°/6.6°. The complication rate was 6.6 % (one superficial wound infection and one insufficient correction in an older child). These results compare favourably with published data on the eight plate., Conclusion: The two hole one-third tubular plate seems to be a clinically and also cost effective alternative to the eight plate. Full deformity correction is gained for a fraction of the cost., Level of Evidence: Level III.

Tumors in the pelvic region cause deformation and destruction of bony structures. Because the original pelvic anatomy cannot be adequately assessed at the tumor site, reconstruction with patient-specific implants is required. A widely used strategy for the reconstructive planning is mirroring of the contralateral side. We analyzed the statistical shape model (SSM)-based reconstruction method and compared it with the mirroring approach. Our approach used a gender-specific pelvic SSM (n = 50 for each gender) to generate implant geometries. The main objectives of this study were to analyze and evaluate the virtual anatomical reconstruction of eight tumor-damaged pelvic bones using the SSM approach. We achieved an overall mean deviation distance of 0.89 mm and 1.26 mm for the reconstruction of the equivalent defect in the healthy hemipelvis. Quantitative comparison with the mirroring method showed that the SSM-based reconstruction method reconstructs the defect with the same clinically acceptable accuracy as the mirroring method. The study demonstrates that the presented model can be a valuable tool for the planning of pelvic reconstructive surgery and implant design.

We analyze the delay in diagnosis and tumor size of malignant bone tumors of the foot in a retrospective study. We compared the oncological and surgical long-term results with identical tumor at other anatomical sites in order to analyze the biological behavior of sarcomas that are found in the foot. Thirty-two patients with a histologically proven malignant bone tumor (fifteen chondrosarcomas, nine osteosarcomas, and eight Ewing sarcomas) between the years 1969 and 2008 were included. The median follow-up was 11.9 years. The overall median time gap between the beginning of symptoms and diagnosis in the study group was 10 months. Ewing sarcoma presented with the longest delay in diagnosis (median of 18 months), followed by osteosarcoma (median of 15 months) and chondrosarcoma (median of 7.5 months). The delay in diagnosis of these tumors was significantly longer than that of equivalent tumors at other skeletal sites, but the 5- and 10-year survival rates and the occurrence of distant metastases were comparable. In contrast, the average size of foot tumors was 5- to 30-fold less than that of tumors analyzed at other skeletal sites. This study indicates that sarcomas of the foot demonstrate a distinct biological behavior compared to the same tumor types at other skeletal sites.

Any lower limb discrepancy may be equalised by conservative means (insoles, prosthesis and orthosis). However, their long-term acceptance is low in regard to function, costs, expenditure and appearance. Timely epiphysiodesis is the best option in uniplanar deformities with adequate remaining growth and for patients whose predicted final body height is above the 50th percentile. However, many patients present late or with multi-planar deformities, which warrant more sophisticated operative approaches. The history of surgical bone lengthening comprises 100 exciting years of struggling, development and ongoing learning. The initial strategy of acute or rapid incremental distraction had lasted almost half a century until Ilizarov recognised the benefits of biological periosteum-preserving osteotomies and incremental lengthening at slow rates (1 mm/day) at a 4 × 0.25-mm daily rhythm, well appreciated as callotasis. In parallel, ring and wire constructs made complex three-dimensional axial, translational and rotational bone moulding possible. Taylor Spatial Frames-built on hexapod strut-linked platform technology as known from flight simulators-took limb correction to a more reliable, more precise and aesthetical level, all the more that the whole process became web-based. It represents state-of-the-art methodology and technology for complex, multi-plane deformities. Due to the significant risk of secondary malalignment, indications for lengthening by unilateral fixation have shrunken to moderate amounts of length disparity and uni- to bi-planar deformities in patients with still open physes. Mechanical or motorised, minimally invasively placed nails prevent muscle fixation and, therefore, ease rehabilitation, increase patient comfort and potentially shorten the overall time of sick leave and refrain from sports activities. Hence, they offer a valuable alternative for low-grade complexity situations. It remains to be proved if the significantly higher implant costs are compensated by lower treatment costs. Overall, limb lengthening, particularly in combination with multi-planar deformity correction, can still be an arduous endeavour. In any case, wise judgement of the patient's deformity, medical and biological situation, psychosocial environment, selection of the appropriate method and hardware, as well as meticulous operating technique by an experienced surgeon are the cornerstones of successful outcomes.

Purpose: Synovial sarcoma (SS) is an aggressive soft-tissue tumor noted for late local recurrence and metastasis. This study investigates the long-term outcome of SS in patients of pediatric age and evaluates potential prognostic factors for SS., Methods: We performed a retrospective review of 13 SS cases in patients younger than 20 years at the time of diagnosis who had a minimum follow-up of 10 years. The mean follow-up for living patients (n = 8) was 20.1 years (12.1-27.6) and for nonsurvivors (n = 5) 4.9 years (range: 2.6-9.3). Nine patients had unplanned excisions (69%), of which 6 (67%) were performed prior to their referral. Re-excisions were necessary in all 13 patients. The factors sex, tumor site, tumor size, tumor grade, histological subtype, fusion type, and type of treatment were evaluated for their prognostic value., Results: Only 2 patients (15%) met the criteria of adequate tumor treatment. Overall, the 5- and 10-year survival rates were 77 and 61%, respectively. The mean time until a local recurrence (n = 5) was 3.2 years (range: 0.7-10.2), while there was a mean time of 2.1 years until the occurrence of late metastases (n = 5; range: 0.8-4.8). A high tumor grade and having a tumor in the trunk were adverse factors in terms of overall, local recurrence-free, and metastasis-free survival. Patients with wide resections or amputations had fewer local recurrences than patients with marginal or intralesional resections., Conclusion: Inadequate primary excision of SS results in incomplete excision in the majority of cases. The tumor site, size, and histological grade should be considered when determining a risk-adapted treatment for SS, and wide surgical excision is the surgical intervention of choice. While local recurrence and late metastases appear to occur after a shorter time period in pediatric patients than in adults, in view of the tendency for late recurrence and metastasis with SS, follow-up should be at least 10 years.

Background: Dynamic three-dimensional (3D) deformation of the pelvic bones is a crucial factor in the successful design and longevity of complex orthopaedic oncological implants. The current solutions are often not very promising for the patient; thus it would be interesting to measure the dynamic 3D-deformation of the whole pelvic bone in order to get a more realistic dataset for a better implant design. Therefore we hypothesis if it would be possible to combine a material testing machine with a 3D video motion capturing system, used in clinical gait analysis, to measure the sub millimetre deformation of a whole pelvis specimen., Method: A pelvis specimen was placed in a standing position on a material testing machine. Passive reflective markers, traceable by the 3D video motion capturing system, were fixed to the bony surface of the pelvis specimen. While applying a dynamic sinusoidal load the 3D-movement of the markers was recorded by the cameras and afterwards the 3D-deformation of the pelvis specimen was computed. The accuracy of the 3D-movement of the markers was verified with 3D-displacement curve with a step function using a manual driven 3D micro-motion-stage., Results: The resulting accuracy of the measurement system depended on the number of cameras tracking a marker. The noise level for a marker seen by two cameras was during the stationary phase of the calibration procedure ± 0.036 mm, and ± 0.022 mm if tracked by 6 cameras. The detectable 3D-movement performed by the 3D-micro-motion-stage was smaller than the noise level of the 3D-video motion capturing system. Therefore the limiting factor of the setup was the noise level, which resulted in a measurement accuracy for the dynamic test setup of ± 0.036 mm., Conclusion: This 3D test setup opens new possibilities in dynamic testing of wide range materials, like anatomical specimens, biomaterials, and its combinations. The resulting 3D-deformation dataset can be used for a better estimation of material characteristics of the underlying structures. This is an important factor in a reliable biomechanical modelling and simulation as well as in a successful design of complex implants.

Background and Purpose: In the last decade, intramedullary limb lengthening has become a viable alternative to traditional external systems. We retrospectively analyzed the use of an intramedullary motorized nail (Fitbone) in a consecutive series of 32 patients., Patients and Methods: During the period September 2006 to December 2008, 32 consecutive patients with a median age of 17 (IQR: 15-19) years were treated with a fully implantable, motorized intramedullary lengthening device (Fitbone). The median leg length discrepancy was 35 (IQR: 30-44) mm at the femur (n = 21) and 28 (IQR: 25-30) mm at the tibia (n = 11)., Results: Leg lengthening was successful in 30 of 32 cases, with no residual relevant discrepancy (± 5 mm). No intraoperative complications were observed. The consolidation index was significantly different (p = 0.04) between femoral lengthening (mean 35 days/cm) and tibial lengthening (mean 48 days/cm) but did not depend on age older/younger than 16 or previous operations at the affected site. 3 problems, 3 obstacles, and 4 complications (3 minor, 1 major) were encountered in 8 patients, 5 of which were implant-associated., Interpretation: This technique even allows correction in patients with multiplanar deformities. Compared to external devices, intramedullary systems provide comfort and reduce complication rates, give improved cosmetic results, and lead to fast rehabilitation since percutaneous, transmuscular fixation is prevented. This results in reasonable overall treatment costs despite the relatively high costs of implants.

Background: Synovial sarcoma (SS) is a malignant soft tissue sarcoma with a poor prognosis because of late local recurrence and distant metastases. To our knowledge, no studies have minimum follow-up of 10 years that evaluate long-term outcomes for survivors., Patients and Methods: Data on 62 patients who had been treated for SS from 1968 to 1999 were studied retrospectively in a multicenter study. Mean follow-up of living patients was 17.2 years and of dead patients 7.7 years., Results: Mean age at diagnosis was 35.4 years (range 6-82 years). Overall survival was 38.7%. The 5-year survival was 74.2%; 10-year survival was 61.2%; and 15-year survival was 46.5%. Fifteen patients (24%) died of disease after 10 years of follow-up. Local recurrence occurred after a mean of 3.6 years (range 0.5-14.9 years) and metastases at a mean of 5.7 years (range 0.5-16.3 years). Only four patients were treated technically correctly with a planned biopsy followed by a wide resection or amputation. Factors associated with significantly worse prognosis included larger tumor size, metastases at the time of diagnosis, high-grade histology, trunk-related disease, and lack of wide resection as primary surgical treatment., Conclusions: In SS, metastases develop late with high mortality. Patients with SS should be followed for >10 years.

Purpose: The Ilizarov hip reconstruction is a well accepted but complication-prone operative salvage procedure in chronically dislocated hips, not least due to the long-term application of external fixation. Although the advantages of fully implantable devices are well known in limb lengthening and are described consistently, until now, external fixation has been used exclusively to perform the Ilizarov hip reconstruction procedure. We present a new technique of Ilizarov hip reconstruction with purely internal implants., Methods: A 14-year-old girl with a history of spina bifida presented with a 4-cm-short right leg, a Trendelenburg gait and a complex neurological disease expression. Because of refusal of external fixation by the patient and significantly lower complication rates, an Ilizarov hip reconstruction without external fixation was performed. A locking compression plate was applied to fix the proximal femoral valgus-extension osteotomy and a motorised intramedullary distraction nail was used for the distal, lengthening-varisation osteotomy., Results: A healing index of 33 days/cm and full weight bearing after 6 months were noted. At the 1 year follow-up, the patient showed an improvement of the Trendelenburg gait, as well as successful leg equalisation. Satisfaction to a high degree was additionally noted by factors such as reduced pain, the ability to wear workaday clothes and cosmetically appealing scars. No complications were recorded., Conclusion: The exclusive use of internal implants for Ilizarov hip reconstruction is a feasible and patient-friendly alternative to traditional methods. Their use, however, may be restricted by geometric preconditions.

Polyethylene wear of acetabular components is a key factor in the development of periprosthetic osteolysis and wear at the articular surface has been well documented and quantified, but fewer data are available about changes which occur at the backside of the liner. At revision surgery for loosening of the femoral component we retrieved 35 conventional modular acetabular liners of the same design. Linear and volumetric articular wear, backside volumetric change and the volume of the screw-head indentations were quantified. These volumes, clinical data and the results from radiological Ein Bild Röntgen Analyse migration analysis were used to identify potential factors influencing the volumetric articular wear and backside volumetric change. The rate of backside volumetric change was found to be 2.8% of the rate of volumetric articular wear and decreased with increasing liner size. Migrated acetabular components showed significantly higher rates of backside volumetric change plus screw-head indentations than those without migration. The backside volumetric change was at least ten times larger than finite-element simulation had suggested. In a stable acetabular component with well-anchored screws, the amount of backside wear should not cause clinical problems. Impingement of the screw-heads could produce more wear particles than those generated at the liner-shell interface. Because the rate of backside volumetric change is only 2.8% of the rate of volumetric articular wear and since creep is likely to contribute a significant portion to this, the debris generated by wear at the backside of the liner may not be sufficient to create a strong osteolytic response.

Staged bilateral hip arthroplasties were carried out in two patients with osteoarthritis of the hip secondary to the development of hip dysplasia in Hereditary Multiple Exostosis (HME).Both patients had near ankylosis of their hips with pain and difficulty in performing activities of daily living. A proximal femur replacing prosthesis was implanted through an extended trochanteric osteotomy, with preservation of as much proximal bone stock as possible. At latest followup (two to five years), there was a sustained improvement in range of motion and symptoms. The Harris Hip Scores improved from 25 and 31 to 83 and 78. The Toronto Extremity Salvage Scores improved from 49% and 55% to 88% and 75%. Radiographs show bony ingrowth and healing of the trochanteric fragments. In severe HME, hip arthroplasty with replacement of the proximal femur provides good symptomatic relief and return of hip joint movement.