The Importance of Monitoring and Factors That May Influence Leg Length Difference in Developmental Dysplasia of the Hip.

In unilateral Developmental Dysplasia of the Hip (DDH), avascular necrosis (AVN), femoral or pelvic osteotomy, and residual dysplasia causing subluxation of the proximal femur may influence Leg Length Discrepancy (LLD). This can lead to gait compensation, pelvic obliquity, and spinal curvature. The aim of this study is to determine the prevalence of LLD, establish which limb segment contributes to the discrepancy, describe how AVN influences LLD, and ascertain variables that may influence the need for LLD corrective procedures. METHODOLOGY: This study assessed long-leg radiographs at skeletal maturity. Radiographs were assessed for the articulo-trochanteric distance (ATD) and femoral and tibial length. AVN was classified according to Kalamchi-MacEwen. RESULTS: 109 patients were included. The affected/DDH leg was longer in 72/109 (66%) patients. The length difference was mainly in the subtrochanteric segment of the femur. AVN negatively influenced leg length. Older (≥three years) patients with multiple procedures were more likely to have AVN. LLD interventions were performed in 30 (27.5%) patients. AVN grade or type of DDH surgery did not influence the odds of needing a procedure to correct LLD. CONCLUSIONS: Procedures to correct LLD were performed irrespective of previous DDH surgery or AVN grades. In most patients, the affected/DDH leg was longer, mainly in the subtrochanteric segment of the femur, largely influenced by femoral osteotomy in patients with multiple operative procedures for DDH. We recommend careful monitoring of LLD in DDH.

Tension-band Plating for Leg-length Discrepancy Correction.

Aim: Dual tension-band plates are used for temporary epiphysiodesis and longitudinal guided growth. The study aim was to assess rate of correction, to identify development of femoral and tibial intra-articular deformity during correction and to document resumption of growth after plate removal. Materials and methods: A retrospective study of 34 consecutive patients treated with dual tension-band plates between 2012 and 2020 was performed. Twenty-four patients had surgery at the distal femur, six at the proximal tibia and four at both. Twenty-five female patients were treated at a mean age of 11.6 (±1.4) years and nine male patients at 13.5 (±1.5) years. Measurements were performed on standardised long-leg radiographs and included leg-length discrepancy (LLD), joint line congruency angle (JLCA), tibial roof angle, femoral floor angle and notch-intercondylar distance. Measurements were taken pre-operatively, at the end of discrepancy correction and at skeletal maturity. Results: The LLD reduced by a mean of 12.9 mm (95% CI 10.2-15.5) with the mean residual difference 8.4 mm (95% CI 5.4-11.4). The mean correction rate for the proximal tibia was 0.40 (SD 0.33) mm/month and 0.68 (SD 0.36) mm/month for the distal femur. A significant mean change in residual LLD [-2.5 mm (95% CI -4.2 to -0.7)] was observed between plate removal and skeletal maturity at the femoral level only. After length discrepancy correction, the tibial roof angle showed a significant difference of 8.4° (95% CI 13.4-3.4) between legs. In femoral epiphysiodesis patients, no important differences were observed. Conclusion: A significant reduction in LLD can be achieved using dual tension-band plating. A change in intra-articular morphology was observed only in the proximal tibia and not in the distal femur. In the authors' opinion, tension-band plating is a useful tool for leg-length equalisation but should be reserved for younger patients or when residual growth is difficult to predict. It is one of the management strategies for limb-length difference prior to skeletal maturity. How to cite this article: Tolk JJ, Merchant R, Calder PR, et al. Tension-band Plating for Leg-length Discrepancy Correction. Strategies Trauma Limb Reconstr 2022;17(1):19-25.

Osteofibrous dysplasia of the tibia : the importance of deformity in surveillance.

AIMS: Osteofibrous dysplasia (OFD) is a rare benign lesion predominantly affecting the tibia in children. Its potential link to adamantinoma has influenced management. This international case series reviews the presentation of OFD and management approaches to improve our understanding of OFD. METHODS: A retrospective review at three paediatric tertiary centres identified 101 cases of tibial OFD in 99 patients. The clinical records, radiological images, and histology were analyzed. RESULTS: Mean age at presentation was 13.5 years (SD 12.4), and mean follow-up was 5.65 years (SD 5.51). At latest review, 62 lesions (61.4%) were in skeletally mature patients. The most common site of the tibial lesion was the anterior (76 lesions, 75.2%) cortex (63 lesions, 62.4%) of the middle third (52 lesions, 51.5%). Pain, swelling, and fracture were common presentations. Overall, 41 lesions (40.6%) presented with radiological deformity (> 10°): apex anterior in 97.6%. A total of 41 lesions (40.6%) were treated conservatively. Anterior bowing < 10° at presentation was found to be related to successful conservative management of OFD (p = 0.013, multivariable logistic regression). Intralesional excision was performed in 43 lesions (42.6%) and a wide excision of the lesion in 19 (18.8%). A high complication rate and surgical burden was found in those that underwent a wide excision regardless of technique employed. There was progression/recurrence in nine lesions (8.9%) but statistical analysis found no predictive factors. No OFD lesion transformed to adamantinoma. CONCLUSION: This study confirms OFD to be a benign bone condition with low rates of local progression and without malignant transformation. It is important to distinguish OFD from adamantinoma by a histological diagnosis. Focus should be on angular deformity, monitored with full-length tibial radiographs. Surgery is indicated in symptomatic patients and predicted by the severity of the initial angular deformity. Surgery should focus more on the deformity rather than the lesion. Cite this article: Bone Joint J 2022;104-B(2):302-308.

Development of a core outcome set for the orthopaedic management of spinal dysraphism : a study protocol.

AIMS: The aim of this study is to define a core outcome set (COS) to allow consistency in outcome reporting amongst studies investigating the management of orthopaedic treatment in children with spinal dysraphism (SD). METHODS: Relevant outcomes will be identified in a four-stage process from both the literature and key stakeholders (patients, their families, and clinical professionals). Previous outcomes used in clinical studies will be identified through a systematic review of the literature, and each outcome will be assigned to one of the five core areas, defined by the Outcome Measures in Rheumatoid Arthritis Clinical Trials (OMERACT). Additional possible outcomes will be identified through consultation with patients affected by SD and their families. RESULTS: Outcomes identified in these stages will be included in a two-round Delphi process that will involve key stakeholders in the management of SD. A final list including the identified outcomes will then be summarized in a consensus meeting attended by representatives of the key stakeholders groups. CONCLUSION: The best approach to provision of orthopaedic care in patients with SD is yet to be decided. The reporting of different outcomes to define success among studies, often based on personal preferences and local culture, has made it difficult to compare the effect of treatments for this condition. The development of a COS for orthopaedic management in SD will enable meaningful reporting and facilitate comparisons in future clinical trials, thereby assisting complex decision-making in the clinical management of these children. Cite this article: Bone Jt Open 2022;3(1):54-60.

The outcomes of idiopathic congenital talipes equinovarus : a core outcome set for research and treatment.

AIMS: To identify the minimum set of outcomes that should be collected in clinical practice and reported in research related to the care of children with idiopathic congenital talipes equinovarus (CTEV). METHODS: A list of outcome measurement tools (OMTs) was obtained from the literature through a systematic review. Further outcomes were collected from patients and families through a questionnaire and interview process. The combined list, as well as the appropriate follow-up timepoint, was rated for importance in a two-round Delphi process that included an international group of orthopaedic surgeons, physiotherapists, nurse practitioners, patients, and families. Outcomes that reached no consensus during the Delphi process were further discussed and scored for inclusion/exclusion in a final consensus meeting involving international stakeholder representatives of practitioners, families, and patient charities. RESULTS: In total, 39 OMTs were included from the systematic review. Two additional OMTs were identified from the interviews and questionnaires, and four were added after round one Delphi. Overall, 22 OMTs reached 'consensus in' during the Delphi and two reached 'consensus out'; 21 OMTs reached 'no consensus' and were included in the final consensus meeting. In all, 21 participants attended the consensus meeting, including a wide diversity of clubfoot practitioners, parent/patient representative, and an independent chair. A total of 21 outcomes were discussed and voted upon; six were voted 'in' and 15 were voted 'out'. The final COS document includes nine OMTs and two existing outcome scores with a total of 31 outcome parameters to be collected after a minimum follow-up of five years. It incorporates static and dynamic clinical findings, patient-reported outcome measures, and a definition of CTEV relapse. CONCLUSION: We have defined a minimum set of outcomes to draw comparisons between centres and studies in the treatment of CTEV. With the use of these outcomes, we hope to allow more meaningful research and a better clinical management of CTEV. Cite this article: Bone Jt Open 2022;3(1):98-106.

A Virtual Clinic for DDH Pavlik Harness Monitoring.

BACKGROUND: Pavlik harness (PH) treatment is successful in treating over 90% of newborns with developmental dysplasia of the hip (DDH). There is a need for close supervision, frequent adjustments, size changes, and monitoring of complications. This paper aims to provide a safe criterion for remote follow-up of DDH patients treated in a PH to reduce the risk of COVID-19 (coronavirus disease 2019) exposure to patients, parents, and health practitioners. METHODS: All infants with stable hips (Graf I, IIa/b/c/d, treated III/IV) with consenting parents after appropriate counseling were enrolled in a virtual clinic. Clinics were conducted using the NHS "Attend anywhere" virtual link service by an extended scope practitioner-specialist physiotherapist and a clinical nurse specialist. The virtual clinic group was compared with a matched cohort of patients from 2018/2019. RESULTS: A total of 141 patients were referred to the neonatal hip clinic; 45 patients were eligible for harness treatment and 20 patients were selected for virtual clinics. In total, there were 35 virtual clinic appointments. Each of the patients had an average of 1.7 virtual appointments ranging from 1 to 3 (26.3% of total number of clinics). Age at presentation of the treated group was 7±4.2 weeks and control group 5.7±5.5 weeks (P=0.59). PH duration of the study group was 9±2.6 weeks and the control group, 7.8±2.5 weeks (P=0.12). There were no missed complications at the follow-up face-to-face appointment. Patients saved an average of 76 km total travel distance. CONCLUSIONS: This study demonstrates adequate evidence that children requiring routine follow-up appointments involving PH adjustment, skincare, and identification of clinical anomalies, can be treated and followed up safely using virtual clinics. Clinical triage of suitable patients for virtual clinic provision must always be made by experienced clinicians. Children presenting with Graf IIa, IIb, IIc, IId, as well as those with stable and improving Graf III at initial diagnoses, had successful treatment with virtual clinic follow-up appointments in this study. LEVEL OF EVIDENCE: Level IV.

Leg length discrepancy in patients with Perthes' disease : a note of caution for the arthroplasty surgeon.

AIMS: Perthes' disease (PD) often results in femoral head deformity and leg length discrepancy (LLD). Our objective was to analyze femoral morphology in PD patients at skeletal maturity to assess where the LLD originates, and evaluate the effect of contralateral epiphysiodesis for length equalization on proximal and subtrochanteric femoral lengths. METHODS: All patients treated for PD in our institution between January 2013 and June 2020 were reviewed retrospectively. Patients with unilateral PD, LLD of ≥ 5 mm, and long-leg standing radiographs at skeletal maturity were included. Total leg length, femoral and tibial length, articulotrochanteric distance (ATD), and subtrochanteric femoral length were compared between PD side and the unaffected side. Furthermore, we compared leg length measurements between patients who did and who did not have a contralateral epiphysiodesis. RESULTS: Overall, 79 patients were included, of whom 21 underwent contralateral epiphysiodesis for leg length correction. In the complete cohort, the mean LLD was 1.8 cm (95% confidence interval (CI) 1.5 to 2.0), mean ATD difference was 1.8 cm (95% CI -2.1 to -1.9), and mean subtrochanteric difference was -0.2 cm (95% CI -0.4 to 0.1). In the epiphysiodesis group, the mean LLD before epiphysiodesis was 2.7 cm (95% CI 1.3 to 3.4) and 1.3 cm (95% CI -0.5 to 3.8) at skeletal maturity. In the nonepiphysiodesis group the mean LLD was 2.0 cm (95% CI 0.5 to 5.1; p = 0.016). The subtrochanteric region on the PD side was significantly longer at skeletal maturity in the epiphysiodesis group compared to the nonepiphysiodesis group (-1.0 cm (95% CI -2.4 to 0.6) vs 0.1 cm (95% CI -1.0 to 2.1); p < 0.001). CONCLUSION: This study demonstrates that LLD after PD originates from the proximal segment only. In patients who had contralateral epiphysiodesis to balance leg length, this is achieved by creating a difference in subtrochanteric length. Arthroplasty surgeons need to be aware that shortening of the proximal femur segment in PD patients may be misleading, as the ipsilateral subtrochanteric length in these patients can be longer. Therefore, we strongly advise long-leg standing films for THA planning in PD patients in order to avoid inadvertently lengthening the limb. Cite this article: Bone Joint J 2021;103-B(11):1736-1741.

Principles of Bracing in the Early Management of Developmental Dysplasia of the Hip.

Bracing is considered a gold standard in treating Developmental Dysplasia of the Hip (DDH) in infants under 6 months of age with reducible hips. A variety of braces are available that work on similar principles of limiting hip adduction and extension. This paper summarises the current evidence regarding bracing in DDH. Most of the literature pertains to the Pavlik harness (PH) and there are few studies for other brace types. Bracing eliminates dislocating forces from the hamstrings, the block to reduction of the psoas and improves the muscle line of pull to stabilise the hip joint. Recent studies have shown no benefit in bracing for stable dysplasia. The rates of PH treatment failure in Ortolani-positive hips have been reported to be high. Barlow positive hips have lower Graf grades and are more amenable to PH treatment. There is consensus that the earlier the diagnosis of DDH and initiation of PH treatment, the better the outcome. Failure rates due to unsuccessful reduction and AVN are higher with treatment initiated after age 4-6 months. Studies have shown no benefits of staged weaning of braces. While there is no maximum time in brace, current consensus suggests a minimum of 6 weeks. The key to successful bracing lies in education and communication with the family.

High-Throughput Digital Image Analysis Reveals Distinct Patterns of Dystrophin Expression in Dystrophinopathy Patients.

Duchenne muscular dystrophy (DMD) is an incurable disease caused by out-of-frame DMD gene deletions while in frame deletions lead to the milder Becker muscular dystrophy (BMD). In the last decade several antisense oligonucleotides drugs have been developed to induce a partially functional internally deleted dystrophin, similar to that produced in BMD, and expected to ameliorate the disease course. The pattern of dystrophin expression and functionality in dystrophinopathy patients is variable due to multiple factors, such as molecular functionality of the dystrophin and its distribution. To benchmark the success of therapeutic intervention, a clear understanding of dystrophin expression patterns in dystrophinopathy patients is vital. Recently, several groups have used innovative techniques to quantify dystrophin in muscle biopsies of children but not in patients with milder BMD. This study reports on dystrophin expression using both Western blotting and an automated, high-throughput, image analysis platform in DMD, BMD, and intermediate DMD/BMD skeletal muscle biopsies. Our results found a significant correlation between Western blot and immunofluorescent quantification indicating consistency between the different methodologies. However, we identified significant inter- and intradisease heterogeneity of patterns of dystrophin expression in patients irrespective of the amount detected on blot, due to variability in both fluorescence intensity and dystrophin sarcolemmal circumference coverage. Our data highlight the heterogeneity of the pattern of dystrophin expression in BMD, which will assist the assessment of dystrophin restoration therapies.

What is the optimal treatment for equinus deformity in walking-age children with clubfoot? A systematic review.

Equinus contracture is the most common deformity at clubfoot relapse and causes pain and functional limitation. It presents a challenge to the orthopaedic surgeon throughout childhood.A systematic review was conducted according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies included were: (i) original articles, (ii) investigating management of relapsed idiopathic clubfoot, (iii) with at least a partial study population of primarily equinus deformity, and (iv) a paediatric study population of independent walking age.Nine studies were included with a total of 163 patients (207 feet). Studies presented five management paradigms: gastrocnemius-soleus complex release, extensive posterior soft tissue and joint release, anterior distal tibial hemi-epiphysiodesis, distal tibial osteotomy, and circular frame distraction.All approaches reported success in at least one of our selected outcome domains: plantigrade status, range of motion, clinical outcome scores, functional status, radiographic outcomes, patient-reported outcomes, and complications. Younger children tend to be managed with soft tissue release while older children tend to require more extensive bone/joint procedures. Relapse in surgically treated feet is harder to treat.Comparison across treatment approaches is limited by the small size and low evidence level of the literature, as well as a lack of consistent outcome reporting. It is therefore not possible to recommend any one treatment option in any age group.This review highlights the need for a validated core outcome set to enable high-quality research into the management of equinus deformity. Cite this article: EFORT Open Rev 2021;6:354-363. DOI: 10.1302/2058-5241.6.200110.

Development of a core outcome set for idiopathic clubfoot management.

AIMS: This study aims to define a set of core outcomes (COS) to allow consistent reporting in order to compare results and assist in treatment decisions for idiopathic clubfoot. METHODS: A list of outcomes will be obtained in a three-stage process from the literature and from key stakeholders (patients, parents, surgeons, and healthcare professionals). Important outcomes for patients and parents will be collected from a group of children with idiopathic clubfoot and their parents through questionnaires and interviews. The outcomes identified during this process will be combined with the list of outcomes previously obtained from a systematic review, with each outcome assigned to one of the five core areas defined by the Outcome Measures Recommended for use in Randomized Clinical Trials (OMERACT). This stage will be followed by a two round Delphi survey aimed at key stakeholders in the management of idiopathic clubfoot. The final outcomes list obtained will then be discussed in a consensus meeting of representative key stakeholders. CONCLUSION: The inconsistency in outcomes reporting in studies investigating idiopathic clubfoot has made it difficult to define the success rate of treatments and to compare findings between studies. The development of a COS seeks to define a minimum standard set of outcomes to collect in all future clinical trials for this condition, to facilitate comparisons between studies and to aid decisions in treatment. Cite this article: Bone Jt Open 2021;2(4):255-260.

The Development of Leg Length Difference and Influence on Persistent Dysplasia in Patients with Developmental Dysplasia of the Hip.

INTRODUCTION: Leg-length difference (LLD) is common in patients with developmental dysplasia of the hip (DDH). LLD of > 1 cm at skeletal maturity is reported in > 40% of patients, with the majority related to ipsilateral overgrowth. A longer DDH leg might lead to excessive mechanical loading at the acetabular margin, resulting in compromised acetabular development. We hypothesised that the LLD would negatively influence acetabular development. If so, it would be advantageous to identify such patients early in the course of follow-up, and address this if necessary. METHODS: A retrospective study was conducted on a consecutive series of DDH patients managed surgically at the Royal National Orthopaedic Hospital, Stanmore, United Kingdom. We included patients with adequate long-leg radiographs at the age of 4-8 years (early-FU) and skeletal maturity (final-FU). Bilateral cases and those who underwent surgical procedures for hip dysplasia during the follow-up period were excluded. Measurements including leg length and centre-edge-angle (CE-angle) were obtained at the 2 time points. RESULTS: Twenty-seven patients were included, mean age at early-FU 5.7 (± 0.9) years, and 13.9 (± 1.0) years at final-FU. Mean LLD at early-FU was 9.5 (± 7.6) mm and 10.9 (± 9.4) mm at final-FU, p = 0.337. Correlation between early- and final-FU LLD was 0.68 (p < 0.001). The mean CE-angle at early follow-up was 14.6° (± 9.8), this improved to 23.2° (± 8.2) at skeletal maturity (p = 0.003, paired samples t-test). Linear regression analysis showed a non-significant trend towards less CE-angle improvement in patients with more initial residual dysplasia and more initial LLD. CONCLUSION: Most leg-length differences can be identified early in the follow-up period, nevertheless, considerable individual changes in LLD are observed on continued follow-up. Furthermore, a trend was observed towards impaired acetabular improvement in patients with more LLD. These findings justify careful clinical and radiological monitoring of LLD from an early stage in the follow-up period. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1007/s43465-021-00492-5.

Bone scan with SPECT/CT in children with complex foot and ankle pain: Initial experience of a paediatric tertiary referral centre.

PURPOSE: This study was designed to review the diagnostic yield of single photon emission computed tomography-computed tomography (SPECT/CT) in children with complex foot/ankle pain. METHODS: We reviewed the records of 33 patients with complex foot and ankle pain referred for SPECT/CT (36 scans) performed between 1st September 2009 to 30th September 2019. All patients had foot and ankle radiographs and 18 out of 33 patients had undergone magnetic resonance imaging (MRI) prior to SPECT/CT. The diagnostic contribution of SPECT/CT was established at the time of the scan during a multi-disciplinary team meeting. RESULTS: The patients' mean age was 13.4 years (range six to 16.5 years) and 58% were female. In total, 18 patients had undergone previous surgical treatment. SPECT/CT was found to have decisive clinical value compared with radiographs and CT/MRI in 28 out of 36 cases. In ten patients it prompted surgical management (coalition excision, arthrodesis), in seven patients it redirected conservative management, in six patients it excluded other pathology and in five patients it showed additional/unexpected focal areas of mechanical stress, thus avoiding surgery. When compared with MRI, SPECT/CT added further clinical information in 13 out of 18 cases. SPECT/CT added decisive clinical value in five out of five patients with accessory ossicles, eight out of nine patients with tarsal coalition, five out of seven patients with surgically treated Congenital Talipes Equinovarus (CTEV) and four out of five patients with neuromuscular feet. In eight out of 36 cases SPECT/CT confirmed the diagnosis without adding significant information. CONCLUSIONS: SPECT/CT can identify foci of active mechanical stress at cortical bone level in children with unexplained complex foot and ankle pain, particularly in the multiply operated foot, accessory ossicles and tarsal coalitions. LEVEL OF EVIDENCE: IV.

Providing a paediatric trauma and orthopaedics service during the peak of the COVID-19 pandemic: The Royal National Orthopaedic Hospital experience.

INTRODUCTION: In response to the COVID-19 pandemic, there was a rapidly implemented restructuring of UK healthcare services. The The Royal National Orthopaedic Hospital, Stanmore, became a central hub for the provision of trauma services for North Central/East London (NCEL) while providing a musculoskeletal tumour service for the south of England, the Midlands, and Wales and an urgent spinal service for London. This study reviews our paediatric practice over this period in order to share our experience and lessons learned. Our hospital admission pathways are described and the safety of surgical and interventional radiological procedures performed under general anaesthesia (GA) with regards to COVID-19 in a paediatric population are evaluated. METHODS: All paediatric patients (≤ 16 years) treated in our institution during the six-week peak period of the pandemic were included. Prospective data for all paediatric trauma and urgent elective admissions and retrospective data for all sarcoma admissions were collected. Telephone interviews were conducted with all patients and families to assess COVID-19 related morbidity at 14 days post-discharge. RESULTS: Overall, 100 children underwent surgery or interventional radiological procedures under GA between 20 March and 8 May 2020. There were 35 trauma cases, 20 urgent elective orthopaedic cases, two spinal emergency cases, 25 admissions for interventional radiology procedures, and 18 tumour cases. 78% of trauma cases were performed within 24 hours of referral. In the 97% who responded at two weeks following discharge, there were no cases of symptomatic COVID-19 in any patient or member of their households. CONCLUSION: Despite the extensive restructuring of services and the widespread concerns over the surgical and anaesthetic management of paediatric patients during this period, we treated 100 asymptomatic patients across different orthopaedic subspecialties without apparent COVID-19 or unexpected respiratory complications in the early postoperative period. The data provides assurance for health care professionals and families and informs the consenting process.Cite this article: Bone Joint Open 2020;1-6:287-292.

A systematic review of reported outcomes following Ponseti correction of idiopathic club foot.

AIMS: To analyze outcomes reported in studies of Ponseti correction of idiopathic clubfoot. METHODS: A systematic review of the literature was performed to identify a list of outcomes and outcome tools reported in the literature. A total of 865 studies were screened following Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines, and 124 trials were included in the analysis. Data extraction was completed by two researchers for each trial. Each outcome tool was assigned to one of the five core areas defined by the Outcome Measures Recommended for use in Randomized Clinical Trials (OMERACT). Bias assessment was not deemed necessary for the purpose of this paper. RESULTS: In total, 20 isolated outcomes and 16 outcome tools were identified representing five OMERACT domains. Most outcome tools were appropriately designed for children of walking age but have not been embraced in the literature. The most commonly reported isolated outcomes are subjective and qualitative. The quantitative outcomes most commonly used are ankle range of motion (ROM), foot position in standing, and muscle function. CONCLUSIONS: There is a diverse range of outcomes reported in studies of Ponseti correction of clubfoot. Until outcomes can be reported unequivocally and consistently, research in this area will be limited. Completing the process of establishing and validating COS is the much-needed next step.Cite this article: Bone Joint Open 2020;1-8:457-464.

Is Research in Simulation as Accessible as Traditional Clinical Research? A Review of the 'Association for Simulated Practice in Healthcare' Conference.

Background Meta-analysis of simulation teaching has shown to be an effective teaching methodology. The Association for Simulated Practice in Healthcare (ASPIH) annual international, multidisciplinary conference is recognised as the leading UK meeting for simulation-based education. We hypothesise that simulation-based research presented at this conference is currently less accessible than more traditional clinical research presentations. Method We reviewed the abstracts of all research presented at the 5th ASPIH Conference, 2014 and then utilised the Bhandari methodology to assess whether an abstract had subsequently been published in a peer review journal. Our secondary aim was to assess for recurring themes that may predict publication. Results Twenty-seven of 197 (14%) abstracts presented at the 2014 meeting were subsequently published. The mean lead time to publication from the conference was 23 (2 - 61) months. Two positive predictive factors for publication were oral presentations (vs poster), and a Kirkpatrick level above 1. Conclusion The publication rate for abstracts from respected clinical conferences is 30%, but the publication rate for ASPIH abstracts is significantly below this. The potential reasons for this may include a lack of simulation specific journals. Authors should aim to publish simulation-based research in peer reviewed publications to help progress the role and the value of simulation in medical education.

Clinical practice recommendations for the diagnosis and management of X-linked hypophosphataemia.

X-linked hypophosphataemia (XLH) is the most common cause of inherited phosphate wasting and is associated with severe complications such as rickets, lower limb deformities, pain, poor mineralization of the teeth and disproportionate short stature in children as well as hyperparathyroidism, osteomalacia, enthesopathies, osteoarthritis and pseudofractures in adults. The characteristics and severity of XLH vary between patients. Because of its rarity, the diagnosis and specific treatment of XLH are frequently delayed, which has a detrimental effect on patient outcomes. In this Evidence-Based Guideline, we recommend that the diagnosis of XLH is based on signs of rickets and/or osteomalacia in association with hypophosphataemia and renal phosphate wasting in the absence of vitamin D or calcium deficiency. Whenever possible, the diagnosis should be confirmed by molecular genetic analysis or measurement of levels of fibroblast growth factor 23 (FGF23) before treatment. Owing to the multisystemic nature of the disease, patients should be seen regularly by multidisciplinary teams organized by a metabolic bone disease expert. In this article, we summarize the current evidence and provide recommendations on features of the disease, including new treatment modalities, to improve knowledge and provide guidance for diagnosis and multidisciplinary care.

Treatment of thoracolumbar kyphosis in patients with mucopolysaccharidosis type I: results of an international consensus procedure.

BACKGROUND: In all patients with mucopolysaccharidosis type I (MPS I), skeletal disease (dysostosis multiplex) is a prominent, debilitating, condition related complication that may impact strongly on activities of daily living. Unfortunately, it is not alleviated by treatment with hematopoietic cell transplantation (HCT) or enzyme replacement therapy (ERT). Although early kyphosis is one of the key features of dysostosis multiplex, there is no international consensus on the optimal management. Therefore, an international consensus procedure was organized with the aim to develop the first clinical practice guideline for the management of thoracolumbar kyphosis in MPS I patients. METHODS: A literature review was conducted to identify all available information about kyphosis and related surgery in MPS I patients. Subsequently, a modified Delphi procedure was used to develop consensus statements. The expert panel included 10 spinal orthopedic surgeons, 6 pediatricians and 3 physiotherapists, all experienced in MPS I. The procedure consisted of 2 written rounds, a face-to-face meeting and a final written round. The first 2 rounds contained case histories, general questions and draft statements. During the face-to-face meeting consensus statements were developed. In the final round, the panel had the opportunity to anonymously express their opinion about the proposed statements. RESULTS: Eighteen case series and case reports were retrieved from literature reporting on different surgical approaches and timing of thoracolumbar kyphosis surgery in MPS I. During the face-to-face meeting 16 statements were discussed and revised. Consensus was reached on all statements. CONCLUSION: This international consensus procedure resulted in the first clinical practice guideline for the management of thoracolumbar kyphosis in MPS I patients, focusing on the goals and timing of surgery, as well as the optimal surgical approach, the utility of bracing and required additional assessments (e.g. radiographs). Most importantly, it was concluded that the decision for surgery depends not only on the kyphotic angle, but also on additional factors such as the progression of the deformity and its flexibility, the presence of symptoms, growth potential and comorbidities. The eventual goal of treatment is the maintenance or improvement of quality of life. Further international collaborative research related to long-term outcome of kyphosis surgery in MPS I is essential as prognostic information is lacking.

Bilateral Developmental Dysplasia of the Hip: Does Closed Reduction Have a Role in Management? Outcome of Closed and Open Reduction in 92 Hips.

BACKGROUND: Bilateral developmental dysplasia of the hip (DDH) is believed to have a worse outcome than unilateral DDH with the optimal treatment unclear. To define indications for treatment we report a retrospective series of 92 hips (46 patients) who underwent closed reduction (CR) and/or open reduction (OR). METHODS: A total of 58 hips in 29 patients underwent attempted CR using our standardized protocol. In the same 12 year time period, 54 hips in 27 patients underwent an OR. Outcome measures included resolution of acetabular dysplasia, need for further surgery, development of osteonecrosis (ON), and modified Severin Grade. RESULTS: CR was successful in 57% of hips; in this group secondary surgery was required in 15% and ON was seen in 12%. In the OR group, 11% failed to stabilize and required further early surgery: ON rate was 15% overall, and secondary surgery was required in 5%. In Tonnis 2/3 hips younger than 2 years, Severin grading was comparable following CR and OR, with 92% and 90% graded as Severin 1, respectively. The rate of significant ON was higher after CR in Tonnis 2/3 hips (12%) than after OR (0%). Overall, Tonnis 4 hips did badly: 94% failed CR and following OR, further surgery for redislocation/residual dysplasia was required in 21%. CONCLUSIONS: CR can be successful in Tonnis 2/3 bilateral hips, with acceptable rates of secondary surgery, ON, and Severin grading, the latter equivalent to open reduction. Importantly, persisting with casting of a unilateral dislocation, to allow the contra-lateral hip to stabilize, does not have an adverse effect. CR is not advised in Tonnis 4 bilateral hips. Parents should be counseled that the outcome of surgery for bilateral hips is not as good as for unilateral DDH, particularly for Tonnis 4 hips which are more difficult to stabilize and more likely to require supplementary surgery even after open reduction. LEVEL OF EVIDENCE: Level IV-Therapeutic Study.