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Background/aims Outpatient physical therapy is the mainstay of treatment in shoulder instability, but lack of motivation and intermittent monitoring of progress is associated with poor compliance. ‘Exergames’ (a combination of videogames and physical exercise) may provide an additional or alternative motivational factor. The aim of this study was to determine the applicability of the Medical Interactive Recovery Assistant Xbox Kinect gaming software in the management of adolescent atraumatic shoulder instability. Methods Patients completed six 30-minute sessions playing games using the Medical Interactive Recovery Assistant while participating in a therapy programme. Pain and fatigue questionnaires, functional assessments and a framework analysis of free-text comments were used. Results A total of 20 female patients (15 unilateral, five bilateral pathology, mean age 14.1 years) completed the sessions. An average session length was 10.8 mins (range 0–24 mins). After each session using the Medical Interactive Recovery Assistant, 80% were neither in pain, nor overly tired. At 2 weeks, the Stanmore Percentage of Normal Shoulder Assessment scores had improved by a mean 17.9% and 10 (50%) patients showed improvements in Oxford Shoulder Instability Scores. Overall, 15 participants (75%) liked the technology; however six (32%) preferred standard physiotherapy, because of software limitations and lack of personal interaction. Conclusions Medical Interactive Recovery Assistant gamification is applicable for this patient cohort and could be integrated into adolescent atraumatic shoulder instability rehabilitation programmes; however, results emphasise the importance of personal contact.

Fibro-adipose vascular anomaly (FAVA) is a discrete type of vascular anomaly. We describe our experience managing FAVA at a tertiary level paediatric hospital and offer a treatment algorithm. Methods A retrospective review of 27 patients with proven FAVA was undertaken. All patients had undergone MRI and USS evaluation. Patient demographics, presenting concerns, treatment methods, and outcomes were recorded and evaluation with the paediatric outcomes data collection instrument (PODCI) completed a minimum of 12 months after definitive treatment. Results Mean age at presentation was 8.9 years (range: 9 m-17.4 y) and mean post-treatment follow-up was 7.4 y (range: 2 y-11.6 y). Twenty of 27 lesions affected the lower limb. Severe neurogenic-type pain was present in 23 cases and contractures across joints in 11 cases. Sclerotherapy with sodium tetradecyl sulphate was used in 11 cases, with no improvement in symptoms. Cryoablation provided pain relief in 3/4 cases, but contracture subsequently increased in one patient and pain recurred in another. Fourteen cases underwent surgery (four surgical excisions alone, 10 in combination with other procedures). Three patients required four further surgical procedures that include one amputation for intractable pain and poor function. PODCI evaluations suggest overall good function, with surgical management and interventional radiology that provide comparable results. Surgery did correct deformity. Conclusion If conservative measures or cryoablation fail to achieve symptomatic control, surgical excision should be considered, combined with adjunctive procedures, to correct contractures and balance muscle forces. Relief of pain may compensate for the loss of muscle mass and overall improves function. Multidisciplinary team working is essential.

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.

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

Open fractures are at greater risk of infection and delayed bone healing. Guidelines to reduce these risks exist for adult open fracture management but not for paediatric open fractures, where there is considerable practice variability. This systematic review evaluates the quality of the evidence and clinical outcomes for paediatric open fractures treated non-operatively versus operatively. A PROSPERO-registered, PRISMA-compliant systematic review and meta-analysis. Databases searched were MEDLINE, Embase and Web of Science (WoS) up to March 2019. Clinical studies reporting adverse outcomes of both non-operative and operative management of paediatric open fractures were included. Data extracted included demographics, treatment methods and outcomes. The primary outcome was infection (osteomyelitis and/or wound infection). Secondary outcomes were abnormalities of bone healing (delayed union, malunion and nonunion) and re-fracture. The ROBINS-I tool was used to assess risk of bias. Seventeen studies reporting 1093 open fractures were included. Non-operatively managed injuries had a lower risk of osteomyelitis (RR 0.33 [95%CI 0.12-0.86]), wound infection (RR 0.47 [95%CI 0.22-0.97]) and nonunion (RR 0.27 [95%CI 0.09-0.80]). Gustilo-Anderson (GA) III injuries had the highest incidence of osteomyelitis (10.7%) with no difference in outcome between operative and non-operative groups (RR 0.67 [95%CI 0.22-2.03]). Tibial GA III fractures were associated with a lower risk of osteomyelitis than femoral fractures: adverse effects were seen in the operative group. All studies were retrospective, observational and at high risk of bias. The quality of the evidence relating to paediatric open fractures is low, and findings are limited by significant methodological flaws in the literature. GA I and II fractures were commonly managed non-operatively and associated with a lower infection rate. Operative intervention was more frequent in GA III fractures, where the risk of infection was highest. Operative management of GA III fractures was not associated with a lower infection risk compared to non-operative management. Robust prospective multi-centre studies are needed to explore further the most effective management of paediatric open fractures and to inform guideline development.

Background Idiopathic toe walking (ITW) is an exclusionary diagnosis resulting in a child walking on the balls of their feet. Preferred treatment options may be due to the severity of the toe or the health professional preference There are limited guidelines supporting consistent treatment recommendations for this condition. This research aimed to understand agreement between health professionals' knowledge of evidence for common treatment strategies for ITW and if health professionals supported these strategies being used in clinical practice. Methods An international online survey was opened to registered health professionals who treat children with ITW between July 2017 and March 2018. The survey had two components: (a) demographic variables and variables relating to knowledge of evidence about ITW treatments and (b) support for common treatment strategies. Additional data on strategy use, referrals, and preference were collected. Kappa statistics described intra-rater agreement between evidence knowledge and support. Multivariable regression analyses identified factors associated with the 10 most commonly preferred treatments. Results There were 908 international responses. Kappa agreement for paired correct responses determined a fair agreement for evidence support knowledge for four strategies including watch and wait (Kappa = 0.24), stretching (Kappa = 0.30), sensory integration strategies (Kappa = 0.40), and motor control strategies (Kappa = 0.24) and moderate responses for 13 others. No strategies had greater than moderate agreement between correct knowledge of evidence and strategy support. Profession, location, number of children seen in practice, and not correctly identifying the evidence factored into many of the most commonly used strategies for ITW (p Conclusions The results from this study, which confirm a variety of interventions, are utilized in the management of ITW around the world. Furthermore, there remains a disconnection between paediatric health professionals' understanding of the evidence of common treatment strategies of ITW and a consensus for the treatment of this condition.

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.

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.

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.

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.

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.

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.

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

Aims The Ponseti method is the benchmark treatment for the correction of clubfoot. The primary rate of correction is very high, but outcome further down the treatment pathway is less predictable. Several methods of assessing severity at presentation have been reported. Classification later in the course of treatment is more challenging. This systematic review considers the outcome of the Ponseti method in terms of relapse and determines how clubfoot is assessed at presentation, correction, and relapse. Patients and Methods A prospectively registered systematic review was carried out according to Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. Studies that reported idiopathic clubfoot treated by the Ponseti method between 1 January 2012 and 31 May 2017 were included. The data extracted included demographics, Ponseti methodology, assessment methods, and rates of relapse and surgery. Results A total of 84 studies were included (7335 patients, 10 535 clubfeet). The relapse rate varied between 1.9% and 45%. The rates of relapse and major surgery (1.4% to 53.3%) and minor surgery (0.6% to 48.8%) both increased with follow-up time. There was high variability in the assessment methods used across timepoints; only 57% of the studies defined relapse. Pirani scoring was the method most often used. Conclusion Recurrence and further surgical intervention in idiopathic clubfoot increases with the duration of follow-up. The corrected and the relapsed foot are poorly defined, which contributes to variability in outcome. The results suggest that a consensus for a definition of relapse is needed. Cite this article: Bone Joint J 2019;101-B:639–645.

Purpose The Ponseti method is a well-established approach to treating clubfoot. Potentially, both the underlying pathology and adherence to post-correction bracing can affect lower limb function and age of independent standing and walking. This cohort study investigates the age at which infants with idiopathic clubfoot treated using the Ponseti method achieved three selected developmental milestones and whether or not this correlated with treatment compliance. Methods A prospectively collected database from four centres was visited. Inclusion criteria were patients with idiopathic clubfoot with no comorbidities or prior treatment. Age at attainment of independent standing, walking, nocturnal continence was compared across three groups: I) congenital talipes equinovarus (CTEV) children compliant with treatment; II) CTEV children non-compliant with treatment; and III) typically-developed siblings. Minimum follow-up was five years. Results In all, 130 patients (198 feet) fitted the inclusion criteria: 43:87 (F:M). Standing was achieved by a mean 12.0 months in group I (sd 2.50); 12.0 months (sd 2.0) in II and ten months (sd 3.0) in III. Walking was achieved by a mean 15 months (sd 4.0) in group I, 14 months (sd 1.75) in II and 12 months (sd 3) in III, respectively. Both the compliant and non-compliant CTEV children were significantly slower at achieving standing and walking compared to sibling controls (p < 0.0001). There was no significant difference between age of nocturnal continence between the three groups. Conclusion Infants with idiopathic clubfoot treated according to the Ponseti method achieve independent standing and walking approximately two months later than their typically-developed siblings. The delay is not related to the use of the foot abduction brace. Level of evidence III

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

Background Congenital Talipes Equinovarus (CTEV) deformity affects 1:1000 new-borns. Since the Ponseti technique became popular, the need for operative intervention beyond tenotomy has reduced. However, mid-long term results remain poorly reported. This prospective case series describes our patient population and outcomes since 2011 with an innovative physiotherapy-led Ponseti service. Methods A prospective study of CTEV patients attending the Ponseti clinic. Primary outcomes included range of movement, calf circumference and functional status. Parents of children over the age of 5 were asked to complete the Oxford Ankle Foot questionnaire (OxFAQ). Results Between Feb 2011-Aug 2020, 75 (M=43) patients were treated. Mean age at follow-up was 87.5 months (range 18–157 m). 42 cases were bilateral and thus 117 feet were treated. 45 (60%) were idiopathic and 30 (40%) non-idiopathic. Of the 75 patients, 50 (67%) underwent tenotomy under local anaesthetic in clinic. Tenotomy rates were higher in non-idiopathic vs idiopathic patients (83% vs 56%)(P Mean dorsiflexion (knee extended) in affected feet was 14°. In unilateral cases mean dorsiflexion (knee extended) in unaffected foot vs affected foot was 17° vs 12°. All idiopathic feet were plantigrade. Abnormal evertor function was seen in 3 of 66 (5%) idiopathic feet versus 22 of 51 non-idiopathic feet (43%) (p Calf circumference discrepancy was higher in unilateral cases versus bilateral (mean 1.3 cm versus 0.6 cm respectively). Unilateral cases undergoing tenotomy had a greater mean circumference difference (1.5 cm) versus those managed without tenotomy (0.7 cm). 43 idiopathic patients were suitable for OXFORD scoring at time of last review (i.e. older than 5 years old). The overall median OxFAQ was 96% with points lost only in the physical scale. Function in non-idiopathic cases was determined by their overall condition. Conclusion The idiopathic club foot is well treated by a physiotherapy-delivered Ponseti technique with excellent outcomes at 5 yr follow-up both subjectively and objectively.

Background Post-operative wound reviews constitute an essential part of care for children undergoing surgery, allowing prompt identification of post-operative complications. Until now it has been routine practice in the orthopaedic department to delegate wound reviews to community services. However, departmental follow-up may have significant benefits including improved continuity of care. Additionally COVID-19 has meant that avoiding face-to-face consultations has greater appeal, particularly for patients with significant co-morbidities. We developed a remote service for safe and patient-centred orthopaedic wound checks. Methods Stake-holder perspectives were captured by surveying parents as well as orthopaedic surgeons within the department. Suitable patients were identified and provided with a choice between remote wound checks (RWC’s) versus community services as standard. Patients opting for RWC’s were asked to upload photographs of their wounds to MyGOSH two weeks post discharge. Once images had been reviewed a telephone consultation took place during which the Bluebelle wound healing questionnaire was carried out. Parental satisfaction scores were gathered following telephone consultation. Results 100% of staff respondents supported a RWC service. A total of 7 patients were identified as suitable for this pilot, all opted for RWC over routine community follow-up. All 7 patients received a remote wound check within 2 weeks of discharge (mean 11.7 days). One parent reported visiting their GP prior to remote review citing low confidence with dressing removal. All patients had reassuring photos and Bluebelle scores requiring no further follow-up prior to routine clinic. The median Bluebelle score for the cohort was 3 (high score indicating wound complications, maximum 41). Mean satisfaction score amongst parents was 4.3 (out of 5). Conclusion RWC’s are desirable amongst our patient cohort. Parents engaged well with the remote service and satisfaction was high. Following this pilot we propose expansion of this service to empower families in the COVID-era and beyond.

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.

AimsTo analyze outcomes reported in studies of Ponseti correction of idiopathic clubfoot.MethodsA 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.ResultsIn 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.ConclusionsThere 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.

Purpose To summarize recent developments and provide recommendations as to whether universal or selective programmes are advisable. Methods A literature review was performed and preference given to studies with higher levels of evidence. All programmes reviewed included clinical screening. Results Recent studies underline the need for high quality screening programmes to promote the early detection of developmental dysplasia of the hip (DDH). A small number of cases may be missed clinically but with universal ultrasound screening programmes the late presentation rates appear to be virtually zero. Contemporary studies show treatment rates with universal screening programmes which are now lower than those with selective ultrasound. There is little agreement over the criteria used for selective programmes. Alternative outcome measures, such as the first operation rate or the percentage undergoing major (open) surgery are both lowest with universal ultrasound screening programmes. Furthermore, a significant reduction in the rate of surgery for DDH later in life was seen after the introduction of universal ultrasound screening, whereas the defined criteria for selective screening may not detect the majority of patients who require late surgery. Abduction bracing with modern orthoses is associated with a zero rate of avascular necrosis (AVN), whereas closed reduction techniques have an overall risk of 10%. Conclusion On clinical grounds, if future studies confirm that hip abduction in flexible orthoses is not associated with AVN, it may be time for a paradigm shift of screening for DDH towards a universal ultrasound protocol. The costs associated both with each type of screening programme and with the management of late presenting cases are also important but may be secondary to clinical benefit.

Objectives Extrathoracic solitary fibrous tumors (ESFTs) are rare low-to-intermediate grade spindle-cell neoplasms of pluripotent fibroblastic or myofibroblastic origin. This review explores prognostic factors in the management of ESFTs and provides guidance on optimal treatment regimens based on the current literature. Patients and methods Electronic searches were performed using MEDLINE, Embase, and the Cochrane library to identify studies on prognostic factors in the management of ESFTs published between January 1970 and June 2016. The literature search and review process identified 100 articles that were included in this review article. This included both surgical and nonsurgical studies on the management of ESFTs. Results Surgical excision with wide resection margins forms the mainstay of treatment and provides optimal long-term oncological outcomes. Large tumor size (>5 to 10 cm diameter), inadequate resection margins, malignant histologic features, dedifferentiation, and tumor location within the abdomen/pelvis are associated with adverse oncological outcomes. Radiotherapy may be used for preoperative tumor shrinkage and/or as adjuvant therapy in patients with malignant disease or incomplete surgical margins. Chemotherapy with molecular-targeted therapies has produced promising results and the results of further phase 2 trials are awaited. Conclusions Routine long-term follow-up is essential for benign and malignant disease to enable early detection and treatment of recurrent disease.

The mucopolysaccharidoses (MPS) are a group of inherited lysosomal storage disorders with clinical manifestations relevant to the orthopaedic surgeon. Our aim was to review the recent advances in their management and the implications for surgical practice. The current literature about MPSs is summarised, emphasising orthopaedic complications and their management. Recent advances in the diagnosis and management of MPSs include the recognition of slowly progressive, late presenting subtypes, developments in life-prolonging systemic treatment and potentially new indications for surgical treatment. The outcomes of surgery in these patients are not yet validated and some procedures have a high rate of complications which differ from those in patients who do not have a MPS. The diagnosis of a MPS should be considered in adolescents or young adults with a previously unrecognised dysplasia of the hip. Surgeons treating patients with a MPS should report their experience and studies should include the assessment of function and quality of life to guide treatment. Cite this article: Bone Joint J 2017;99-B:1132–9

Purpose Mucopolysaccharidoses (MPS) are a group of rare lysosomal storage disorders associated with involvement of multiple organs along with a generalised skeletal dysplasia. Both haematopoetic stem cell transplant and enzyme replacement therapy have improved the outlook for patients while surgery remains high-risk and there is little information on clinical or functional outcome to justify many of the surgical procedures performed. This paper aims to summarise the orthopaedic surgical procedures in MPS patients for which quality of life (QoL) and functional data are available and to describe additional QoL and functional measurement tools of relevance to the assessment of orthopaedic outcomes in MPS. Methods We reviewed the available literature to look for reported outcomes of orthopaedic surgery to lower and upper limbs and the spine. In addition, we describe the general and MPS-specific health measures that might be of relevance to the orthopaedic surgeon. Results There is some evidence in the literature that orthopaedic surgery may improve QoL and function in some specific aspects of the MPS condition (in relation to genu valgum, carpal tunnel syndrome and trigger digits); however, the literature is sparse and consists of level 4/5 studies only. Further studies of these conditions should include QoL and functional assessment in order to confirm or refute these reports. In other areas (spine and hip), outcomes are judged largely on radiographic appearances with little clinical correlation and short follow-up; however, one long-term study of function following hip dysplasia surgery suggests poor outcomes. Anaesthetic morbidity/mortality is not insignificant in these complex patients with multi-organ involvement. Careful assessment is required, particularly when there is neurological involvement. Conclusions Orthopaedic surgeons involved with MPS patients should be encouraged to use and report measures of QoL and function with respect to musculoskeletal manifestations and response to surgery, recognising that such assessments in these complex and challenging patients may require a multidisciplinary approach.

Purpose Complete fibula absence often presents with significant lower-limb deformity. Parental counselling regarding management is paramount in achieving the optimum functional outcome. Amputation offers a single surgical event with minimal complications. This study compares outcomes with an amputation protocol to those using an extension prosthesis. Method Thirty-two patients were identified. Nine patients (2 males, 7 females; median age at assessment of 23.5 years) used an extension prosthesis. Twenty-three patients (16 males, 7 females; median age at assessment of eight years) underwent 25 amputations during childhood. Mobility was assessed using SIGAM and K scores. Quality of life was assessed using the PedsQL inventory questionnaire; pain by a verbal severity score. Results The 19 Syme and one Boyd amputation in 19 patients were performed early (mean age 15 months). Four Syme and one trans-tibial amputation in four patients took place in older children (mean age 6.6 years). Only two underwent tibial kyphus correction to aid prosthetic fitting. K scores were significantly higher (mean 4 vs 2) and pain scores lower in the amputation group allowing high impact activity compared with community ambulation with an extension prosthesis. The SIGAM and PedsQL scores were all better in the amputation group, but not significantly so. Conclusion Childhood amputation for severe limb length inequality and foot deformity in congenital fibula absence offers excellent short-term functional outcome with prosthetic support. The tibial kyphus does not need routine correction and facilitates prosthetic suspension. Accommodative extension prostheses offer reasonable long-term function but outcome scores are lower.

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., In this Evidence-Based Guideline on X-linked hypophosphataemia, the authors identify the criteria for diagnosis of this disease, provide guidance for medical and surgical treatment and explain the challenges of follow-up.

The majority of patients with osteogenesis imperfecta (OI) have mutations in the COL1A1 or COL1A2 gene, which has consequences for the composition of the bone matrix and bone architecture. The mutations result in overmodified collagen molecules, thinner collagen fibres and hypermineralization of bone tissue at a bone matrix level. Trabecular bone in OI is characterized by a lower trabecular number and connectivity as well as a lower trabecular thickness and volumetric bone mass. Cortical bone shows a decreased cortical thickness with less mechanical anisotropy and an increased pore percentage as a result of increased osteocyte lacunae and vascular porosity. Most OI patients have mutations at different locations in the COL1 gene. Disease severity in OI is probably partly determined by the nature of the primary collagen defect and its location with respect to the C-terminus of the collagen protein. The overall bone biomechanics result in a relatively weak and brittle structure. Since this is a result of all of the above-mentioned factors as well as their interactions, there is considerable variation between patients, and accurate prediction on bone strength in the individual patient with OI is difficult. Current treatment of OI focuses on adequate vitamin-D levels and interventions in the bone turnover cycle with bisphosphonates. Bisphosphonates increase bone mineral density, but the evidence on improvement of clinical status remains limited. Effects of newer drugs such as antibodies against RANKL and sclerostin are currently under investigation. This paper was written under the guidance of the Study Group Genetics and Metabolic Diseases of the European Paediatric Orthopaedic Society. Cite this article: Nijhuis WH, Eastwood DM, Allgrove J, Hvid I, Weinans HH, Bank RA, Sakkers RJ. Current concepts in osteogenesis imperfecta: bone structure, biomechanics and medical management.

The muscular dystrophies (MD) are a group of inherited conditions characterized by a progressive muscle weakness and increasing disability. The most common and well-known is Duchenne muscular dystrophy (DMD). DMD is essentially the only MD associated with hip problems.

Rett syndrome is a neurodevelopmental disorder first described in 1966 (Rett, Wien Med Wochnschr 116:723–6, 1966). The characteristic picture includes a regression in learnt motor and social skills often with the later development of increased tone, muscle imbalance and subsequent joint contractures leading to scoliosis and hip subluxation. In the 50 years since the description of Rett Syndrome, progress has been made in understanding the underlying genetic abnormality and the accompanying effects on neurobiology and skeletal growth: in general, the more severe the genotype, the more severe the phenotype (Chahrour and Zoghbi, Neuron 56:422–37, 2007).

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. Electronic supplementary material The online version of this article (10.1186/s13023-019-0997-5) contains supplementary material, which is available to authorized users.

Aims Our aim was to assess the effectiveness of a protocol involving a standardised closed reduction for the treatment of children with developmental dysplasia of the hip (DDH) in maintaining reduction and to report the mid-term results. Methods A total of 133 hips in 120 children aged less than two years who underwent closed reduction, with a minimum follow-up of five years or until subsequent surgery, were included in the study. The protocol defines the criteria for an acceptable reduction and the indications for a concomitant soft-tissue release. All children were immobilised in a short- leg cast for three months. Arthrograms were undertaken at the time of closed reduction and six weeks later. Follow-up radiographs were taken at six months and one, two and five years later and at the latest follow-up. The Tönnis grade, acetabular index, Severin grade and signs of osteonecrosis were recorded. Results A total of 67 hips (51%) were Tönnis grade 3/4 hips. By 12 months, 20 reductions (15%) had not been maintained, and these required open reduction. In all, 55% of these were Severin 1; the others were Severin 2, due to minor acetabular dysplasia. Of the 113 successful closed reductions, 98 hips (87%) were Severin 1. Surgery for residual DDH was offered for ten hips. Osteonecrosis was seen in 32 hips (29%) but was transient in 28. In total, two children (1.5%) had severe osteonecrosis. Bilateral dislocations were significantly more likely to fail and most Tönnis 4 hips failed. Conclusion Closed reduction, with concomitant adductor and psoas release when required and the use of a short leg plaster of Paris cast for three months, can produce good mid-term results in children with DDH aged less than two years. This protocol is not recommended for Tönnis 4 hips. Cite this article: Bone Joint J 2016;98-B:1548–53.

Morquio-Brailsford syndrome or mucopolysaccharidosis type IV-A (MPS IV-A) is an inherited metabolic disease in which skeletal deformities can be extreme. An international registry has shown that 46% of patients are unable to walk 200 m and 30% use a wheelchair. Lower limb surgery is performed to restore alignment with the expectation of maintaining mobility. We are, however, not aware of reports correlating function with alignment. This study assesses the role of orthopaedic intervention with reference to anatomic and functional outcome in MPS IV-A: specifically hemiepiphysiodesis correction of genu valgum.Interrogation of the MPS patient database identified 63 patients. Patient demographic and radiographic data were collected along with information on deformity, correction, mobility, and genotype. Genu valgum was assessed by intermalleolar distance and mobility by a standardized 6-minute walk test. Sequential hip anatomy was assessed radiographically.Twenty-six surgical hemiepiphysiodesis episodes were performed on 23 patients. There were no cases of implant failure, loosening, or infection. The average intermalleolar distance reduction was 6.12 cm (paired t test, P=0.0001) (95% confidence interval, 7.6-4.7 cm) and the mean change in 6-minute walk test was +69.5 m (P=0.0339). There was no correlation between hip subluxation/dislocation and mobility (Fisher exact test, P=1.000), although hip parameters deteriorated over time. Three patients required repeat 8-plate insertion and 1 a femoral osteotomy.Eight-plate hemiepiphysiodesis correction of genu valgum in MPS IV-A is an effective treatment with low complication rates. The insult of surgery is minimal compared with that of osteotomy which can reduce mobility further. Our outcomes suggest that mobility is maintained or improved even in severe genotypes where mobility typically worsens. We have found hip pathology to be less debilitating, but further studies are needed to assess the effect of genu valgum correction upon femoral head subluxation and collapse.We suggest hemiepiphysiodesis should be considered as part of the treatment strategy for limb alignment in MPS IV-A patients, depending on remaining growth, mobility status, and genotype.Level IV-therapeutic case series.

Background The purpose of this study is to describe the natural history of hip morphology in patients with mucopolysaccharidoses (MPS) I and MPS II. Methodology This is a retrospective radiographic analysis of 88 hips in 44 children with MPS I and II. Radiographs were examined to determine hip migration, femoral head sphericity, and acetabular dysplasia at different ages throughout childhood. In individual hips, change in morphology and rate of change were analyzed. Results There was a high rate of hip migration and femoral head dysplasia in both MPS I and MPS II. Progressive migration was seen in three quarters of hips and progressive femoral head deformity in over half of hips. Acetabular dysplasia was variable, ranging from normal to severely dysplastic, but did not change with time. Overall, hips were more dysplastic in MPS I than MPS II. Conclusions Hip morphology is variable in MPS I and MPS II ranging from almost normal to severely dysplastic. Some hips do not deteriorate with time and thus surgical intervention may not be necessary in all cases. Deterioration is slow allowing time to plan a holistic approach to treatment. Level of evidence Level IV-case series.

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.

Retrospective case series OBJECTIVE.: The aim of this study was to review clinical and radiological outcomes of craniovertebral surgery in children with Morquio A syndrome (Mucopolysaccharidosis type IVA) and develop an evidence-based management algorithm.Myelopathy secondary to craniovertebral pathology is a common cause of neurological disability in Morquio A syndrome. Previously unresolved surgical controversies include the value of surveillance, surgical indications, and operative technique.A retrospective case-based review of children with Morquio A syndrome and craniovertebral pathology seen in a tertiary referral pediatric center from 1992 to 2016 was performed. Patients treated nonoperatively and operatively were included. Medical records and imaging were reviewed to determine clinical and radiological findings at initial assessment, before cervical spine surgery, early postoperative period, and final follow-up. The clinical outcomes of interest were neurological status and mobility at follow-up, complications, and need for further surgery.Twenty-seven patients were included. Surgical indications were radiological evidence of cervicomedullary compression alone (six cases) or with clinical evidence of myelopathy (12 cases). Eighteen patients (median age 6.2 years, range 3.5-15.9 years) underwent surgery, with median follow-up of 8.5 years. Occiput to upper cervical spine fusion with C1 decompression was performed in all cases with the addition of autologous calvarial graft in young patients (12 cases) and occipital-cervical plate fixation in older children (six cases). Neurological improvement occurred in 38% of cases but by one functional level only. Six of nine conservatively treated patients remained independent walkers.Surgery for craniovertebral pathology is required in the majority of children with Morquio A syndrome. Close clinical and radiological surveillance is essential for timely intervention. Occiput to cervical fusion is safe and feasible even in young patients and improves clinical and radiological parameters.4.

Despite preventative screening programmes in some countries, hip dislocation is still a common problem in many severely affected, non-ambulant patients with cerebral palsy. There are two scenarios where pain and stiffness affect all aspects of patient comfort and care and where a proximal femoral excision with an interpositional arthroplasty (myoplasty) must be considered: (1) the enlocated hip following hip reconstruction that is associated with a poor clinical outcome and (2) the dislocated hip, which due to anatomical or clinical factors is not reconstructible. Both indications are most common in patients assessed as IV or V on the Gross Motor Function Classification Scale (GMFCS).

Dysostosis multiplex is a progressive skeletal disease in patients with mucopolysaccharidosis type I (MPS I). One of its key features is thoracolumbar kyphosis and no guideline for surgical treatment is available yet. Therefore, we investigated the natural course of kyphosis and initiated an international consensus procedure with the aim to develop the first clinical practice guideline for the management of kyphosis in MPS I patients. Sequential radiographs (WKZ/UMC Utrecht and Amsterdam UMC, the Netherlands Royal Manchester Children’s Hospital, United Kingdom San Gerardo Hospital, Italy) were obtained and measurements included the kyphotic angle (modified Cobb angle (CA) and posterior angle (PA), both developed by us for measurement of kyphosis in dysplastic vertebrae) and spondylolisthesis. A modified Delphi procedure was used to develop statements with an international expert panel including 10 spinal/orthopedic surgeons, 6 metabolic pediatricians and 3 physiotherapists, all experienced in MPS I. The procedure consisted of 3 written rounds and a face-to-face meeting. In the natural history study 44 patients (233 radiographs) were included. Intra- and inter-rater reliability was ≥ 0.9 for all measurements. The median modified CA was larger in Hurler patients (38°) compared to non-Hurler patients (9°) and an abnormal modified CA was present at a younger age. A modeled trend showed a progression of the modified CA of 1.8°/year for Hurler patients. The modified CA and the PA correlated strongly. Spondylolisthesis was present in 34 patients. During the modified Delphi procedure 16 statements on decision for surgery and goals of treatment were developed. Consensus was reached on all statements. In conclusion, we present an extensive radiographic assessment of thoracolumbar kyphosis in MPS I patients showing kyphosis is progressive over time. These natural history data may be important for future studies. In addition, we present the first clinical practice guideline for management of kyphosis in MPS I patients.

Purpose Proximal femoral excision is a salvage procedure for painful chronic hip dislocation in cerebral palsy (CP) patients. The primary objective of this article is to describe our experience of an amplified interposition myoplasty, with appropriate peri-operative pain and tone management strategies, in a cohort of non-ambulatory CP patients with painful chronic hip dislocation. Our secondary objective is to present the clinical outcomes of these patients. Methods We describe our experience in 20 CP patients (25 procedures) at mean 54-month (range 27–169) follow-up with a surgical technique that includes an augmented interposition myoplasty and tone management. The indications for surgery were pain (21 hips), poor sitting tolerance (11) and difficulty with perineal care (8). Results The mean age was 22 years (range 10–40) with 11 patients Gross Motor Function Classification Scale (GMFCS) IV and 9 patients GMFCS V. Mean length of stay was 13 days (3–35). One procedure required revision at 12 months. Mean pain score improved from 7.8 (5–10) pre-operatively to 2.8 (1–5) post-operatively ( p < 0.001). Sitting tolerance improved in all patients and in 75 % (15) perineal care was easier. Conclusions Our interposition myoplasty technique with individualised pain/tone management has good outcomes in this cohort of patients with multiple co-morbidities.