Aim. To assess the efficacy of combined medical and surgical management in obtaining normal lower limb mechanical alignment in a patient cohort with genotypically similar hypophosphataemic rickets. Methods. A notes and radiograph audit was performed of all patients attending our institution with hypophosphataemic rickets: a subset with PHEX gene anomalies was studied further. Lower limb radiographs were assessed at two points during childhood and note made of treatment start, compliance; indication, timing and result of surgery. Standing leg alignment radiographs were measured at skeletal maturity or at latest review. Results. 35 patients (16 females, 18 skeletally mature) were identified. 10 commenced treatment at <12m. 11 patients (5 female) underwent 24 surgical procedures (13 for varus deformities). Surgery was bilateral in 10/11 patients. 5/14 osteotomies were performed after skeletal maturity. Malalignment was common: with NSA (neck-shaft angle) abnormalities in 20%, abnormal angles at distal femur and proximal tibia in 58% and 60% respectively and ankle abnormalities in 24%, prior to surgery. Surgical management led to normal mechanical alignment at skeletal maturity. At latest review, no patient had a leg length difference. 40% of non-operated, skeletally immature limbs have significant malalignment despite medical therapy. There was no statistical relationship between treatment onset and need for surgical correction. The relationship between non-compliance with medical treatment and surgical intervention was confounded by the changing emphasis on preferred method of surgical correction from osteotomy to guided growth. Conclusion. Even when medical treatment is commenced promptly and adhered to, significant lower limb malalignment can occur requiring surgical correction. Guided growth principles allow early deformity correction. Significance. Lower limb malalignment should not be considered a failure of medical treatment but more a consequence of the disease process. Earlier surgical intervention may encourage a more normal pattern of growth

This study evaluated the effect of limb lengthening on longitudinal growth in patients with achondroplasia. Growth of the lower extremity was assessed retrospectively by serial radiographs in 35 skeletally immature patients with achondroplasia who underwent bilateral limb lengthening (Group 1), and in 12 skeletally immature patients with achondroplasia who did not (Group 2). In Group 1, 23 patients underwent only tibial lengthening (Group 1a) and 12 patients underwent tibial and femoral lengthening sequentially (Group 1b). The mean lengthening in the tibia was 9.2 cm (59.5%) in Group 1a, and 9.0 cm (58.2%) in the tibia and 10.2 cm (54.3%) in the femur in Group 1b. The mean follow-up was 9.3 years (8.6 to 10.3). The final mean total length of lower extremity in Group 1a was 526.6 mm (501.3 to 552.9) at the time of skeletal maturity and 610.1 mm (577.6 to 638.6) in Group 1b, compared with 457.0 mm (411.7 to 502.3) in Group 2. However, the mean actual length, representing the length solely grown from the physis without the length of distraction, showed that there was a significant disturbance of growth after limb lengthening. In Group 1a, a mean decrease of 22.4 mm (21.3 to 23.1) (4.9%) was observed in the actual limb length when compared with Group 2, and a greater mean decrease of 38.9 mm (37.2 to 40.8) (8.5%) was observed in Group 1b when compared with Group 2 at skeletal maturity. In Group 1, the mean actual limb length was 16.5 mm (15.8 to 17.2) (3.6%) shorter in Group 1b when compared with Group 1a at the time of skeletal maturity. Premature physeal closure was seen mostly in the proximal tibia and the distal femur with relative preservation of proximal femur and distal tibia. We suggest that significant disturbance of growth can occur after extensive limb lengthening in patients with achondroplasia, and therefore, this should be included in pre-operative counselling of these patients and their parents

This study aimed to define the rates of lower limb angular correction using temporary hemiepiphysiodesis in differing skeletal pathologies. A retrospective review of 61 children (36M:25F) with angular deformities about the knee who underwent 8-plate hemiepiphysiodesis (mean age 10.8y) was undertaken. The children were divided into 9 groups based on their underlying pathology (lower limb hypoplasia, Blount's disease, skeletal dysplasia, rickets, metabolic disease, acquired growth disturbance, vascular malformation, steroid use and complex genetic disorders). Radiographic measurements of each limb segment was undertaken using the TraumaCad® digital templating software based on standing long-leg radiographs - mechanical lateral distal femoral angle (mLDFA) and mechanical medial proximal tibial angle (mMPTA). The rate of correction of each parameter was calculated as a function of the time lapse between the operation date and first radiographic evidence of full correction of the mechanical axis (zone 1). A total of 144 limb segments (80 distal femoral, 64 proximal tibial physes) were analysed. 62.5% of children had mechanical axes outside the knee joint at the time of operation; 63.2% achieved full correction. The rate of angular correction at the distal femur (mLDFA) was quickest in those with acquired growth disturbance (1.15°/month), complex genetic disorders (1.12°/month) and rickets (0.93°/month). It was slowest in those with vascular malformation (0.40°/month), lower extremity hypoplasia (0.44°/month) and metabolic disease (0.49°/month). At the proximal tibia, mMPTA correction was quickest in those with acquired growth disturbance (0.77°/month) and skeletal dysplasia (0.57°/month); whilst being slowest in those with metabolic disease (0.22°/month) and Blount's disease (0.29°/month). The rate of angular correction about the knee varies with the underlying pathology with correction rates varying up to 3-fold. This study demonstrated the differential rate of correction of angular deformities in children with different skeletal pathologies, which would help guide the timing of hemiepiphysiodesis

Aim. The aim of the study was to characterise gait in patients with achondroplasia post lengthening. Patients/Materials and Methods. Full kinematic and kinetic lower limb gait analysis was performed at the Sheffield Children's Hospital gait laboratory, Sheffield, using a Vicon system (6 cameras working at 50Hz) and processed using Plug In Gait modelling software. The lengthened Achondroplasia group (n=11, mean age = 24.5 ± 6.1) had previously undergone surgical lengthening of the legs. The lengthened Achondroplasia group was compared to a control group of 11 adult normal subjects. Results. Averaged sagittal plane kinematics are shown in Figure 1. The key sagittal plane characteristics we found were increased anterior pelvic tilt (Achondroplasia mean = 22.2 ± 7.1°, normal mean = 14.2 ± 5.3°), decreased peak hip extension (Achondroplasia = 10.5 ± 11.2°, normal = −5.8 ± 5.3°), increased peak hip flexion (Achondroplasia = 49.3 ± 9.8°, normal = 40.0 ± 5.2°), and reduced knee extension in midstance (Achondroplasia = 14.0 ± 8.5°, normal = 4.4 ± 8.5°) At the ankle the mean of the lengthened Achondroplasia group lies within one standard deviation of the normal dataset throughout the gait cycle. Summary and Conclusions. Three dimensional gait analysis of people with Achondroplasia following lower limb-lengthening surgery showed characteristic Achondroplasic gait, that is: increased anterior pelvic tilt, reduced hip extension and increased hip flexion. The group of lengthened Achondroplasia participants also failed to extend the knees fully in midstance. At the ankle the sagittal plane kinematics were within one standard deviation of the normal dataset

The Sheffield Expanding Intramedullary Rod System was developed after experiencing problems with existing rod systems in the management of osteogenesis imperfecta. Between 1986 and 1996 we treated 74 bones in the lower limb in 28 children at a median follow-up of 5.25 years. We have reviewed 24 children with a total of 60 rods. Before surgery, all children had had multiple fractures of the lower limb. At review eight patients had experienced no further fractures, but three had suffered five or more subsequently. Before initial stabilisation, 15 children had never walked, and only three (13%) used walking as their main means of mobility. After surgery, half of those who showed motor arrest were able to walk (p = 0.016). The number of patients able to walk, with or without aids, increased to 17 (p = 0.0001). We have experienced no evidence of epiphyseal damage after the procedure, and complication rates requiring rod exchange have been low (7%)

We performed limb lengthening and correction of deformity of nine long bones of the lower limb in six children (mean age, 14.7 years) with osteogenesis imperfecta (OI). All had femoral lengthening and three also had ipsilateral tibial lengthening. Angular deformities were corrected simultaneously. Five limb segments were treated using a monolateral external fixator and four with the Ilizarov frame. In three children, lengthening was done over previously inserted femoral intramedullary rods. The mean lengthening achieved was 6.26 cm (mean healing index, 33.25 days/cm). Significant complications included one deep infection, one fracture of the femur and one anterior angulation deformity of the tibia. The abnormal bone of OI tolerated the external fixators throughout the period of lengthening without any episodes of migration of wires or pins through the soft bone. The regenerate bone formed within the time which is normally expected in limb-lengthening procedures performed for other conditions. We conclude that despite the abnormal bone characteristics, distraction osteogenesis to correct limb-length discrepancy and angular deformity can be performed safely in children with OI

Purpose

To examine the effect of limb lengthening on the quality of life of people who have disproportionate short stature (DSS).

Aims. The aim of this study is to develop a core set of outcome domains that should be considered and reported in all future trials of childhood limb fractures. Methods. A four-phase study was conducted to agree a set of core outcome domains. Identification of candidate outcome domains were identified through systematic review of trials, and outcome domains relevant to families were identified through semi-structured interviews with 20 families (parent-child pairing or group). Outcome domains were prioritized using an international three-round Delphi survey with 205 panellists and then condensed into a core outcome set through a consensus workshop with 30 stakeholders. Results. The systematic review and interviews identified 85 outcome domains as relevant to professionals or families. The Delphi survey prioritized 30 upper and 29 lower limb outcome domains at first round, an additional 17 upper and 18 lower limb outcomes at second round, and four additional outcomes for upper and lower limb at the third round as important domains. At the consensus workshop, the core outcome domains were agreed as: 1) pain and discomfort; 2) return to physical and recreational activities; 3) emotional and psychosocial wellbeing; 4) complications from the injury and treatment; 5) rturn to baseline activities daily living; 6) participation in learning; 7) appearance and deformity; and 8) time to union. In addition, 9a) recovery of mobility and 9b) recovery of manual dexterity was recommended as a core outcome for lower and upper limb fractures, respectively. Conclusion. This set of core outcome domains is recommended as a minimum set of outcomes to be reported in all trials. It is not an exhaustive set and further work is required to identify what outcome tools should be used to measure each of these outcomes. Adoption of this outcome set will improve the consistency of research for these children that can be combined for more meaningful meta-analyses and policy development. Cite this article: Bone Joint J 2021;103-B(12):1821–1830

Aims. Children with spinal dysraphism can develop various musculoskeletal deformities, necessitating a range of orthopaedic interventions, causing significant morbidity, and making considerable demands on resources. This systematic review aimed to identify what outcome measures have been reported in the literature for children with spinal dysraphism who undergo orthopaedic interventions involving the lower limbs. Methods. A PROSPERO-registered systematic literature review was performed following PRISMA guidelines. All relevant studies published until January 2023 were identified. Individual outcomes and outcome measurement tools were extracted verbatim. The measurement tools were assessed for reliability and validity, and all outcomes were grouped according to the Outcome Measures Recommended for use in Randomized Clinical Trials (OMERACT) filters. Results. From 91 eligible studies, 27 individual outcomes were identified, including those related to clinical assessment (n = 12), mobility (n = 4), adverse events (n = 6), investigations (n = 4), and miscellaneous (n = 1). Ten outcome measurement tools were identified, of which Hoffer’s Functional Ambulation Scale was the most commonly used. Several studies used unvalidated measurement tools originally developed for other conditions, and 26 studies developed new measurement tools. On the OMERACT filter, most outcomes reported pathophysiology and/or the impact on life. There were only six patient- or parent-reported outcomes, and none assessed the quality of life. Conclusion. The outcomes that were reported were heterogenous, lack validation and failed to incorporate patient or family perceptions. Until outcomes can be reported unequivocally, research in this area will remain limited. Our findings should guide the development of a core outcome set, which will allow consistency in the reporting of outcomes for this condition. Cite this article: Bone Joint J 2024;106-B(3):277–285

Aims. To analyze outcomes reported in trials of childhood fractures. Methods. OVID MEDLINE, Embase, and Cochrane CENTRAL databases were searched on the eighth August 2019. A manual search of trial registries, bibliographic review and internet search was used to identify additional studies. 11,476 studies were screened following PRISMA guidelines. 100 trials were included in the analysis. Data extraction was completed by two researchers for each trial. Study quality was not evaluated. Outcomes reported by trials were mapped onto domains in the World Health Organization (WHO) International Classification of Function framework. Results. In all, 525 outcomes were identified representing 52 WHO domains. Four domains were reported in more than 50% of trials: structure of upper/lower limb, sensation of pain, mobility of joint function, and health services, systems and policies. The Activities Scale for Kids performance (ASK-p) score was the most common outcome score reported in 6/72 upper limb and 4/28 lower limb trials. Conclusion. There is a diverse range of outcomes reported in trials of childhood fractures covering all areas in the International Classification of Functioning, Disability and Health (ICF) framework. There were three common upper limb and three common lower limb outcomes. In the absence of a core outcome set, we recommend that upper limb trials report pain, range of movement and radiograph appearance of the arm and lower limb trials report pain, radiograph appearance of the leg and healthcare costs to improve consistency of reporting in future trials. Cite this article: Bone Joint Open 2020;1-5:167–174

Aims. Paediatric fractures are highly prevalent and are most often treated with plaster. The application and removal of plaster is often an anxiety-inducing experience for children. Decreasing the anxiety level may improve the patients’ satisfaction and the quality of healthcare. Virtual reality (VR) has proven to effectively distract children and reduce their anxiety in other clinical settings, and it seems to have a similar effect during plaster treatment. This study aims to further investigate the effect of VR on the anxiety level of children with fractures who undergo plaster removal or replacement in the plaster room. Methods. A randomized controlled trial was conducted. A total of 255 patients were included, aged five to 17 years, who needed plaster treatment for a fracture of the upper or lower limb. Randomization was stratified for age (five to 11 and 12 to 17 years). The intervention group was distracted with VR goggles and headphones during the plaster treatment, whereas the control group received standard care. As the primary outcome, the post-procedural level of anxiety was measured with the Child Fear Scale (CFS). Secondary outcomes included the children’s anxiety reduction (difference between CFS after and CFS before plaster procedure), numerical rating scale (NRS) pain, NRS satisfaction of the children and accompanying parents/guardians, and the children’s heart rates during the procedure. An independent-samples t-test and Mann-Whitney U test (depending on the data distribution) were used to analyze the data. Results. The post-procedural CFS was significantly lower (p < 0.001) in the intervention group (proportion of children with no anxiety = 78.6%) than in the control group (56.8%). The anxiety reduction, NRS pain and satisfaction scores, and heart rates showed no significant differences between the control group and the intervention group. Subanalyses showed an increased effect of VR on anxiety levels in young patients, females, upper limb fractures, and those who had had previous plaster treatment. Conclusion. VR effectively reduces the anxiety levels of children in the plaster room, especially in young girls. No statistically significant effects were seen regarding pain, heart rate, or satisfaction scores. Cite this article: Bone Joint J 2024;106-B(7):728–734

Aims. The study was undertaken to compare the efficacy of Woodcast splints and plaster-of-Paris casts in maintaining correction following sequential manipulation of idiopathic clubfeet. Methods. In this randomized prospective trial, 23 idiopathic clubfeet were immobilized with plaster-of-Paris casts and 23 clubfeet were immobilized with a splint made of Woodcast that encircled only two-thirds the circumference of the limb. The number of casts or splints needed to obtain full correction, the frequency of cast or splint-related complications, and the time taken for application and removal of the casts and splints were compared. Results. The mean number of casts required to obtain full correction of the deformity (Pirani Score 0) was 4.35 (95% confidence interval (CI) 3.74 to 4.95) when plaster-of-Paris was used and 4.87 (95% CI 4.33 to 5.41) when the Woodcast splint was used (p = 0.190). The time required for application and removal of the Woodcast splint were significantly less than that required for application and removal of plaster-of-Paris casts (p < 0.001). Woodcast splint-related complications were not more frequent than plaster-of-Paris cast related complications. Conclusion. Though Woodcast splints covering two-thirds of the circumference of the lower limbs of infants were effective in maintaining the correction of clubfoot deformity during serial manipulation and casting treatment, the superiority of Woodcasts over plaster-of-Paris could not be established. Cite this article: Bone Joint J 2020;102-B(10):1399–1404

Aims. The health-related quality of life (HRQoL) of paediatric patients with orthopaedic conditions and spinal deformity is important, but existing generic tools have their shortcomings. We aim to evaluate the use of Paediatric Quality of Life Inventory (PedsQL) 4.0 generic core scales in the paediatric population with specific comparisons between those with spinal and limb pathologies, and to explore the feasibility of using PedsQL for studying scoliosis patients’ HRQoL. Methods. Paediatric patients attending a speciality outpatient clinic were recruited through consecutive sampling. Two groups of patients were included: idiopathic scoliosis, and paediatric orthopaedic upper and lower limb condition without scoliosis. Patients were asked to complete PedsQL 4.0 generic core scales, Youth version of 5-level EuroQol-5-dimension questionnaire, and Refined Scoliosis Research Society 22-item (SRS-22r) questionnaire. Statistical analyses included scores comparison between scoliosis and limb pathology patients using independent-samples t-test, and correlation tests of PedsQL and SRS-22r. Results. A total of 566 paediatric patients were recruited: 357 (63.0%) having idiopathic scoliosis, and 209 (37.0%) with limb conditions. Patients with limb pathology had lower functioning scale, summary, and total scores of PedsQL than scoliosis patients (p < 0.05 to p < 0.001). No floor or ceiling effects (< 15%) were detected for PedsQL Psychosocial Health Summary and total scores in both groups. PedsQL was sensitive in differentiating patients with/without problems in their daily lives (p < 0.05 to p < 0.01). PedsQL summary and total scores correlated with SRS-22r total score. Conclusion. PedsQL is an effective HRQoL measure for both paediatric orthopaedic groups with minimal ceiling and floor effects, and is capable of detecting worse HRQoL in patients with limb pathology. The multidimensional PedsQL is sensitive in differentiating among those with daily life problems, especially for scoliosis patients. Cite this article: Bone Joint J 2020;102-B(7):890–898

Aims. There is increasing evidence that flexible flatfoot (FF) can lead to symptoms and impairment in health-related quality of life. As such we undertook an observational study investigating the aetiology of this condition, to help inform management. The hypothesis was that as well as increased body mass index (BMI) and increased flexibility of the lower limb, an absent anterior subtalar articulation would be associated with a flatter foot posture. . Patients and Methods. A total of 84 children aged between eight and 15 years old were prospectively recruited. The BMI for each child was calculated, flexibility was assessed using the lower limb assessment scale (LLAS) and foot posture was quantified using the arch height index (AHI). Each child underwent a sagittal T1-weighted MRI scan of at least one foot. . Results. An absent anterior subtalar articulation (p < 0.001) and increased LLAS (p = 0.001) predicted a low AHI. BMI was not a significant predictive factor (p = 0.566). . Conclusion. This is the first study to demonstrate the importance of the morphology of the subtalar joint on the underlying foot posture in vivo. . Take home message: Flexibility of the lower limb and absence of the anterior facet of the subtalar joint are associated with flexible FF and may influence management of this common condition. Cite this article: Bone Joint J 2016;98-B:564–8

Aims. The Fassier Duval (FD) rod is a third-generation telescopic implant for children with osteogenesis imperfecta (OI). Threaded fixation enables proximal insertion without opening the knee or ankle joint. We have reviewed our combined two-centre experience with this implant. Methods. In total, 34 children with a mean age of five years (1 to 14) with severe OI have undergone rodding of 72 lower limb long bones (27 tibial, 45 femoral) for recurrent fractures with progressive deformity despite optimized bone health and bisphosphonate therapy. Data were collected prospectively, with 1.5 to 11 years follow-up. Results. A total of 24 patients (33%) required exchange of implants (14 femora and ten tibiae) including 11 rods bending with refracture. Four (5%) required reoperation with implant retention. Loss of proximal fixation in the femur and distal fixation in the tibia were common. Four patients developed coxa vara requiring surgical correction. In total, 13 patients experienced further fractures without rod bending; eight required implant revision. There was one deep infection. The five-year survival rate, with rod revision as the endpoint, was 63% (95% confidence interval (CI) 44% to 77%) for femoral rods, with a mean age at implantation of 4.8 years (1.3 to 14.8), and 64% (95% CI 36% to 82%) for tibial rods, with a mean age at implantation of 5.2 years (2.0 to 13.8). Conclusion. FD rods are easier to implant but do not improve on the revision rates reported for second generation T-piece rods. Proximal femoral fixation is problematic in younger children with a partially ossified greater trochanter. Distal tibial fixation typically fails after two years. Future generation implants should address proximal femoral and distal tibial fixation to avoid the majority of complications in this series. Cite this article: Bone Joint J 2020;102-B(8):1048–1055

Aims. Perthes’ disease is a condition which leads to necrosis of the femoral head. It is most commonly reported in children aged four to nine years, with recent statistics suggesting it affects around five per 100,000 children in the UK. Current treatment for the condition aims to maintain the best possible environment for the disease process to run its natural course. Management typically includes physiotherapy with or without surgical intervention. Physiotherapy intervention often will include strengthening/stretching programmes, exercise/activity advice, and, in some centres, will include intervention, such as hydrotherapy. There is significant variation in care with no consensus on which treatment option is best. The importance of work in this area has been demonstrated by the British Society for Children’s Orthopaedic Surgery through the James Lind Alliance’s prioritization of work to determine/identify surgical versus non-surgical management of Perthes’ disease. It was identified as the fourth-highest priority for paediatric lower limb surgery research in 2018. Methods. Five UK NHS centres, including those from the NEWS (North, East, West and South Yorkshire) orthopaedic group, contributed to this case review, with each entre providing clinical data from a minimum of five children. Information regarding both orthopaedic and physiotherapeutic management over a two-year post-diagnosis period was reviewed. Results. Data were extracted from the clinical records of 32 children diagnosed with Perthes’ disease; seven boys and 25 girls. The mean age of the children at diagnosis was 6.16 years (standard deviation (SD) 3.001). In all, 26 children were referred for physiotherapy. In the two-year period following diagnosis, children were seen a median of 7.5 times (interquartile range (IQR) 4.25 to 11) by an orthopaedic surgeon, and a median of 9.5 times (IQR 8 to 18.25) by a physiotherapist. One centre had operated on all of their children, while another had operated on none. Overall, 17 (53%) of the children were managed conservatively in the two-year follow-up period, and 15 (47%) of the children underwent surgery in the two-year follow-up period. Conclusion. The results of this case review demonstrate a variation of care provided to children in the UK with Perthes’ disease. Further national and international understanding of current care is required to underpin the rationale for different treatment options in children with Perthes’ disease. Cite this article: Bone Joint Open 2020;1-11:691–695

Aims

Within healthcare, several measures are used to quantify and compare the severity of health conditions. Two common measures are disability weight (DW), a context-independent value representing severity of a health state, and utility weight (UW), a context-dependent measure of health-related quality of life. Neither of these measures have previously been determined for developmental dysplasia of the hip (DDH). The aim of this study is to determine the DW and country-specific UWs for DDH.

Aims

The aim of this study was to investigate the agreement in interpretation of the quality of the paediatric hip ultrasound examination, the reliability of geometric and morphological assessment, and the relationship between these measurements.

Aims

The aim of this study was to gain a consensus for best practice of the assessment and management of children with idiopathic toe walking (ITW) in order to provide a benchmark for practitioners and guide the best consistent care.

Aims

Accurate skeletal age and final adult height prediction methods in paediatric orthopaedics are crucial for determining optimal timing of growth-guiding interventions and minimizing complications in treatments of various conditions. This study aimed to evaluate the accuracy of final adult height predictions using the central peak height (CPH) method with long leg X-rays and four different multiplier tables.