Morquio B disease: a case report.

Mucopolysaccharidosis IV type B, or Morquio B disease (MBD), is an autosomal recessive disorder caused by a genetic mutation in GLB1 gene encoding for ß-galactosidase on chromosome 3p22.33. ß-galactosidase deficiency can result in two different conditions, GM1 gangliosidosis and MBD, of which MBD has a milder phenotype and presents later in life with keratan sulfate accumulation in the retina and cartilage. In this case report, we present a patient diagnosed with MBD at the age of 5 after initially presenting with Morquio dysostosis multiplex and characteristic radiographic findings. Genetic testing confirmed that the patient has ß-galactosidase deficiency due to mutation W273l/N484K on GLB1 gene. The patient exhibited elevated mucopolysaccharide levels in urine at 18 mg/mmol and demonstrated an abnormal band pattern of urine oligosaccharides on electrophoresis. The activity of ß-galactosidase in his white blood cells was reduced to 12.3 nmol/h/mg protein. At the time of diagnosis, the patient did not present with gait and ambulation issues, but his ability to walk progressively deteriorated in his adolescence as a result of instability and pain in the ankle, knee, and hip joints, accompanied by a global decrease in muscle strength. This case report is the first in the literature to provide an in-depth exploration of the orthopedic treatment and follow-up received by a young adolescent with MBD to provide symptom relief and improve walking ability.

Fully Automated Analysis of the Anatomic and Mechanical Axes From Pediatric Standing Lower Limb Radiographs Using Convolutional Neural Networks.

BACKGROUND: Lower limb alignment is the quantification of a set of parameters that are commonly measured radiographically to test for and track a wide range of skeletal pathologies. Determining limb alignment is a commonly performed yet laborious task in the pediatric orthopaedic setting and is therefore an interesting goal for automation. METHODS: We employ a machine learning approach using convolutional neural networks (CNNs) to segment pediatric weight-bearing lower limb radiographs. The results are then used with custom Matlab code to extract anatomic landmarks and to determine lower limb alignment parameters. RESULTS: Measurements obtained from the automated workflow proposed here were compared with manual measurements performed by orthopaedic surgery fellows. Mechanical axis deviation was determined within a mean of 2.02 mm. Lateral distal femoral angle and medial proximal tibial angle were determined with a mean deviation of 1.73 and 2.90 degrees, respectively. The calculation speed for the full set of mechanical and anatomic axis parameters was found to be ~2 seconds per radiograph. CONCLUSIONS: The CNN-based approach proposed in this work was shown to produce results comparable to orthopaedic surgery fellows at fast calculation speed. Although further work is needed to validate these results against radiographs and measurements from other centers, we see this as a promising start and a functional path that can be employed in further research. CLINICAL RELEVANCE: CNNs are a promising approach to automating commonly performed, repetitive tasks, especially those pertaining to image processing. The time savings are particularly important in clinical research applications where large sets of radiographs are routinely available and require analysis. With further development of these algorithms, we anticipate significantly improved agreement with expert-measured results and the calculation speed.

Inter- and Intra-rater Reliability of the Checketts' Grading System for Pin-site Infections across All Skin Colours.

The Checketts' grading system (CGS) is the only classification that provides both a description of how to visually grade the infection and the appropriate course of treatment. There are no studies on the reliability of this system nor on whether skin colour can influence applicability. This study aims to determine the inter-rater and intra-rater reliability of the CGS to assess whether this scale could be used as a universal grading system across all skin colours. A survey consisting of 134 anonymised photographs of pin-site infections was sent out to orthopaedic surgeons specialising in limb lengthening and reconstruction and to patients or carers of individuals who had external fixators. For each photograph, the participants were asked to grade the infection using the CGS, rate their confidence in their chosen grade on a Likert scale and assign a treatment option. The participants were supplied with the CGS at the beginning of the survey, after the 45th and 90th photographs. The inter-rater reliability of the CGS between the surgeons, expressed as an intraclass correlation coefficient (ICC), was poor-to-moderate at both time points (ICC = 0.56 for baseline survey and ICC = 0.48 for follow-up). This was similar for the patient or caretaker group. There was a lower inter-rater reliability for grading of dark skin as opposed to light skin by surgeons but not for patients or caretakers. The inter-rater reliability of treatment decisions between the surgeons was poor at both time points (kappa = 0.30 and 0.22) with similar inter-rater reliability for dark (kappa = 0.26 and 0.23) compared with light skin (kappa = 0.29 and 2.6). This was similar for the patient or caretaker group. The surgeons' confidence (Table 4) in grading was low (median = 1). The patient or caretaker group's confidence in their grading was modest (median = 2). The reliability of the CGS as assessed here demonstrates poor-to-moderate inter-rater reliability which makes interpretation of published pin site infection rates using this scale difficult. The design of new grading systems will need to consider skin colour to reduce inequities in medical decision-making. How to cite this article: Groenewoud R, Chhina H, Bone J, et al. Inter- and Intra-rater Reliability of the Checketts' Grading System for Pin Site Infections across All Skin Colours. Strategies Trauma Limb Reconstr 2023;18(1):2-6.

Danish Linguistic Validation and Cultural Adaptation of the LIMB-Q Kids.

(1) Background: Lower Limb deformities include many conditions where pain and physical limitations negatively impact the health-related quality of life (HRQL) of children. The ideal way to assess this impact is by using a Patient Reported Outcome Measure (PROM). Such a condition-specific PROM is under development, called LIMB-Q Kids. This study aimed to perform a translation and cultural adaption (TCA) of the LIMB-Q Kids for use in Danish-speaking children. (2) Methods: TCA guidelines established by the World Health Organization (WHO) and the Professional Society for Health Economics and Outcomes Research (ISPOR) were followed. This process consisted of two independent forward translations, a reconciliation meeting, a backward translation with an assessment, an expert meeting, cognitive interviews with patients, editing based on the interviews, and proofreading. (3) Results: The TCA process contributed to the Danish version of LIMB-Q Kids. The reconciliation meeting resulted in a reconciled Danish version. The revision of the backward translation led to 16 corrections, and after the expert panel meeting, 26 changes were made. Twelve cognitive interviews led to nine changes, which were validated by two further interviews. Proofreading led to no further comments. (4) Conclusions: The TCA process led to a linguistically validated and culturally adapted Danish version of LIMB-Q Kids. This version is being used in the international field test study.

Early Investigation and Bracing in DDH Impacts Maternal Wellbeing and Breastfeeding.

BACKGROUND: The outcomes of Pavlik Harness (PH) management for Developmental Dysplasia of the Hip (DDH) are equivalent regardless of the initiation timing, if it is within the first 6 weeks of life. A PH may be a physical barrier to breastfeeding, which is important for nutrition, immunity, and normal child development. The diagnosis of DDH and early management with a PH may also negatively affect the maternal psychosocial wellbeing and the infant-maternal relationship. The purpose of this study is to investigate the impact of the diagnosis of DDH and the management with a PH has on maternal wellbeing and maintenance of breastfeeding, compared with being screened for but not diagnosed with DDH. METHODS: A retrospective cohort of the mothers of infants who were diagnosed with DDH and treated with a PH brace was compared with the mothers of infants who were screened for DDH only. The Hip Worries Inventory and Edinburgh Postnatal Depression Scale were completed by the mothers in both groups. The PH group also completed an in-house questionnaire specific to PH and breastfeeding. RESULTS: Eighty completed surveys were included, 50 from the treatment group. The mean age of the PH initiation was 6.2 weeks. The modified Hip Worries Inventory score was higher in the treatment group, with a mean difference (MD) of 9.7 out of 50 (95% confidence interval, CI, 6.8, 12.5). The MD of the Edinburgh Postnatal Depression Scale was 2.0 out of 30 (CI -0.5, 4.5). Although there was no difference in the breastfeeding ease before and after the PH initiation (MD-0.2, CI-0.7, 0.2), 83% of mothers found breastfeeding more difficult with a PH and 11% of mothers stopped breastfeeding earlier than planned because of the PH. CONCLUSIONS: Mothers of infants with DDH worry more about their child's hips and the PH. Screening alone may contribute to maternal psychological dejection and negative thoughts. The presence of a PH makes breastfeeding more difficult. LEVEL OF EVIDENCE: Retrospective comparative study, level III.

Multimodal Analgesia for Paediatric Patients Undergoing Lower Limb Reconstruction with External Fixators: A Prospective Case Series of Post-operative Pain and Functional Goals.

Aim: Limb reconstruction with external fixators requires appropriate pain management to promote effective analgesia and healing while minimising adverse events of the analgesic technique used. The objective of this prospective case series was to evaluate a multimodal analgesia regimen designed to reduce opioid requirements and hence reduce the opioid-related side effect profile. Materials and methods: A prospective cohort of patients undergoing lower limb reconstruction surgery (LRS) were managed through an evidence-informed multimodal analgesia guideline (MMAG), including acetaminophen, pregabalin, dexmedetomidine, IV lidocaine, and opioids. Outcome measures included intraoperative and post-operative opioid administration, post-operative pain scores, time to achieve mobilisation milestones, and post-operative complications. Surveys were conducted to obtain patient reported experiences. Results: 26 patients were included in this prospective case series. 110.59 (84.29, 162.13) (median, interquartile range) µg/kg/hr intraoperative IV morphine equivalent opioids were administered. In the first 48 hours post-operatively, patients received 11.49 (6.41, 19.35) µg/kg/hr of IV morphine equivalent dose. Median level of pain (0-10) in the first 48 post-operative hours was 2 (1, 2). Patients achieved mobilisation. And 19/20 patients surveyed reported 'yes' to having effective pain management; 17/20 patients had no unwanted side effects associated with analgesia medications. There were no cases of compartment syndrome. Conclusion: This multimodal analgesia regime applied to patients undergoing lower LRS with external fixators demonstrates the feasibility of this analgesic regimen which revealed effective pain control, early mobilisation, with minimal side effects, but warrants further study. Clinical significance: This study provides valuable evidence that this standardised multimodal anaesthesia and analgesia regimen is feasible, offers adequate post-operative comfort and encourages early mobilization while minimising opioid use and adverse events in a paediatric LRS population at our institution. How to cite this article: Wang AWT, Chhina H, Cooper A. Multimodal Analgesia for Paediatric Patients Undergoing Lower Limb Reconstruction with External Fixators: A Prospective Case Series of Post-operative Pain and Functional Goals. Strategies Trauma Limb Reconstr 2023;18(3):140-147.

LIMB-Q Kids-German Translation and Cultural Adaptation.

(1) Purpose: Lower limb deformities can have a severe impact on health-related quality of life (HRQL). LIMB-Q Kids is a new patient-reported outcome measure (PROM) aiming to elucidate the experience of 8-18-year-old patients before, during and after treatment, and to measure the different aspects of HRQL. The aim of this study was to translate and culturally adapt LIMB-Q Kids to German. (2) Methods: The International Society for Pharmacoeconomics and Outcomes Research (ISPOR) guidelines were followed. Three forward translations, a backward translation, an expert panel meeting with eight participants, and twenty cognitive debriefing interviews led to the final German version of LIMB-Q Kids. (3) Results: In the forward translations, 4/159 items were difficult to translate, and 2/159 items in the backward translation differed from the original English version. Cognitive debriefing interviews with 20 patients identified 7/159 items that were difficult to comprehend/answer, and 2 of these items were changed. (4) Conclusions: Lower limb deformities can have a great impact on children, and it is important to measure and consider the impact on HRQL. In order to be able to use PROMs in different countries, conceptually equivalent translations and cultural adaptations should be performed in order to ensure comprehensibility. The final German version of LIMB-Q Kids is ready for use in an international field test.

Efficacy and Safety of "Sleeper Plate" in Temporary Hemiepiphysiodesis and the Observation of "Tethering".

BACKGROUND: Guided growth is commonly performed by placing an extraperiosteal 2-hole plate across the growth plate with one epiphyseal and one metaphyseal screw. Recent studies investigated the efficacy of the removal of the metaphyseal screw only (sleeper plate) after correction. They concluded the practice to be unnecessary as only 19% of patients showed recurrence of deformity. This study aims to examine the incidence of rebound and undesired bony in-growth of the plate (tethering) after metaphyseal screw removal only. METHODS: In this retrospective case series, patient data on 144 plates inserted around the knee were obtained. Plates still in situ (n=69) at the time of study and full hardware removal (n=50) were excluded. The remaining 25 plates had only the metaphyseal screw removed after completed deformity correction. We analyzed the rate of tethering, rebound, and maintenance of correction in 2 age groups at latest follow (mean of 3.5 y). The Fisher exact test with Freeman-Halton extension was used to analyze categorical data and the Student t test for descriptive variables. RESULTS: Twenty-five plates were identified as "sleeper plates" in our series. Thirteen plates (52%) maintained the achieved correction after a mean of 21 months (range: 4 to 39 mo), 9 plates (36%) required screw reinsertion due to rebound after a mean of 22 months (range: 12 to 48 mo) from screw removal, and 4 plates (16%) showed tethering with undesired continuation of guided growth after a mean of 14 months (range: 7 to 22 mo) from screw removal. Younger patients (<8 y at time of plate insertion) had higher rates of rebound and tethering ( P =0.0112, Fisher exact test). All tethering occurred in titanium plates, none occurred in steel plates. CONCLUSIONS: The sleeper plate is an acceptable treatment strategy for coronal deformities around the knee, however, tethering and rebound may occur, especially in younger patients. Titanium plates may increase the risk of tethering, however, further long-term follow-up is needed as there were only 6 steel plates versus 19 titanium in this study. We stress the importance of close postoperative follow-up to identify signs of tethering and rebound early to prevent over-correction. LEVEL OF EVIDENCE: Level IV-retrospective case study.

Establishing content validity of LIMB-Q Kids: a new patient-reported outcome measure for lower limb deformities.

PURPOSE: To establish the content validity of LIMB-Q Kids, a new patient-reported outcome measure (PROM) for children with lower limb deformities. This study focused on three key components of content validity, i.e., comprehension, comprehensiveness, and relevance. METHODS: Cognitive debriefing interviews (CDIs) with children with lower limb deformities were performed, and expert input from parents and clinicians directly involved in the care of children with lower limb deformities was obtained. CDIs were conducted with children from sites in Australia, Canada, and the USA. All interviews were recorded and transcribed verbatim. Analyses were conducted using the reparative approach, which involved close examination of the transcribed interviews and summarising edits after each interview. RESULTS: Forty patients, experts, and parents reviewed LIMB-Q Kids and provided feedback. At the start, LIMB-Q Kids had 10 scales and 124 total items. Five rounds of input was obtained from children, parents, and experts. Overall, 37 new items were added. Thirty-three of the new items were included to measure symptoms experienced in different parts of the legs. Final version of LIMB-Q Kids had 11 scales and 159 items. CONCLUSION: This study established content validity of the LIMB-Q Kids, a new PROM for children with lower limb deformities. An international field-test study is underway. Translation and cultural adaptation are underway for sites where English is not the first language. Scoring algorithms will be developed, following which the scale could be used to inform clinical practice and research.

Paediatric elbow fractures and public play spaces: adherence to standards for children's playground equipment and surfacing.

Background: Supracondylar humerus fractures (SCHF) are the most common fractures sustained following a fall onto an outstretched hand among healthy children, and one of the leading causes of hospital admission and surgical intervention. The aim of this study was to examine SCHF occurring at public play spaces-particularly to determine whether or not the playground equipment implicated in injurious falls aligned with Canadian playground safety standards. Methods: Cases of children who attended the provincial paediatric orthopaedic clinic following SCHF at a public playground between April 2017 and October 2019 were included in the study. A research assistant visited each playground to measure the play structure type and dimensions, height of the equipment at the point from which the child fell and the type and depth of the surface material, and compare measurements to the 2016 safety standards. Child demographics and injury classification were also noted. Descriptive statistics were calculated and a scatterplot of fall height and surface depth was generated. Results: Forty-three sites, representing 47 SCHF cases (18 female, 29 male), were included in the final analysis. Fourteen children sustained type 1 fracture, 23 had type 2 fracture and the remaining 10 had type 3 fracture. Five children with type 2 fracture and all 10 children with type 3 fracture required surgery. The majority of sites had engineered wood fibre surfacing, with surfacing at 35 sites being less than 300 mm deep. Twenty-six play structures were upper body equipment (ie, monkey bars or similar), seven were track rides, five were rotating structures and the rest comprised a variety of classified and unclassified structures. Twenty-seven children fell from a height exceeding 2 m. Conclusions: The majority of SCHF cases occurred at playgrounds with insufficient surface depth and/or non-compliant equipment. Upper body equipment, track rides and rotating play structures were of particular concern, as the children fell from heights exceeding the recommended standard, likely reflecting the degradation and compaction of the surfacing material over time.

Clinical outcomes in pediatric tibial lengthening and deformity correction: a comparison of the Taylor Spatial Frame with the Orthex Hexapod System.

PURPOSE: Comparison of two hexapod frame systems in paediatric tibial deformity correction; the Taylor Spatial Frame (TSF) and Orthex Hexapod System. METHODS: Paediatric patients with congenital and acquired tibial deformities treated with either TSF (between 2014 and 2016) or Orthex (between 2017 and 2019) frames were included in a retrospective comparative study. Outcome measures were healing index, pin infection rate, regenerate quality and density, software residual rate, deformity correction accuracy, strut exchanges and quality of life (QoL). RESULTS: The TSF group had 17 patients (18 frames) and the Orthex group had 21 patients (25 frames). The most common indications for tibial deformity correction were fibular hemimelia (14) and septic or traumatic growth arrest (8). The median time in frame was 230 days (TSF) versus 203 days (Orthex) (p= 0.06). The mean lengthening achieved was 54 mm (TSF) and 51 mm (Orthex) (p = 0.41). The healing index was 41 days/cm (TSF) versus 43 days/cm (Orthex) (p = 0.70). Pin site infections occurred more in the TSF cohort (40%) than in the Orthex cohort (18%) (p < 0.001). The regenerate in the Orthex group showed higher density at three months (p = 0.029) and was more homogenous (p = 0.023) at six months after frame application. Strut exchanges were less frequent with the Orthex system (p < 0.0001). QoL measures were similar in both cohorts (p = 0.92). CONCLUSIONS: This is the first study to compare two hexapod designs in paediatric orthopaedics. The Orthex system showed superiority in regenerate quality and a significant reduction in pin site infection rates. Both systems delivered predictable and accurate limb deformity correction. LEVEL OF EVIDENCE: III.

What matters to children with lower limb deformities: an international qualitative study guiding the development of a new patient-reported outcome measure.

BACKGROUND: Lower limb deformities include conditions such as leg length discrepancy, lower limb deficiency and associated angular and rotational deformities of the hips, knees, ankles and feet. Children with lower limb deformities often have physical limitations due to gait irregularities and pain. The differences in the appearance and function of their lower limbs can discourage participation in social, recreational and leisure activities, which may result in behavioural, emotional, psychological and social adjustment problems. The health-related quality of life (HRQL) of these children is often impacted due to the factors discussed above, as well as by the complex surgical procedures. Surgical treatment options for limb deformities in children vary from limb lengthening and reconstruction to amputation. The lack of evidence demonstrating superiority of either treatment options and their effect on HRQL limits the ability of healthcare providers to counsel families on the best evidence-based treatment option for them. This manuscript describes the international qualitative study which guided the development of a new patient-reported outcome measure (PROM). Individual semi-structured face-to-face interviews with children with lower limb deformities and their parents were conducted at five sites: Canada (2 sites), Ethiopia, India and the USA. RESULTS: Seventy-nine interviews were conducted at five international sites. Five main themes emerged from the qualitative interviews and formed the basis of the conceptual framework. These themes were: 1) appearance, 2) physical health, 3) psychological health 4) school and 5) social health. CONCLUSIONS: Lower limb deformities have a substantial impact on the HRQL of children. The concepts of interest identified in our study were similar across children from all countries. The conceptual framework guided the development of outcome scales specific to these patients. The information about the impact of various treatment options on the HRQL of children with lower limb deformities, collected using this new PROM, could be used to inform parents and children about outcomes (physical, social, psychological) associated with specific treatment options. This information could supplement other objective outcome information (e.g., complication rates, how the leg will look, etc.) to help families to come to a more informed decision on a child's course of treatment.

Dynamic deformation of the femoral head occurs on weightbearing in Legg-Calves-Perthes disease: a translational pilot study.

AIMS: Though the pathogenesis of Legg-Calve-Perthes disease (LCPD) is unknown, repetitive microtrauma resulting in deformity has been postulated. The purpose of this study is to trial a novel upright MRI scanner, to determine whether any deformation occurs in femoral heads affected by LCPD with weightbearing. METHODS: Children affected by LCPD were recruited for analysis. Children received both standing weightbearing and supine scans in the MROpen upright MRI scanner, for coronal T1 GFE sequences, both hips in field of view. Parameters of femoral head height, width, and lateral extrusion of affected and unaffected hips were assessed by two independent raters, repeated at a one month interval. Inter- and intraclass correlation coefficients were determined. Standing and supine measurements were compared for each femoral head. RESULTS: Following rigorous protocol development in healthy age-matched volunteers, successful scanning was performed in 11 LCPD-affected hips in nine children, with seven unaffected hips therefore available for comparison. Five hips were in early stage (1 and 2) and six were in late stage (3 and 4). The mean age was 5.3 years. All hips in early-stage LCPD demonstrated dynamic deformity on weightbearing. Femoral head height decreased (mean 1.2 mm, 12.4% decrease), width increased (mean 2.5 mm, 7.2% increase), and lateral extrusion increased (median 2.5 mm, 23% increase) on standing weightbearing MRI compared to supine scans. Negligible deformation was observed in contra-lateral unaffected hips, with less deformation observed in late-stage hips. Inter- and intraclass reliability for all measured parameters was good to excellent. CONCLUSION: This pilot study has described an effective novel research investigation for children with LCPD. Femoral heads in early-stage LCPD demonstrated dynamic deformity on weightbearing not previously seen, while unaffected hips did not. Expansion of this protocol will allow further translational study into the effects of loading hips with LCPD.Cite this article: Bone Joint Open 2020;1-7:364-369.

Methodological Challenges in Investigating Supracondylar Fractures of the Humerus From a Child's Viewpoint: Evolution of Study Protocol.

BACKGROUND: Outdoor play and risk-taking behaviors, including play at heights, are important to children's physical, social, and cognitive development. These aspects of play are important to consider when informing prevention policies for serious injuries that commonly occur on play structures. Supracondylar fractures of the humerus (SCH) are the most common type of elbow fractures that result from falls on an outstretched hand among healthy children. Despite being one of the leading causes of admission to the hospital and surgical intervention, the details surrounding the cause of these injuries are often not recorded. Previous research has correlated decreased overall playground safety with higher rates of SCH fractures. Play structure height and the type of undersurface have been identified as potential risk factors for severe injuries, including SCH fractures, in part due to low compliance with safety standards. This paper explores the challenges we encountered designing the study and the resulting insights and methodological modifications we made. OBJECTIVE: The aim of this paper is to discuss the challenges related specifically to clinical research in pediatrics and strategies developed to conduct a study that prioritizes the engagement and perspective of children and their families. METHODS: To explore the link between the severity of SCH fractures and children's behavioral, environmental, and mechanistic factors, we conducted a mixed-methods study. RESULTS: During phase 1 (the original methodology) from April 2017 to July 2018, there were 58 eligible study participants and 17 were recruited. For phase 2 (the revised methodology) between October 2018 and October 2019, there were 116 eligible participants and 47 were recruited. CONCLUSIONS: The changes in methodology made following the first phase of data collection were effective in our ability to recruit participants. By identifying and addressing challenges pertaining to recruitment and resource limitations, we were able to collect data in a concise manner while not compromising the quality of the data and make for an easily adoptable methodology for other sites interested in participating in the study. We hope that future studies that plan to employ a similar methodology can gain insight through the methodological challenges we have encountered and the way we adapted the methodology to build a more pragmatic approach. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/21816.

Modernization of bone age assessment: comparing the accuracy and reliability of an artificial intelligence algorithm and shorthand bone age to Greulich and Pyle.

Greulich and Pyle (GP) is one of the most common methods to determine bone age from hand radiographs. In recent years, new methods were developed to increase the efficiency in bone age analysis like the shorthand bone age (SBA) and automated artificial intelligence algorithms. OBJECTIVE: The aim of this study is to evaluate the accuracy and reliability of these two methods and examine if the reduction in analysis time compromises their efficacy. METHODS: Two hundred thirteen males and 213 females had their bone age determined by two separate raters using the SBA and GP methods. Three weeks later, the two raters repeated the analysis of the radiographs. The raters timed themselves using an online stopwatch. De-identified radiographs were securely uploaded to an automated algorithm developed by a group of radiologists in Toronto. The gold standard was determined to be the radiology report attached to each radiograph, written by experienced radiologists using GP. RESULTS: Intraclass correlation between each method and the gold standard fell within the range of 0.8-0.9, highlighting significant agreement. Most of the comparisons showed a statistically significant difference between the new methods and the gold standard; however, it may not be clinically significant as it ranges between 0.25 and 0.5 years. A bone age is considered clinically abnormal if it falls outside 2 standard deviations of the chronological age; standard deviations are calculated and provided in GP atlas. CONCLUSION: The shorthand bone age method and the automated algorithm produced values that are in agreement with the gold standard while reducing analysis time.

Support, information, and integration of genetics for children with congenital lower limb deficiencies in British Columbia, Canada.

OBJECTIVES: Children and families affected by congenital limb deficiencies (CLD) require a unique level of emotional support from diagnosis through to adolescence. The following study aims to collect data on Canadian paediatric patients affected by a CLD followed at BC Children's Hospital (BCCH), Department of Orthopaedics. METHODS: Parents of children with a CLD were asked to complete a written questionnaire examining their experiences. Qualitative and quantitative data were collected concerning parent satisfaction with patient referrals, emotional support, and knowledge of their child's diagnosis. RESULTS: Twenty-five completed questionnaires were returned. Fifty per cent of the parents reported they were either very satisfied, or satisfied, with the emotional support provided by health care providers (HCPs). Twenty-five per cent of the parents were unsatisfied with the emotional support received by HCPs. Forty-eight per cent of the parents could not recall the specific name of their child's diagnosis; 20% of the parents reported their child did not have diagnosis. All the patients in our study had received a clinical diagnosis. Twenty-eight per cent of the parents in this study were also seen in medical genetics. CONCLUSIONS: Families require additional resources for emotional support, peer support, and referrals to support organizations. Gaps in parent knowledge regarding their child's CLD suggest the need for formalized communication strategies for HCPs. Furthermore, patients with CLDs and their families may benefit from improved communication between orthopaedic and medical genetic services at the time of diagnosis. Integration of genetic counsellors may improve emotional supports and education for families with regards to testing and reproductive planning.

International multiphase mixed methods study protocol to develop a patient-reported outcome instrument for children and adolescents with lower limb deformities.

INTRODUCTION: Our recent systematic review has indicated the lack of a patient-reported outcome (PRO) instrument to measure health-related quality of life (HRQOL) of children and adolescents with lower limb deformities. We are developing a PRO instrument which will be applicable internationally across various countries. This manuscript describes our approach to the development of a new PRO instrument for measuring HRQOL for children and adolescents with lower limb deformities. METHODS AND ANALYSIS: Three phases in the development of this PRO instrument are as described: (1) This phase involves the development of a conceptual framework of HRQOL and item pool that is used to inform a set of preliminary scales. We have developed a preliminary conceptual framework of HRQOL based on our systematic review. Qualitative interviews are being conducted at five sites in Canada, Ethiopia, India and the USA. An item pool will be generated from this qualitative phase. The preliminary items and scales will be sent out to children at the five participating centres. Cognitive debriefing interviews will gather detailed feedback on the items from the children. Expert opinion will be sought from clinicians from the participating centres. (2) During this phase, an international field-test study will be conducted to refine the scales and examine their psychometric properties. (3) During this phase, tests of reliability, validity and responsiveness will be conducted. Phase 1 will also involve translations and cultural adaptations. At the end of this study, we expect to produce an internationally applicable PRO instrument which is scientifically sound and clinically relevant to the lower limb deformity population. ETHICS AND DISSEMINATION: This study is approved by Research Ethics Boards for each of the participating sites.Results of this study will be published in peer-reviewed journals and presented at national and international conferences. An integrated knowledge translation approach is applied to engage patients, families and clinicians from the start of the study.

Botulinum Toxin Type A Versus Placebo for Idiopathic Clubfoot: A Two-Center, Double-Blind, Randomized Controlled Trial.

BACKGROUND: Congenital idiopathic clubfoot is a condition that affects, on average, approximately 1 in 1,000 infants. One broadly adopted method of management, described by Ponseti, is the performance of a percutaneous complete tenotomy when hindfoot stall occurs. The use of onabotulinum toxin A (BTX-A) along with the manipulation and cast protocol described by Ponseti has been previously reported. Our goal was to compare the clinical outcomes between BTX-A and placebo injections into the gastrocnemius-soleus muscle at the time of hindfoot stall in infants with idiopathic clubfoot treated with the Ponseti method of manipulation and cast changes. METHODS: This was a double-blind, placebo-controlled, parallel-group study with balanced randomization. RESULTS: At 6 weeks after the study injection (T1), 66% of the 32 feet in the BTX-A arm and 63% of the 30 in the placebo arm responded to the treatment (i.e., obtained ≥15° of dorsiflexion). Seven of the 11 patients in the BTX-A arm and all of the 11 in the placebo arm who had not responded at T1 responded to a rescue BTX-A injection at 12 weeks after the first injection (T2). The combined response rate at T2, which included the first-time responders as well as the patients who did not respond at T1 but did at T2, was 88% in the BTX-A arm and 100% in the placebo arm, culminating in a 94% response rate at T2. At T3 (2 years of age), 89% of the feet continued to respond and there was an 8% surgical rate. CONCLUSIONS: There was no difference in outcomes between the BTX-A and placebo groups when the injection was performed at the time of hindfoot stall. Overall, 92% of the clubfeet in this study responded to a manipulation and cast protocol alone, with or without BTX-A injection, by 12 weeks after hindfoot stall, or we can say that 92% of the clubfeet did not require percutaneous Achilles tendon lengthening by 2 years of age. The need for tenotomy is limited to those who have not responded to treatment at this point, and the need for surgery is limited to those for whom all attempts at treatment with sequential casts, BTX-A, and percutaneous Achilles tendon lengthening have failed. LEVEL OF EVIDENCE: Therapeutic Level I. See Instructions for Authors for a complete description of levels of evidence.

Practical strategies and perceptions from community pharmacists following their experiences with conducting pharmacy practice research: a qualitative content analysis.

OBJECTIVES: While prior research identified barriers to conducting research in community pharmacies, there remains a need to better understand facilitators to ensure successful collaborations between academic researchers and pharmacists. Our objective was to determine the experiences and perspectives of community pharmacists who have recently conducted a pharmacy practice-based research study to gain in-depth understanding of challenges as well as facilitators and identify strategies and solutions. METHODS: We conducted a qualitative study involving one-on-one semi-structured telephone interviews with community pharmacists following the completion of a practice-based research study in their pharmacies. Interview transcripts were analysed using inductive content analysis involving open coding, creating categories and abstraction into final themes. KEY FINDINGS: Eleven pharmacists participated in the qualitative interviews. We identified six major themes including: (1) barriers (e.g. time constraints); (2) facilitators (e.g. ideal pharmacy layout); (3) support and resources from academic researchers (e.g. helpfulness of training, easy-to-use study materials); (4) pharmacist-initiated strategies for conducting research (beyond prior suggestions from researchers); (5) suggestions for future pharmacy practice research; and (6) motivation for conducting pharmacy practice research. These findings informed practical strategies targeted at academic researchers and pharmacists, respectively, to facilitate the conduct of research in community pharmacists across various stages of the research process. CONCLUSIONS: Our study adds to better understanding of community pharmacists' perspectives on conducting research and identifies practical solutions that can be readily implemented by academic researchers and pharmacists participating in research.

The contralateral foot in children with unilateral clubfoot, is the unaffected side normal?

The unilateral unaffected clubfoot has previously been used as a control in longitudinal studies of clubfoot outcomes. However, we have observed that the unaffected clubfoot does not necessarily exhibit the same pedobarographic measurements as seen in normal control subjects. The purpose of this study was to evaluate whether the unaffected foot is indeed normal or if there are differences in the pedobarographic measurements of the unaffected foot compared to healthy normal controls.The Tekscan HR Mat™ was used to dynamically test the walking pattern of 103 subjects with unilateral clubfeet and compare the results to our previously published series of normal controls. Patients were divided into three groups: Group 1 (< 2 years), Group 2 (2-5 years) and Group 3 (>5 years). An unpaired t-test (p < 0.05) was used to compare percentage of stance at initiation of force, the percentage of stance at maximum force, the percentage of stance at termination of force, the maximum percentage force and the average force/time integral between a group of normal age matched controls and the unaffected foot in patients with unilateral clubfoot. Significant differences were identified between the unaffected side and normal controls for the pressure distribution, order of initial contact and foot contact time. These differences evolved and changed with age. The pedobarographic measurements of patients with clubfoot are not normal for the unaffected foot. As such the unaffected foot should not be referred to as normal, nor should it be used as a control.