Aims. Sickle cell disease (SCD) is an autosomal recessive inherited condition that presents with a number of clinical manifestations that include musculoskeletal manifestations (MM). MM may present differently in different individuals and settings and the predictors are not well known. Herein, we aimed at determining the predictors of MM in patients with SCD at the University Teaching Hospital, Lusaka, Zambia. Methods. An unmatched case-control study was conducted between January and May 2019 in children below the age of 16 years. In all, 57 cases and 114 controls were obtained by systematic sampling method. A structured questionnaire was used to collect data. The different MM were identified, staged, and classified according to the Standard Orthopaedic Classification Systems using radiological and laboratory investigations. The data was entered in Epidata version 3.1 and exported to STATA 15 for analysis. Multiple logistic regression was used to determine predictors and predictive margins were used to determine the probability of MM. Results. The cases were older median age 9.5 (interquartile range (IQR) 7 to 12) years compared to controls 7 (IQR 4 to 11) years; p = 0.003. After multivariate logistic regression, increase in age (adjusted odds ratio (AOR) = 1.2, 95% confidence interval (CI) 1.04 to 1.45; p = 0.043), increase in the frequency of vaso-occlusive crisis (VOC) (AOR = 1.3, 95% CI 1.09 to 1.52; p = 0.009) and increase in percentage of haemoglobin S (HbS) (AOR = 1.18, 95% CI 1.09 to 1.29; p < 0.001) were significant predictors of MM. Predictive margins showed that for a 16-year-old the average probability of having MM would be 51 percentage points higher than that of a two-year-old. Conclusion. Increase in age, frequency of VOC, and an increase in the percentage of HbS were significant predictors of MM. These predictors maybe useful to clinicians in determining children who are at risk. Cite this article: Bone Joint Open 2020;1-6:175–181

Aims. To identify a suite of the key physical, emotional, and social outcomes to be employed in clinical practice and research concerning Perthes' disease in children. Methods. The study follows the guidelines of the COMET-Initiative (Core Outcome Measures in Effectiveness Trials). A systematic review of the literature was performed to identify a list of outcomes reported in previous studies, which was supplemented by a qualitative study exploring the experiences of families affected by Perthes’ disease. Collectively, these outcomes formed the basis of a Delphi survey (two rounds), where 18 patients with Perthes’ disease, 46 parents, and 36 orthopaedic surgeons rated each outcome for importance. The International Perthes Study Group (IPSG) (Dallas, Texas, USA (October 2018)) discussed outcomes that failed to reach any consensus (either ‘in’ or ‘out’) before a final consensus meeting with representatives of surgeons, patients, and parents. Results. In total, 23 different outcome domains were identified from the systematic review, and a further ten from qualitative interviews. After round one of the Delphi survey, participants suggested five further outcome domains. A total of 38 outcomes were scored in round two of the Delphi. Among these, 16 outcomes were scored over the prespecified 70% threshold for importance (divided into six main categories: adverse events; life impact; resource use; pathophysiological manifestations; death; and technical considerations). Following the final consensus meeting, 14 outcomes were included in the final Core Outcome Set (COS). Conclusion. Core Outcome Sets (COSs) are important to improve standardization of outcomes in clinical research and to aid communication between patients, clinicians, and funding bodies. The results of this study should be a catalyst to develop high-quality clinical research in order to determine the optimal treatments for children with Perthes’ disease. Cite this article: Bone Joint J 2020;102-B(5):611–617

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

Aims. The objective of this study was to evaluate the clinical and radiological outcomes of patients younger than six months of age with developmental dysplasia of the hip (DDH) managed by either a Pavlik harness or Tübingen hip flexion splint. Methods. Records of 251 consecutive infants with a mean age of 89 days (SD 47), diagnosed with DDH between January 2015 and December 2018, were retrospectively reviewed. Inclusion criteria for patients with DDH were: younger than 180 days at the time of diagnosis; ultrasound Graf classification of IIc or greater; treatment by Pavlik harness or Tübingen splint; and no prior treatment history. All patients underwent hip ultrasound every seven days during the first three weeks of treatment and subsequently every three to four weeks until completion of treatment. If no signs of improvement were found after three weeks, the Pavlik harness or Tübingen splint was discontinued. Statistical analysis was performed. Results. The study included 251 patients with Graf grades IIc to IV in 18 males and 233 females with DDH. Mean follow-up time was 22 months (SD 10). A total of 116 hips were graded as Graf IIc (39.1%), nine as grade D (3.0%), 100 as grade III (33.7%), and 72 as grade IV (24.2%). There were 109 patients (128 hips) in the Pavlik group and 142 patients (169 hips) in the Tübingen group (p = 0.227). The Tübingen group showed a 69.8% success rate in Graf III and Graf IV hips while the success rate was significantly lower in the Pavlik group, 53.9% (p = 0.033). For infants older than three months of age, the Tübingen group showed a 71.4% success rate, and the Pavlik group a 54.4% success rate (p = 0.047). Conclusion. The Tübingen splint should be the preferred treatment option for children older than three months, and for those with severe forms of DDH such as Graf grade III and IV, who are younger than six months at time of diagnosis. The Tübingen hip flexion splint is a valid alternative to the Pavlik harness for older infants and those with more severe DDH. Cite this article: Bone Joint J 2021;103-B(5):991–998

Aims. Open reduction in developmental dysplasia of the hip (DDH) is regularly performed despite screening programmes, due to failure of treatment or late presentation. A protocol for open reduction of DDH has been refined through collaboration between surgical, anaesthetic, and nursing teams to allow same day discharge. The objective of this study was to determine the safety and feasibility of performing open reduction of DDH as a day case. Methods. A prospectively collected departmental database was visited. All consecutive surgical cases of DDH between June 2015 and March 2020 were collected. Closed reductions, bilateral cases, cases requiring corrective osteotomy, and children with comorbidities were excluded. Data collected included demographics, safety outcome measures (blood loss, complications, readmission, reduction confirmation), and feasibility for discharge according to the Face Legs Activity Cry Consolidability (FLACC) pain scale. A satisfaction questionnaire was filled by the carers. Descriptive statistics were used for analysis. Results. Out of 168 consecutive DDH cases, 16 patients fit the inclusion criteria (age range 10 to 26 months, 13 female). Intraoperative blood loss ranged from "minimal" to 120 ml, and there were no complications or readmissions. The FLACC score was 0 for all patients. The carers satisfaction questionnaire expressed high satisfaction from the experience with adequate information and support provided. Conclusion. Open reduction in DDH, without corrective osteotomy, is safe and feasible to be managed as a day case procedure. It requires a clear treatment pathway, analgesia, sufficient counselling, and communication with carers. It is even more important during the COVID-19 pandemic when reduced length of hospital stay is likely to be safer for both patient and their parents. Cite this article: Bone Joint Open 2021;2(4):271–277

Aims. The aim of this study was to determine the extent to which patient demographics, clinical presentation, and blood parameters vary in Kingella kingae septic arthritis when compared with those of other organisms, and whether this difference needs to be considered when assessing children in whom a diagnosis of septic arthritis is suspected. Methods. A prospective case series was undertaken at a single UK paediatric institution between October 2012 and November 2018 of all patients referred with suspected septic arthritis. We recorded the clinical, biochemical, and microbiological findings in all patients. Results. A total of 160 patients underwent arthrotomy for a presumed septic arthritis. Of these, no organism was identified in 61 and only 25 of these were both culture- and polymerase chain reaction (PCR)-negative. A total of 36 patients did not undergo PCR analysis. Of the remaining 99 culture- and PCR-positive patients, K. kingae was the most commonly isolated organism (42%, n = 42). The knee (n = 21), shoulder (n = 9), and hip (n = 5) were the three most commonly affected joints. A total of 28 cases (66%) of K. kingae infection were detected only on PCR. The mean age of K. kingae-positive cases (16.1 months) was significantly lower than that of those whose septic arthitis was due to other organisms (49.4 months; p < 0.001). The mean CRP was significantly lower in the K. kingae group than in the other organism group (p < 0.001). The mean ESR/CRP ratio was significantly higher in K. kingae (2.84) than in other infections (1.55; p < 0.008). The mean ESR and ESR/CRP were not significantly different from those in the 'no organism identified' group. Conclusion. K. kingae was the most commonly isolated organism from paediatric culture- and/or PCR-positive confirmed septic arthritis, with only one third of cases detected on routine cultures. It is important to develop and maintain a clinical suspicion for K. kingae infection in young patients presenting atypically. Routine PCR testing is recommended in these patients. Cite this article: Bone Joint J 2021;103-B(3):584–588

Aims. The significance of the ‘clicky hip’ in neonatal and infant examination remains controversial with recent conflicting papers reigniting the debate. We aimed to quantify rates of developmental dysplasia of the hip (DDH) in babies referred with ‘clicky hips’ to our dedicated DDH clinic. Patients and Methods. A three-year prospective cohort study was undertaken between 2014 and 2016 assessing the diagnosis and treatment outcomes of all children referred specifically with ‘clicky hips’ as the primary reason for referral to our dedicated DDH clinic. Depending on their age, they were all imaged with either ultrasound scan or radiographs. Results. There were 69 ‘clicky hip’ referrals over the three-year period. This represented 26.9% of the total 257 referrals received in that time. The mean age at presentation was 13.6 weeks (1 to 84). A total of 19 children (28%) referred as ‘clicky hips’ were noted to have hip abnormalities on ultrasound scan, including 15 with Graf Type II hips (7 bilateral), one Graf Type III hip, and three Graf Type IV hips. Of these, ten children were treated with a Pavlik harness, with two requiring subsequent closed reduction in theatre; one child was treated primarily with a closed reduction and adductor tenotomy. In total, 11 (15.9%) of the 69 ‘clicky hip’ referrals required intervention with either harness or surgery. Conclusion. Our study provides further evidence that the ‘clicky hip’ referral can represent an underlying diagnosis of DDH and should, in our opinion, always lead to further clinical and radiological assessment. In the absence of universal ultrasound screening, we would encourage individual units to carefully assess their own outcomes and protocols for ‘clicky hip’ referrals and tailor ongoing service provision to local populations and local referral practices. Cite this article: Bone Joint J 2018;100-B:1249–52

The recognition of hips at risk of displacement in children with cerebral palsy (CP) is a difficult problem for the orthopaedic surgeon. The Gross Motor Function Classification System (GMFCS) and head–shaft angle (HSA) are prognostic factors for hip displacement. However, reference values for HSA are lacking. This study describes and compares the development of HSA in normal hips and children with CP. . We selected 33 children from a retrospective cohort with unilateral developmental dysplasia of the hip (DDH) (five boys, 28 girls) and 50 children (35 boys, 15 girls) with CP with GMFCS levels II to V. HSA of normal developing hips was measured at the contralateral hip of unilateral DDH children (33 hips) and HSA of CP children was measured in both hips (100 hips). Measurements were taken from the radiographs of the children at age two, four and seven years. The normal hip HSA decreased by 2° per year (p < 0.001). In children with CP with GMFCS levels II and III HSA decreased by 0.6° (p = 0.046) and 0.9° (p = 0.049) per year, respectively. The HSA did not alter significantly in GMFCS levels IV and V. . Between the ages of two and eight years, the HSA decreases in normal hips and CP children with GMFCS level, II to III but does not change in GMFCS levels IV to V. As HSA has a prognostic value for hip displacement, these reference values may help the orthopaedic surgeon to predict future hip displacement in children with CP. Cite this article: Bone Joint J 2015;97-B:1291–5

Aims. Aneurysmal bone cysts (ABCs) are locally aggressive lesions typically found in the long bones of children and adolescents. A variety of management strategies have been reported to be effective in the treatment of these lesions. The purpose of this review was to assess the effectiveness of current strategies for the management of primary ABCs of the long bones. Methods. A systematic review of the published literature was performed to identify all articles relating to the management of primary ABCs. Studies required a minimum 12-month follow-up and case series reporting on under ten participants were not included. Results. A total of 28 articles meeting the eligibility criteria were included in this review, and all but one were retrospective in design. Due to heterogeneity in study design, treatment, and outcome reporting, data synthesis and group comparison was not possible. The most common treatment option reported on was surgical curettage with or without a form of adjuvant therapy, followed by injection-based therapies. Of the 594 patients treated with curettage across 17 studies, 86 (14.4%) failed to heal or experienced a recurrence. Similar outcomes were reported for 57 (14.70%) of the 387 patients treated with injection therapy across 12 studies. Only one study directly compared curettage with injection therapy (polidocanol), randomizing 94 patients into both treatment groups. This study was at risk of bias and provided low-quality evidence of a lack of difference between the two interventions, reporting success rates of 93.3% and 84.8% for injection and surgical treatment groups, respectively. Conclusion. While both surgery and sclerotherapy are widely implemented for treatment of ABCs, there is currently no good quality evidence to support the use of one option over the other. There is a need for prospective multicentre randomized controlled trials (RCTs) on interventions for the treatment of ABCs. Cite this article: Bone Jt Open 2021;2(2):125–133

Aims. The aim of this study was to establish the incidence of developmental dysplasia of the hip (DDH) diagnosed after one-year of age in England, stratified by age, gender, year, and region of diagnosis. Patients and Methods. A descriptive observational study was performed by linking primary and secondary care information from two independent national databases of routinely collected data: the United Kingdom Clinical Practice Research Datalink and Hospital Episode Statistics. The study examined all children from 1 January 1990 to 1 January 2016 who had a new first diagnostic code for DDH aged between one and eight years old. Results. The incidence of late-diagnosed DDH was 1.28 per 1000 live births. Within the study population, 754 children were identified with a diagnosis of DDH after one-year of age. Of all late diagnoses, 536 (71.1%) were detected between one to two years of age. There were 608 female patients (80.6%) and 146 male patients (19.4%), giving a female-to-male ratio of 4.2:1. Distribution was evenly spread throughout England. Conclusion. The incidence of late-diagnosed DDH has not been reduced from that reported 35 years ago, prior to the introduction of the national selective screening programme for DDH. Cite this article: Bone Joint J 2019;101-B:281–287

Aims. The aims of this study were to describe the course of non-operatively managed, bilateral Perthes’ disease, and to determine specific prognostic factors for the radiographic and clinical outcome. . Patients and Methods. We identified 40 children with a mean age of 5.9 years (1.8 to 13.5), who were managed non-operatively for bilateral Perthes’ disease from our prospective, multicentre study of this condition, which included all children in Norway who were diagnosed with Perthes’ disease in the five-year period between 1996 and 2000. All children were followed up for five years. . The hips were classified according to the Catterall classification. A modified three-group Stulberg classification was used as an outcome measure, with a spherical femoral head being defined as a good outcome, an oval head as fair, and a flat femoral head as a poor outcome. . Results. Concurrent, simultaneous bilateral Perthes’ disease was seen in 23 children and 17 had the sequential onset of bilateral disease. The mean delay in onset for the second hip in the latter group was 1.9 years (0.3 to 5.5). . The five-year radiographic outcome was good in 30 (39%), fair in 25 (33%) and poor in 21 (28%) of the hips. The strongest predictors of poor outcome were > 50% necrosis of the femoral head, with odds ratio (OR) 19.6, and age at diagnosis > 6 years (OR 3.3). Other risk factors for poor outcome were the timing of the onset of disease, where children with the sequential onset of bilateral disease had a higher risk than those with the concurrent onset of bilateral disease (p = 0.021, chi-squared test). . Following a diagnosis of Perthes’ disease in one hip, there was a 5% chance of developing it in the contralateral hip. . Conclusion. These results imply that we need to distinguish between children with concurrent onset and those with sequential onset of bilateral Perthes’ disease, as the outcomes may be different. This has not been previously described. Children with concurrent onset of bilateral disease had a similar outcome to our previous series of those with unilateral disease, whereas children with sequential onset of bilateral disease had a worse prognosis. The increased risk of developing Perthes’ disease in the contralateral hip in those with unilateral disease is important information for the child and parents. . Cite this article: Bone Joint J 2016;98-B:569–75

Aims. Our aim was to describe the mid-term appearances of the repair process of the Achilles tendon after tenotomy in children with a clubfoot treated using the Ponseti method. Patients and Methods. A total of 15 children (ten boys, five girls) with idiopathic clubfoot were evaluated at a mean of 6.8 years (5.4 to 8.1) after complete percutaneous division of the Achilles tendon. The contour and subjective thickness of the tendon were recorded, and superficial defects and its strength were assessed clinically. The echogenicity, texture, thickness, peritendinous irregularities and potential for deformation of the tendon were evaluated by ultrasonography. Results. The appearance of the Achilles tendon was slightly abnormal, with more thickening and less conspicuous contours than a normal tendon. Its strength was grossly normal, with no insufficiency of the triceps surae. Ultrasonographic findings revealed a mild fusiform thickening in 12 children (80%). The tissue at the site of the repair had a slightly hypoechoic, fibrillar quality with hyperechoic striation and the anterior contour was irregular and blurred. There was a focal narrowing within the healing tissue in two children. Conclusion. This mid-term evaluation of the ability of the Achilles tendon to repair after division suggests a combination of intrinsic and extrinsic mechanisms. There were minor abnormalities which did not appear to affect function. Cite this article: Bone Joint J 2017;99-B:139–44

Unstable pelvic injuries in young children with an immature pelvis have different modes of failure from those in adolescents and adults. We describe the pathoanatomy of unstable pelvic injuries in these children, and the incidence of associated avulsion of the iliac apophysis and fracture of the ipsilateral fifth lumbar transverse process (L5-TP). We retrospectively reviewed the medical records of 33 children with Tile types B and C pelvic injuries admitted between 2007 and 2014; their mean age was 12.6 years (2 to 18) and 12 had an immature pelvis. Those with an immature pelvis commonly sustained symphyseal injuries anteriorly with diastasis, rather than the fractures of the pubic rami seen in adolescents. Posteriorly, transsacral fractures were more commonly encountered in mature children, whereas sacroiliac dislocations and fracture-dislocations were seen in both age groups. Avulsion of the iliac apophysis was identified in eight children, all of whom had an immature pelvis with an intact ipsilateral L5-TP. Young children with an immature pelvis are more susceptible to pubic symphysis and sacroiliac diastasis, whereas bony failures are more common in adolescents. Unstable pelvic injuries in young children are commonly associated with avulsion of the iliac apophysis, particularly with displaced SI joint dislocation and an intact ipsilateral L5-TP. Cite this article: Bone Joint J 2015; 97-B:696–704

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

Aims. This study compared the long-term results following Salter osteotomy and Pemberton acetabuloplasty in children with developmental dysplasia of the hip (DDH). We assessed if there was a greater increase in pelvic height following the Salter osteotomy, and if this had a continued effect on pelvic tilt, lumbar curvature or functional outcomes. Patients and Methods. We reviewed 42 children at more than ten years post-operatively following a unilateral Salter osteotomy or Pemberton acetabuloplasty. We measured the increase in pelvic height and the iliac crest tilt and sacral tilt at the most recent review and at an earlier review point in the first decade of follow-up. We measured the lumbar Cobb angle and the Short Form-36 (SF-36) and Harris hip scores were collected at the most recent review. Results. During the first decade of follow-up, there was a greater increase in pelvic height in the children who had a Salter osteotomy (Salter, 10.1%; Pemberton, 4.3%, p < 0.001). The difference in the increase in pelvic height was insignificant at the most recent review (Salter, 4.4%; Pemberton, 3.1%, p = 0.249). There was no significant difference between the two groups for the lumbar Cobb angle, (Salter, 3.1°; Pemberton, 3.3°, p = 0.906). A coronal lumbar curve was seen in 41 children (97%), 30 of these had a compensatory curve. Sacral tilt was the radiographic parameter for pelvic imbalance that correlated most with the lumbar Cobb angle (Pearson correlation co-efficient 0.59). The Harris hip score and SF-36 were good and showed no differences between the two groups. Conclusion. In the long-term, we found no difference in the functional results or pelvic imbalance between Salter osteotomy and Pemberton acetabuloplasty in the management of children with DDH. Cite this article: Bone Joint J 2016;98-B:1145–50

Hip displacement, defined in this study as a migration percentage (MP) of more than 40%, is a common, debilitating complication of cerebral palsy (CP). In this prospective study we analysed the risk of developing hip displacement within five years of the first pelvic radiograph. . All children with CP in southern and western Sweden are invited to register in the hip surveillance programme CPUP. Inclusion criteria for the two groups in this study were children from the CPUP database born between 1994 and 2009 with Gross Motor Function Classification System (GMFCS) III to V. Group 1 included children who developed hip displacement, group 2 included children who did not develop hip displacement over a minimum follow-up of five years. A total of 145 children were included with a mean age at their initial pelvic radiograph of 3.5 years (0.6 to 9.7). The odds ratio for hip displacement was calculated for GMFCS-level, age and initial MP and head-shaft angle. A risk score was constructed with these variables using multiple logistic regression analysis. The predictive ability of the risk score was evaluated using the area under the receiver operating characteristics curve (AUC). . All variables had a significant effect on the risk of a MP > 40%. The discriminatory accuracy of the CPUP hip score is high (AUC = 0.87), indicating a high ability to differentiate between high- and low-risk individuals for hip displacement. The CPUP hip score may be useful in deciding on further follow-up and treatment in children with CP. Cite this article: Bone Joint J 2015;97-B:1441–4