X-Linked Hypophosphataemia (XLH) is a rare, progressive, hereditary phosphate-wasting disorder characterised by excessive activity of fibroblast growth factor 23. The International XLH Registry was established to provide information on the natural history of XLH and impact of treatment on patient outcomes. The cross-sectional orthopaedic data presented are from the first interim analysis. The XLH Registry (NCT03193476) was initiated in August 2017, aims to recruit 1,200 children and adults with XLH, and will run for 10 years. At the time of analysis (Last Patient In: 30/11/2020; Database Lock: 29/03/2021) 579 subjects diagnosed with XLH were enrolled from 81 hospital sites in 16 countries (360 (62.2%) children, 217 (37.5%) adults, and 2 subjects of unknown age). Of subjects with retrospective clinical data available, skeletal deficits were the most frequently self-reported clinical problems for children (223/239, 93.3%) and adults (79/110, 71.8%). Retrospective fracture data were available for 183 subjects (72 children, 111 adults); 50 had a fracture (9 children, 41 adults). In children, fractures tended to occur in tibia/fibula and/or wrist; only adults reported large bone fractures. Joint conditions were noted for 46 subjects (6 children, 40 adults). For adults reporting osteoarthritis, knees (60%), hips (42.5%), and shoulders (22.5%) were the most frequently affected joints. Retrospective orthopaedic surgery data were collected for 151 subjects (52 children, 99 adults). Osteotomy was the most frequent surgery reported (n=108); joint replacements were recorded for adults only. This is the largest set of orthopaedic data from XLH subjects collected to date. Longitudinal information collected during the 10-year Registry duration will generate real-world evidence which will help to inform clinical practice. Authors acknowledge the contribution of all International XLH Registry Steering Committee members

Excessive opioid prescriptions after total joint arthroplasty (TJA) increase risks for adverse opioid related events, chronic opioid use, and increase the availability of opioids for unlawful diversion. Thus, decreasing postoperative prescriptions may improve quality after TJA. Concerns exist that a decrease in opioids prescribed may increase complications such as readmissions, emergency department (ED) visits or worsened patient reported outcomes (PROs). The purpose of this quality improvement study was to explore whether a reduction in opioids prescribed after TJA resulted in increased complications. Methods: Data originated from a statewide arthroplasty database (MARCQI). The database collects over 96% of all TJA performed in the state of Michigan, USA. Data was prospectively abstracted and included OMEs prescribed at discharge, readmissions, ED visits within 30 days and PROs. Data was collected one year before and after the creation of an opioid prescribing protocol that had decreased prescriptions by approximately 50% in opioid naive and tolerant patients. Trends were monitored using Shewhart control charts. 84,998 TJA over two-years were included. All groups showed a reduction in opioids prescribed. Importantly, no increased complications occurred concomitant to this reduction. No increases in ED visits or readmissions, and no decreases in KOOSJR/HOOSJR/PROMIS10 scores were noted in any of the groups. Using large data sets and registries can drive performance and improve quality. The MARCQI Postoperative opioid prescription recommendations and performance measures decreased total oral morphine equivalents prescribed over a large and diverse population by approximately 50% without decreasing PROs or increasing ED visits or hospital readmissions. A reduction in opioids prescribed after TJA can be accomplished safely and without an increase in complications across a large population

Abstract. Introduction. Component mal-positioning in total hip replacement (THR) and total knee replacement (TKR) can increase the risk of revision for various reasons. Compared to conventional surgery, relatively improved accuracy of implant positioning can be achieved using computer assisted technologies including navigation, patient-specific jigs, and robotic systems. However, it is not known whether application of these technologies has improved prosthesis survival in the real-world. This study aimed to compare risk of revision for all-causes following primary THR and TKR, and revision for dislocation following primary THR performed using computer assisted technologies compared to conventional technique. Methods. We performed an observational study using National Joint Registry data. All adult patients undergoing primary THR and TKR for osteoarthritis between 01/04/2003 to 31/12/2020 were eligible. Patients who received metal-on-metal bearing THR were excluded. We generated propensity score weights, using Sturmer weight trimming, based on: age, gender, ASA grade, side, operation funding, year of surgery, approach, and fixation. Specific additional variables included position and bearing for THR and patellar resurfacing for TKR. For THR, effective sample sizes and duration of follow up for conventional versus computer-guided and robotic-assisted analyses were 9,379 and 10,600 procedures, and approximately 18 and 4 years, respectively. For TKR, effective sample sizes and durations of follow up for conventional versus computer-guided, patient-specific jigs, and robotic-assisted groups were 92,579 procedures over 18 years, 11,665 procedures over 8 years, and 644 procedures over 3 years, respectively. Outcomes were assessed using Kaplan-Meier analysis and expressed using hazard ratios (HR) and 95% confidence intervals (CI). Results. For THR, analysis comparing computer-guided versus conventional technique demonstrated HR of 0.771 (95%CI 0.573–1.036) p=0.085, and 0.594 (95%CI 0.297–1.190) p=0.142, for revision for all-causes and dislocation, respectively. When comparing robotic-assisted versus conventional technique, HR for revision for all-causes was 0.480 (95%CI 0.067 –3.452) p=0.466. For TKR, compared to conventional surgery, HR for all-cause revision for procedures performed using computer guidance and patient-specific jigs were 0.967 (95% CI 0.888–1.052) p=0.430, and 0.937 (95% CI 0.708–1.241) p=0.65, respectively. HR for analysis comparing robotic-assisted versus conventional technique was 2.0940 (0.2423, 18.0995) p = 0.50. Conclusions. This is the largest study investigating this topic utilising propensity score analysis methods. We did not find a statistically significant difference in revision for all-causes and dislocation although these analyses are underpowered to detect smaller differences in effect size between groups. Additional comparison for revision for dislocation between robotic-assisted versus conventionally performed THR was not performed as this is a subset of revision for all-causes and wide confidence intervals were already observed for that analysis. It is also important to mention this NJR analysis study is of an observational study design which has inherent limitations. Nonetheless, this is the most feasible study design to answer this research question requiring use of a large data set due to revision being a rare outcome. Declaration of Interest. (b) declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research reported:I declare that there is no conflict of interest that could be perceived as prejudicing the impartiality of the research project

This was a retrospective study of registry data from a National Orthopaedic Hospital for all THRs with 10-year follow-up data. Inclusion criteria were all THRs with a minimum of 10-year follow-up data. All metal-on-metal (MoM) THRs and MoM resurfacings were excluded from the analysis due to the high rate of revision associated with these bearings. Univariate and multivariate analyses controlling for confounding variables were performed to compare outcomes. A total of 1,697 THRs were performed in 1,553 patients. The four significant predictors for revision were fixation type (p<0.01), surface bearing type (p<0.01), age (P<0.05) and head size (p<0.05). Gender, BMI and approach had no effect on revision rates. The lowest 10-year all-cause revision rates were seen in cemented THRs at 1.7%. Ceramic-on-poly bearings had the lowest revision rate at only 1.2%. Metal-on-poly bearings had a 1.7% revision rate. Ceramic on ceramic bearings had a 7.1% revision rate with 1 revision for squeak and 1 revision for ceramic head fracture. The causes for revision in order of decreasing frequency were as follows: Infection (n=13, 0.7%), dislocation (n=7, 0.4%), periprosthetic fracture (n=3, 0.2%) and aseptic loosening (n=2, 0.1%). There were 2 re-revisions at 10 years in total. The smaller 22.225mm head sizes had a significantly lower revision rate than other head sizes (p<0.05). Ceramic-on-poly bearings, cemented fixation and smaller head sizes perform better in the experience of this registry. However, with multivariate analysis, these differences were shown to be insignificant

Patients ≤ 55 years have a high primary TKA revision rate compared to patients >55 years. Guided motion knee devices are commonly used in younger patients yet outcomes remain unknown. In this sub-group analysis of a large multicenter study, 254 TKAs with a second-generation guided motion knee implant were performed between 2011–2017 in 202 patients ≤ 55 years at seven US and three European sites. Revision rates were compared with Australian Joint Registry (AOANJRR) 2017 data. Average age 49.7 (range 18–54); 56.4% females; average BMI 34 kg/m2; 67.1% obese; patellae resurfaced in 98.4%. Average follow-up 4.2 years; longest follow-up six years; 27.5% followed-up for ≥ five years. Of eight revisions: total revision (one), tibial plate replacements (three), tibial insert exchanges (four). One tibial plate revision re-revised to total revision. Revision indications were mechanical loosening (n=2), infection (n=3), peri-prosthetic fracture (n=1), and instability (n=2). The Kaplan-Meier revision estimate was 3.4% (95% C.I. 1.7% to 6.7%) at five years compared to AOANJRR rate of 6.9%. There was no differential risk by sex. The revision rate of the second-generation guided motion knee system is lower in younger patients compared to registry controls

Bony tumours of the foot account for approximately 3% of all osseous tumours. However, literature regarding os calcis tumours comprises individual case reports, short case series or literature reviews, with the last large case series in 1973. We retrospectively reviewed the medical notes and imaging for all patients with calcaneal tumours recorded in the Scottish Bone Tumour Registry since the 1940's. Demographics, presentation, investigation, histology, management and outcome were reviewed. 38 calcaneal tumours were identified. Male to female ratio 2:1, mean age at presentation 30 with heel pain and swelling, average length of symptoms 9 months. 4 cases present with pathological fracture. 24 tumours benign including 6 unicameral bone cysts, 3 chondroblastoma, 3 PVNS with calcaneal erosion, and a wide variety of individual lesions. 13 malignant tumours comprising 6 osteosarcoma, 5 chondrosarcoma and 2 Ewings sarcoma. 1 metastatic carcinoma. Tumours of the calcaneus frequently are delayed in diagnosis due to their rarity and lack of clinician familiarity. They are more common in men and have a 1 in 3 risk of malignancy, covering a wide variety of lesions. Outcome is dependent on early diagnosis, timely surgery and most importantly neo-adjuvant chemotherapy. Diagnosis is often made on plain radiograph but MRI is the gold standard. We present the largest case series of calcaneal tumours, from our experience with the Scottish Bone Tumour Registry. Despite their rarity clinicians should maintain a high index of suspicion as accurate and timely diagnosis is important to management and outcome

Bizarre parosteal osteochondromatous proliferation (BPOP) is a benign lesion of bone originally described by Nora et al in 1983. To date there are no UK-based case series in the literature. Here we present the Scottish Bone Tumour Registry (SBTR) experience of this rare lesion. A retrospective analysis of SBTR records was performed. Histological specimens were re-examined by a consultant musculoskeletal oncology pathologist. Radiographs were re-reported by a consultant musculoskeletal radiologist. From 1983-2009, 13 cases were identified; 6 male, 7 female. Age ranged from 13-65. All patients presented with localised swelling. Pain was present in 5 and trauma in 2. 9 lesions affected the hand, 3 the foot, and 1 the tibial tuberosity. 12 lesions were excised and 1 curetted. There were 7 recurrences of which 6 were excised. 1 patients' recurrence was not treated. 1 lesion recurred a second time. This was excised. There were no metastases. Radiographs typically showed densely mineralised lesions contiguous with an uninvolved cortex. Cortical breakthrough was present in 1 case and scalloping in another. Histology characteristically showed hypercellular cartilage with pleomorphism and calcification/ossification without atypia; bone undergoing maturation; and a spindle-cell stroma. SBTR records indicate that BPOP is a rare lesion with no sex predilection that affects patients over a wide age range. Minor antecedent trauma was present in only 2 cases. In agreement with Nora et al. we feel that trauma is unlikely to represent an aetiological factor. Recurrence was over 50% in this series. Although this is similar to that found in other reports, it may indicate that more extensive resection is required for this aggressive lesion. Finally, although radiological/histological findings are often bizarre there have been no reported metastases and so it is important that BPOP is not mistaken for, or treated as, a malignant process such as chondrosarcoma

Primary bony tumours of the elbow account for approximately 1% of all osseous tumours. The delayed diagnosis is commonly reported in the literature as a result of lack of clinician familiarity. We present the largest series of primary bone tumours of the elbow in the English literature. We sought to identify characteristics specific to primary elbow tumours and compare these to the current literature. We discuss cases of misdiagnosis and reasons for any delay in diagnosis. The authors also recommend a collaborative protocol for the diagnosis and management of these rare tumours. A prospectively collected national database of all bone tumours is maintained by an independent clerk. The registry and case notes were retrospectively reviewed from January 1954 until June 2013. Eighty cases of primary osseous elbow tumours were studied. Tumours were classified as benign or malignant and then graded according to the Enneking spectrum. There were no benign latent cases in this series. All cases in this series required surgical intervention. These cases presented with persistent rest pain, with or without swelling. The distal humerus was responsible for the majority and most aggressive of cases. The multidisciplinary approach at a specialist centre is integral to management. Misdiagnosis was evident in 12.5 % of all cases. Malignant tumours carried a 5-year mortality of 61%. Benign tumours exhibited a 19% recurrence rate and in particular, giant cell tumour was very aggressive. The evolution in treatment modalities has clearly benefited patients. Clinicians should be aware that elbow tumours can be initially misdiagnosed as soft tissue injuries or cysts. The suspicion of a tumour should be raised in the patient with unremitting, unexplained non-mechanical bony elbow pain. We suggest an investigatory and treatment protocol to avoid a delay to diagnosis. With high rates of local recurrence, we recommend regular postoperative reviews

Primary bone tumours of the clavicle are rare. Currently the existing literature is limited to a single case series and case reports or cases. Information regarding the patient's demographics and tumour types is therefore limited.

The aim of this study was to investigate the and also suggest a management protocol for suspected primary bone tumours of the clavicle. We retrospectively reviewed the Scottish Bone Tumour Register from January 1971 to January 2012 and included all primary bone tumours of the clavicle.

We identified only sixteen primary bone tumours over forty one year's highlighting the rarity of these tumours. There were ten benign and six malignant tumours with a mean age of 32 years (Range 4 to 66). The average presentation to orthopaedics after onset of symptoms was two months with five patients presenting following a pathological fracture. Malignant tumour types identified were consistent with previous literature with two cases of Ewing's sarcoma and osteosarcoma and a single case of osteosarcoma post radiotherapy and a single case of chondrosarcoma. Benign tumours were treated effectively with intralesional procedures. Malignant tumours were treated with wide local excision and subtotal or total clavicle excision.

We suggest an investigatory and treatment protocol for patients with a suspected primary bone tumour of the clavicle. This is the largest series of primary bone tumours of the clavicle in the literature.

Primary bone tumours of the talus are rare. Currently the existing literature is limited to a single case series and case reports or cases described in series of foot tumours. Information regarding the patient's demographics and tumour types is therefore limited.

The aim of this study was to investigate these questions and also suggest a management protocol for suspected primary bone tumours of the talus. We retrospectively reviewed the Scottish Bone Tumour Register from January 1954 to May 2010 and included all primary bone tumours of the talus. We identified only twenty three bone tumours over fifty six years highlighting the rarity of these tumours. There were twenty benign and three malignant tumours with a mean age of twenty eight years. A delay in presentation was common with a mean time from onset of symptoms to diagnosis of ten months. Tumour types identified were consistent with previous literature. We identified cases of desmoplastic fibroma and intraosseous lipodystrophy described for the first time.

We suggest an investigatory and treatment protocol for patients with a suspected primary bone tumour of the talus. This is the largest series of primary bone tumours of the talus in the literature.