Congenital growth hormone deficiency associated with hip dysplasia and Legg-Calve-Perthes disease

Objective: Growth hormone deficiency (GHD) is usually treated with recombinant human GH (rhGH), and this has been rarely associated with hip disorders. We analysed the clinical data of patients with congenital GHD receiving rhGH who had associated hip dysplasia or Legg‐Calve‐Perthes disease (LCPD), with a view to determining whether the hip dysplasia was associated with the underlying disease or with rhGH treatment. / Design: We performed a retrospective analysis of paediatric and adolescent patients seen between 1992–2018 with congenital GHD and hip disorders. Data were collected through a review of the patients’ medical records and included demographics, clinical and imaging data, and the time frame between the onset of the symptoms related to the hip disorders and the onset of GH treatment. / Results: Of the 13 patients with hip disorders, hip dysplasia was present in ten patients and LCPD in three. Hip dysplasia was diagnosed before rhGH was initiated in 50% of cases. These patients had bilateral hip dysplasia and isolated GHD. LCPD was diagnosed in one patient before rhGH was commenced and did not progress. In two patients, LCPD was diagnosed after rhGH was started and did temporarily progress in one of them, but rhGH was not discontinued because LCPD did not seem to be related to rhGH treatment. / Conclusions: This study suggests that hip dysplasia could be a manifestation of an underlying GHD. Additionally, rhGH treatment may not necessarily be causative of LCPD

Management of slipped capital femoral epiphysis: results of a survey of the members of the European Paediatric Orthopaedic Society

AIM: To determine current practice recommendations for the treatment of slipped capital femoral epiphysis (SCFE) among members of the European Paediatric Orthopaedic Society (EPOS). MATERIALS AND METHODS: A questionnaire with 4 case vignettes of a 12-year-old boy presenting with a stable and unstable SCFE. Each, stable and unstable slips, was of mild (20° epiphyseal-shaft angle) and of severe (60° epiphyseal-shaft angle) degree was sent to all members of EPOS in 2009 in order to ascertain their views on the best management of SCFE. Specifically, respondents were asked about the role of reduction, methods of fixation, prophylactic fixation of the non-affected hip, postoperative management and their view on the anticipated need for secondary surgery. RESULTS: The response rate was 25% (72/287). The participating surgeons' average workload was 76% in paediatric orthopaedics, with mean 16 years of experience. Surgeons were most consistent in their advice for stable slips, where around 90% of the respondents did not recommend a reduction of the slip regardless of severity of slip. Seventy per cent of the respondents recommended the use of only one screw for fixation of a stable slip and for mild unstable slips. For severe unstable slips, 46% of surgeons recommended reduction only by positioning of the hip on the fracture table, 35% by manipulation and 11% advised open reduction. Responders were less consistent in their advice on the anticipated need for secondary osteotomies (in mild slips about 40% and about 60% in severe slips would advise an osteotomy) and on treatment of the contralateral hip (with 32% of surgeons recommending prophylactic fixation of the contralateral hip). CONCLUSION: Within members of EPOS, there is controversy on several aspects of the management of SCFE particularly on aspects of the treatment of unstable SCFE. SIGNIFICANCE: Members of EPOS predominantly use traditional means of treatment for patients with SCFE. In contrast, the more modern treatment concepts, such as open reduction via surgical dislocation, are rarely used

Study protocol for evaluation of aid to diagnosis for developmental dysplasia of the hip in general practice: controlled trial randomised by practice

Introduction: In the UK, a compulsory ‘6-week hip check’ is performed in primary care for the detection of developmental dysplasia of the hip (DDH). However, missed diagnoses and infants incorrectly labelled with DDH remain a problem, potentially leading to adverse consequences for infants, their families and the National Health Service. National policy states that infants should be referred to hospital if the 6-week check suggests DDH, though there is no available tool to aid examination or offer guidelines for referral. We developed standardised diagnostic criteria for DDH, based on international Delphi consensus, and a 9-item checklist that has the potential to enable non-experts to diagnose DDH in a manner approaching that of experts. / Methods and analysis: We will conduct a controlled trial randomised by practice that will compare a diagnostic aid against standard care for the hip check. The primary objective is to determine whether an aid to the diagnosis of DDH reduces the number of clinically insignificant referrals from primary care to hospital and the number of late diagnosed DDH. The trial will include a qualitative process evaluation, an assessment of professional behavioural change and a full health economic evaluation. We will recruit 152 general practitioner practices in England. These will be randomised to conduct the hip checks with use of the study diagnostic aid and/or as per usual practice. The total number of infants seen during a 15-month recruitment period will be 110 per practice. Two years after the 6-week hip check, we will measure the number of referred infants that are (1) clinically insignificant for DDH and (2) those that constitute appropriate referrals. / Ethics and dissemination: This study has approval from the Health Research Authority (16/1/2020) and the Confidentiality Advisory Group (18/2/2020). Results will be published in peer-reviewed academic journals, disseminated to patient organisations and the media. / Trial registration number: NCT04101903; Pre-results

A consensus exercise identifying priorities for research into clinical effectiveness among children's orthopaedic surgeons in the United Kingdom

Aims: High-quality clinical research in children’s orthopaedic surgery has lagged behind other surgical subspecialties. This study used a consensus-based approach to identify research priorities for clinical trials in children’s orthopaedics. / Methods: A modified Delphi technique was used, which involved an initial scoping survey, a two-round Delphi process and an expert panel formed of members of the British Society of Children’s Orthopaedic Surgery. The survey was conducted amongst orthopaedic surgeons treating children in the United Kingdom and Ireland. / Results: A total of 86 clinicians contributed to both rounds of the Delphi process, scoring priorities from one (low priority) to five (high priority). Elective topics were ranked higher than those relating to trauma, with the top ten elective research questions scoring higher than the top question for trauma. Ten elective, and five trauma research priorities were identified, with the three highest ranked questions relating to the treatment of slipped capital femoral epiphysis (mean score 4.6/ 5), Perthes’ disease (4.5) and bone infection (4.5). / Conclusion: This consensus-based research agenda will guide surgeons, academics and funders to improve the evidence in children’s orthopaedic surgery and encourage the development of multicentre clinical trials

Research priorities in children requiring elective surgery for conditions affecting the lower limbs: a James Lind Alliance Priority Setting Partnership

Objective: To identify and prioritise research questions concerning the elective surgical management of children with conditions affecting the lower limb by engaging patients, carers and healthcare professionals. Design: A modified nominal group technique. Setting: UK. Participants: 388 individuals (29 patients, 155 parents/carers, 204 healthcare professionals) were recruited through hospital clinics, patient charities and professional organisations and participated in the initial prioritisation survey; 234 individuals took part in the interim prioritisation survey. 33 individuals (3 patients, 9 parents/carers, 11 healthcare professionals, 7 individuals representing the project’s steering group and 3 James Lind Alliance (JLA) facilitators) attended the final face-to-face workshop to rank the top 10 research priorities. Interventions: Surveys were distributed using various media resources such as newsletters, internet messaging boards and the ‘Paediatric Lower Limb Surgery Priority Setting Partnership (PSP) website. Printed copies of the questionnaire were also made available to families in outpatient clinics. Outcome measures: Survey results, top 10 and top 26 priority rankings Results: The process took 18 months to complete (July 2017–January 2019); 388 people generated 1023 questions; a total of 801 research questions were classified as true uncertainties. Following the JLA methodology, 75 uncertainties were developed from the initial 801 questions. Twenty six of those were selected through a second survey and were taken to the final face-to-face workshop where the top 10 research priorities were selected. The top10 priorities included questions on cerebral palsy, common hip conditions (ie, Perthes’ disease and developmental dysplasia of the hip) as well as rehabilitation techniques and methods to improve shared decision-making between clinicians and patients/families. Conclusions: This is the first JLA PSP in children’s orthopaedic surgery, a particularly under-researched and underfunded area. We have identified important research topics which will guide researchers and funders and direct their efforts in future research

Feeling the clunk: Managing and attributing uncertainty in screening for developmental dysplasia of the hip in infancy

This is the final version. Available on open access from Elsevier via the DOI in this recordThe management of uncertainty in clinical practice has been an enduring topic of sociological scholarship. However, little of this addresses how uncertainty and non-knowledge are attributed to the self and other actors. We take the example of checking for developmental dysplasia of the hip (DDH), part of infant screening in UK primary care, to examine the ‘double contingency’ of attributions of uncertainty and ignorance. Our data come from interviews with parents and General Practitioners (GPs), and observations of the six-week check conducted as part of a study to develop a checklist to aid GPs' diagnostic and referral decisions. Parents' pervasive uncertainties about managing with a new-born infant place them in a trusting relation to biomedicine, in which knowledge about infant hips is delegated to the clinical team: most described themselves as not-knowing about DDH. GPs focus on the uncertainties of applying sensory and experiential knowledge of infant bodies, in a consultation with more diffuse aims than screening for DDH. A prototype checklist, developed by orthopaedic specialists, was an explicit attempt to reduce uncertainty around thresholds for referral. However, using the checklist surfaced multiple logics of uncertainty. It also surfaced attributions of uncertainty and non-knowledge to other actors: orthopaedic specialists' assumptions about GPs' uncertain technical knowledge; GPs' assumptions about orthopaedic specialists' ignorance of the primary care setting; and clinicians' assumptions about the role of parental ignorance. This ‘double contingency’ of attributions of other actors' non-knowledge is a salient additional dimension to the uncertainty that infuses biomedical practice.Wellcome TrustNational Institute for Health Research (NIHR

Timing of surgical intervention for developmental dysplasia of the hip: a randomised controlled trial (Hip 'Op)

Background: Developmental dysplasia of the hip (DDH) is a very common congenital disorder, and late-presenting cases often require surgical treatment. Surgical reduction of the hip may be complicated by avascular necrosis (AVN), which occurs as a result of interruption to the femoral head blood supply during treatment and can result in long-term problems. Some surgeons delay surgical treatment until the ossific nucleus (ON) has developed, whereas others believe that the earlier the reduction is performed, the better the result. Currently there is no definitive evidence to support either strategy. Objectives: To determine, in children aged 12 weeks to 13 months, whether or not delayed surgical treatment of a congenitally dislocated hip reduces the incidence of AVN at 5 years of age. The main clinical outcome measures were incidence of AVN and the need for a secondary surgical procedure during 5 years’ follow-up. In addition, to perform (1) a qualitative evaluation of the adopted strategy and (2) a health economic analysis based on NHS and societal costs. Design: Phase III, unmasked, randomised controlled trial with qualitative and health economics analyses. Participants were randomised 1 : 1 to undergo either early or delayed surgery. Setting: Paediatric orthopaedic surgical centres in the UK. Participants: Children aged 12 weeks to 13 months with DDH, either newly diagnosed or following failed splintage, and who required surgery. We had a target recruitment of 636 children. Interventions: Surgical reduction of the hip performed as per the timing allocated at randomisation. Main outcome measures: Primary outcome – incidence of AVN at 5 years of age (according to the Kalamchi and MacEwen classification). Secondary outcomes – need for secondary surgery, presence or absence of the ON at the time of primary treatment, quality of life for the main carer and child, and a health economics and qualitative analysis. Results: The trial closed early after reaching < 5% of the recruitment target. Fourteen patients were randomised to early treatment and 15 to delayed treatment. Implementation of rescue strategies did not improve recruitment. No primary outcome data were collected, and no meaningful conclusions could be made from the small number of non-qualitative secondary outcome data. The qualitative work generated rich data around three key themes: (1) access to, and experiences of, primary and secondary care; (2) the impact of surgery on family life; and (3) participants’ experiences of being in the trial. Limitations: Overoptimistic estimates of numbers of eligible patients seen at recruiting centres during the planning of the trial, as well as an overestimation of the recruitment rate, may have also contributed to unrealistic expectations on achievable patient numbers. Future work: There may be scope for investigation using routinely available data. Conclusions: Hip ’Op has highlighted the importance of accurate advance information on numbers of available eligible patients, as well as support from all participating investigators when conducting surgical research. Despite substantial consultation with parents of children in the planning stage, the level of non-participation experienced during recruitment was much higher than anticipated. The qualitative work has emphasised the need for appropriate advice and robust support for parents regarding the ‘real-life’ aspects of managing children with DDH. Trial registration: Current Controlled Trials ISRCTN76958754. Funding: This project was funded by the National Institute for Health Research (NIHR) Health Technology Assessment programme and will be published in full in Health Technology Assessment; Vol. 21, No. 63. See the NIHR Journals Library website for further project information

Parents' expectations and experiences of the 6-week baby check: a qualitative study in primary care

This s the final published version, available from Royal College of General Practitioners via the DOI in this recordBackground: The Newborn and Infant Physical Examination (NIPE) programme requires all babies to have a comprehensive health check at 6-8 weeks of age. These are typically completed by GPs. Although person-centred care has achieved prominence in maternity care policy in recent years, there is limited empirical evidence on what parents and/or carers expect from the check, and how far experiences meet their needs. Aim: To explore the expectations and experiences of parents attending their GP for a baby check. Design & setting: A qualitative study was undertaken in primary care in London. Method: Content analysis was undertaken of transcripts of semi-structured interviews. Interviews were conducted with a total of 16 participants (14 mothers and two fathers) who had recently attended for a 6-week check for their baby. Results: Despite the availability of plentiful sources of general advice on infants' health and development, a thorough check by a trusted GP was an important milestone for most parents. They had few specific expectations of the check in terms of what examinations were undertaken, but even experienced parents anticipated reassurance about their baby's normal development. Many also hoped for reassurance about their own parenting. Parents appreciated GPs who explained what they were doing during the examination; space to raise any concerns; and combined mother and baby checks. Referrals to secondary care were generally experienced as reassuring rather than a source of anxiety. Conclusion: The baby check meets needs beyond those of the NIPE screening programme. Protecting the time for a thorough consultation is important for parents at what can be a vulnerable time.National Institute for Health Research (NIHR

Predicting developmental dysplasia of the hip in at-risk newborns.

BACKGROUND: The development of developmental dysplasia of the hip can be attributed to several risk factors and often in combination with each other. When predicting the likelihood of developing this condition, clinicians tend to over and underestimate its likelihood of occurring. Therefore, the study aim is to determine among at-risk newborns how to best predict developmental dysplasia of the hip (DDH) within 8 weeks post-partum. METHODS: Prospective cohort study in secondary care. Patient population included newborns at-risk for DDH - we assessed 13,276 consecutive newborns for the presence of DDH risk factors. Only newborns with at least one of the predefined risk factors and those showing an abnormal examination of the hip were enrolled (n = 2191). For the development of a risk prediction model we considered 9 candidate predictors and other variables readily available at childbirth. The main outcome measure was ultrasonography at a median age of 8 weeks using consensus diagnostic criteria; outcome assessors were blinded. RESULTS: The risk model includes four predictors: female sex (OR = 5.6; 95% CI: 2.9-10.9; P  4000 g (OR = 1.6; 95% CI: 0.6-4.2; P = 0.34), and abnormal examination of hip (OR = 58.8; 95% CI: 31.9, 108.5; P <  0.001). This model demonstrated excellent discrimination (C statistic = 0.9) and calibration of observed and predicted risk (P = 0.35). A model without the variable 'hip examination' demonstrated similar performance. CONCLUSION: The risk model quantifies absolute risk of DDH within 8 weeks postpartum in at-risk newborns. Based on clinical variables readily available at the point of childbirth, the model will enhance parental counselling and could serve as the basis for real time decisions prior to discharge from maternity wards