Major Orthopaedic Surgery in Persons with Haemophilia A with and without Inhibitors Treated by Emicizumab: A Mid-Term, Large, and Successful Series at a Single Center.

Introduction: Patients with Haemophilia (PWH) need orthopaedic treatments and often they undergo surgery. Classically, PWH with inhibitors have to face such procedures earlier than other patients. Major orthopaedic surgery is not easy and complications are frequent. Emicizumab is the first monoclonal antibody introduced for haematological prophylaxis for PWH with inhibitors, achieving an efficacious haemostasis also in patients with severe haemophilia A with inhibitors, later demonstrated for PWH without inhibitors. A few years ago, emicizumab was also proposed for PWH undergoing surgery, as it supports excellent bleeding control. The literature on orthopaedic surgery using an emicizumab protocol is scarce: only isolated case reports with short-term follow-ups are available. Aim: The purpose of this study is the assessment of the mid-term outcomes of major orthopaedic surgery performed in a population of patients with and without inhibitors and an emicizumab regimen. Methods: We reviewed the records of 13 PWH (eight with high-titre inhibitors, five without) with a mean age of 54.6 years, undergoing 15 orthopaedic surgical procedures between 2017 and 2022: primary knee and hip arthroplasty, revision, pseudotumor excision, or amputation. Their prophylaxis consisted of the combination of emicizumab and boluses of rFVIIa (PWH with inhibitors) or rFVIII (PWH without inhibitors). The clinical parameters of evaluation were: VAS, Haemophilic Joint Health Score (HJHS), and standard radiologic studies. Follow-up was conducted at 1, 3, 6 months, and then yearly. The survival rate of all implants was also assessed. Results: The mean follow-up was 38.8 months (range: 12-65). All patients were successfully treated without complications during surgery. During the postoperative period, a patient affected by a septic complication two months after his pseudotumor excision underwent an above-the-knee amputation. All patients were regularly discharged to the rehabilitative ward, reporting satisfaction for pain reduction and improved joint and global function at the VAS and HJHS scores. No revisions or implant failures were recorded. Conclusions: A prophylaxis regimen with emicizumab and factor replacement in PWH with or without inhibitors undergoing major orthopaedic surgery ensures effective bleeding control and good postoperative clinical outcomes at mid-term follow-up, and may be routinely adopted in dedicated high-volume hospitals. This series is the most consistent to date reported at a single Haemophilia centre.

A dual telescoping lag screw nailing system for intertrochanteric fractures: retrospective analysis of clinical and radiologic outcomes.

BACKGROUND AND PURPOSE: The aim of this study was to evaluate whether this system is associated with a reduced rate of failure and complications in patients treated for proximal femoral fractures with intramedullary nailing. MATERIALS AND METHODS: 742 Patients with AO-OTA 31-A intertrochanteric fractures were enrolled at a single Institution. Functional evaluation was assessed through the Functional Independence Measure (FIM™) instrument and Parker's New Mobility Score (NMS). Radiological follow-up included the degree of the reduction according to the Baumgartner criteria, the Tip-Apex Distance, and the shortening of the telescoping screws and its lateral protrusion. RESULTS: Pre-operative mean FIM™ and NMS were 4.3 (range 1-9) and 98.7 (range 22-126), respectively. At the 12-month follow-up the average FIM™ and NMS were 95.3 (range 22-126) and 3.7 (range 1-9), respectively. Mean shortening of the lag screws was 4.3 mm (range 1-8) and mean lateral protrusion was 1.7 mm (range 0-3). 3 Cases (0.70%) of non-consolidation requiring reoperation were recorded. 1 Case (0.24%) of these cases was also characterized by nail breakage. No case of cut-out has been reported at our follow-up. CONCLUSIONS: This dual telescoping nail system is effective and safe. The sliding of the telescoping screws within the barrel is able to decrease strain from the femoral head during weight bearing reducing the risk of cut-out.

The value-based healthcare approach to haemophilia: Development of outcome measures for the evaluation of care of people with haemophilia.

INTRODUCTION: Considering the advances in haemophilia management and treatment observed in the last decades, a new set of value-based outcome indicators is needed to assess the quality of care and the impact of these medical innovations. AIM: The Value-Based Healthcare in Haemophilia project aimed to define a set of clinical outcome indicators (COIs) and patient-reported outcome indicators (PROIs) to assess quality of care in haemophilia in high-income countries with a value-based approach to inform and guide the decision-making process. METHODS: A Value-based healthcare approach based on the available literature, current guidelines and the involvement of a multidisciplinary group of experts was applied to generate a set of indicators to assess the quality of care of haemophilia. RESULTS: A final list of three COIs and five PROIs was created and validated. The identified COIs focus on two domains: musculoskeletal health and function, and safety. The identified PROIs cover five domains: bleeding frequency, pain, mobility and physical activities, Health-Related Quality of Life and satisfaction. Finally, two composite outcomes, one based on COIs, and one based on PROIs, were proposed as synthetic outcome indicators of quality of care. CONCLUSION: The presented standard set of health outcome indicators provides the basis for harmonised longitudinal and cross-sectional monitoring and comparison. The implementation of this value-based approach would enable a more robust assessment of quality of care in haemophilia, within a framework of continuous treatment improvements with potential added value for patients. Moreover, proposed COIs and PROIs should be reviewed and updated routinely.

Accuracy of radiographic projections to guide cephalic screw position in pertrochanteric fracture: a cadaveric study.

PURPOSE: The aim of this study was to evaluate the relationship between the Löwenstein Lateral view and the True Lateral view for the positioning of the cephalic hip screw, through a cadaveric study. MATERIALS AND METHODS: We placed two Kirschner wires in eight femur specimens using an Antero-Posterior view, Löwenstein Lateral view and True Lateral view. The distances between the Kirschner wires and the anterior, posterior, superior and inferior cortex were measured in all projections. The head of the femur was then sectioned, and the same macroscopic distances were measured. Finally, we could calculate the accuracy of the two radiographic lateral projections. RESULTS: When the Kirschner wire was placed in the center of the head using the Antero-Posterior and the True Lateral view, the accuracy of Antero-Posterior view was 0.9705 while the accuracy of True Lateral view and Löwenstein Lateral view was 1.1479 and 1.1584, respectively. When the Kirschner wire was placed superior on the Antero-Posterior and centrally on the True Lateral view, the accuracy of Antero-Posterior view was 0.9930 while the accuracy of True Lateral view and Löwenstein Lateral view was 1.1159 and 0.7224, respectively. CONCLUSION: When the Kirschner wire was positioned proximal in Antero-Posterior view and central in True Lateral view, only the True Lateral view showed high accuracy.

Groin Pain Syndrome Italian Consensus Conference update 2023.

Groin pain syndrome (GPS) is a controversial topic in Sports Medicine. The GPS Italian Consensus Conference on terminology, clinical evaluation and imaging assessment of groin pain in athletes was organized by the Italian Society of Arthroscopy in Milan, on 5 February 2016. In this Consensus Conference (CC) GPS etiology was divided into 11 different categories for a total of 63 pathologies. The GPS Italian Consensus Conference update 2023 is an update of the 2016 CC. The CC was based on a sequential, two-round online Delphi survey, followed by a final CC in the presence of all panelists. The panel was composed of 55 experts from different scientific and clinical backgrounds. Each expert discussed 6 different documents, one of which regarded the clinical and imaging definition of sports hernias, and the other 5 dealt with 5 new clinical situations thought to result in GPS. The panelists came to an agreement on the definition of a sports hernia. Furthermore, an agreement was reached, recognizing 4 of the 5 possible proposed pathologies as causes to GPS. On the contrary, the sixth pathology discussed did not find consensus given the insufficient evidence in the available scientific literature. The final document includes a new clinical and imaging definition of sports hernia. Furthermore, the etiology of GPS was updated compared to the previous CC of 2016. The new taxonomic classification includes 12 categories (versus 11 in the previous CC) and 67 pathologies (versus 63 in the previous CC).

Total Knee Arthroplasty in Haemophilia: Long-Term Results and Survival Rate of a Modern Knee Implant with an Oxidized Zirconium Femoral Component.

(1) Background: Total Knee Arthroplasty (TKA) in patient with haemophilia (PWH) has usually been performed with the use of cobalt-chrome femoral and titanium tibial components, coupled with standard polyethylene (PE) inserts. The aim of this retrospective study was to evaluate the long-term outcomes and survival rates of TKA in a series of consecutive PWH affected by severe knee arthropathy at a single institution. (2) Methods: We followed 65 patients undergoing 91 TKA, implanted using the same implant, characterized by an oxidized zirconium femoral component, coupled with a titanium tibial component, and a highly crosslinked PE. At 1, 6, and 12 months; then every year for 5 years; and finally, every other 3 years, all patients were scored for pain (VAS), function (HJHS; KSS), ROM, and radiographic changes. Kaplan-Meier survivorship curves were used to calculate the implant survival rates. (3) Results: The mean follow-up was 12.3 years (4.2-20.6). All clinical and functional scores improved significantly from preoperatively to the latest follow-up (VAS: from 6.9 to 1.3; HJHS: from 13.4 to 1.9; KSS: from 19.4 to 79; ROM: from 42.4° to 83.6°). The overall survivorship of the implants was 97.5% at the latest follow-up. (4) Conclusions: The present series showed a high survival rate of specific implants potentially linked to the choice of an oxidized zirconium coupled with a highly crosslinked PE. We promote the use of modern implants in these patients in order to ensure long-lasting positive outcomes.

Managing Relevant Clinical Conditions of Hemophilia A/B Patients.

The Medical Directors of nine Italian Hemophilia Centers reviewed and discussed the key issues concerning the replacement therapy of hemophilia patients during a one-day consensus conference held in Rome one year ago. Particular attention was paid to the replacement therapy needed for surgery using continuous infusion (CI) versus bolus injection (BI) of standard and extended half-life Factor VIII (FVIII) concentrates in severe hemophilia A patients. Among the side effects, the risk of development of neutralizing antibodies (inhibitors) and thromboembolic complications was addressed. The specific needs of mild hemophilia A patients were described, as well as the usage of bypassing agents to treat patients with high-responding inhibitors. Young hemophilia A patients may take significant advantages from primary prophylaxis three times or twice weekly, even with standard half-life (SHL) rFVIII concentrates. Patients affected by severe hemophilia B probably have a less severe clinical phenotype than severe hemophilia A patients, and in about 30% of cases may undergo weekly prophylaxis with an rFIX SHL concentrate. The prevalence of missense mutations in 55% of severe hemophilia B patients allows the synthesis of a partially changed FIX molecule that can play some hemostatic role at the level of endothelial cells or the subendothelial matrix. The flow back of infused rFIX from the extravascular to the plasma compartment allows a very long half-life of about 30 h in some hemophilia B patients. Once weekly, prophylaxis can assure a superior quality of life in a large severe or moderate hemophilia B population. According to the Italian registry of surgery, hemophilia B patients undergo joint replacement by arthroplasty less frequently than hemophilia A patients. Finally, the relationships between FVIII/IX genotypes and the pharmacokinetics of clotting factor concentrates have been investigated.

Safe and Successful Surgical Outcome in Persons with Hemophilia A with and without Inhibitors Treated with Emicizumab: A Large, Single Center, Real-World Experience.

Emicizumab is a humanized recombinant bispecific antibody, bridging together activated factor IX (FIXa) and factor X (FX), thus mimicking the activity of FVIII in vivo. Emicizumab is designed for long-term prophylaxis in patients with severe hemophilia A with and without inhibitors. This approach provides constant protection, with significant reduction in bleeding rate and improved quality of life. However, protection provided by emicizumab is not absolute, and clotting factor concentrates (FVIII, rFVIIa, aPCC) may be necessary for post-traumatic bleeding or surgery, with a potential thrombotic risk or difficulty in preventing bleeding. Real world evidence is still scanty, especially for managing major surgery. In this study, 75 surgeries were managed in 28 patients (27 major procedures in 15 patients and 48 minor procedures in 20 patients. In 17 patients without inhibitors, 30 minor surgeries were carried out by using FVIII in 5, with only a bleeding event, which was successfully treated with FVIII concentrate. Six major surgeries were uneventfully performed with FVIII concentrate. Eleven PWHA and high-titer inhibitors underwent 39 surgical procedures (18 minor and 21 major surgeries). Minor surgeries were mostly performed without prophylaxis with rFVIIa, with only a single bleeding complication. All 21 major surgeries were covered with a homogeneous protocol using rFVIIa. In four instances, bleeding complications occurred, treated with rFVIIa. Of them, a single patient only failed to respond and died because of an uncontrollable bleeding from a large ruptured retroperitoneal pseudotumor. Surgery in patients with emicizumab can be safely carried out with the use of appropriate replacement therapy protocols.

Acetabular Morphology Predicts the Risk of Dislocation Following Hemiarthroplasty for Femoral Neck Fractures in the Elderly.

BACKGROUND: Hip hemiarthroplasty dislocation is a devastating complication. Among other preoperative risk factors, acetabular morphology has been rarely studied. The purpose of the study was to evaluate the influence of preoperative native acetabular morphology on hemiarthroplasty dislocation. METHODS: We retrospectively reviewed 867 patients who underwent hip hemiarthroplasty for femoral neck fracture between January 1, 2014 and January 1, 2019. The 380 included patients were treated with an anterior-based muscle-sparing approach. The central-edge angle (CEA) and acetabular depth-to-width ratio (ADWR) of the fractured hip were measured preoperatively on the anteroposterior pelvic view. Receiver operating characteristic curves were performed to analyze the optimal cutoff for CEA and ADWR. Hemiarthroplasty dislocation occurred in 18 patients (4.7%), and the remaining 362 patients were used as the control group. RESULTS: No significant differences in terms of sex, age, dementia, neuromuscular disease, and body mass index were found between the 2 groups. The 18 patients who had a hip dislocation had significantly smaller mean CEA than the control group (P = .0001) (mean 36.1 ± 7.5° and 43.2 ± 5.6°, respectively) as well as ADWR (mean 34 ± 6 versus 37 ± 4, respectively) (P = .001). Using the receiver operating characteristic analysis, we report significant cutoffs of 38.5° for CEA (P = .0001) and 34.5 for the ADWR (P = .017). CONCLUSION: Higher rates of hemiarthroplasty dislocation were observed in patients who had a preoperative CEA of less than 38.5° and an ADWR of less than 34.5. Patients who have preoperative acetabular morphological risk factors for dislocation might be better candidates for a total hip arthroplasty.

Laying the foundations for gene therapy in Italy for patients with haemophilia A: A Delphi consensus study.

INTRODUCTION: Current treatment for haemophilia A involves factor VIII replacement or non-replacement (emicizumab) therapies, neither of which permanently normalise factor VIII levels. Gene therapy using adeno-associated viral (AAV) vectors is an emerging long-term treatment strategy for people with severe haemophilia A (PwSHA) that is likely to be available for clinical use in the near future. AIM: This article proposes practical guidelines for the assessment, treatment, and follow-up of potential PwSHA candidates for AAV-based gene therapy. METHOD: Using the Delphi method, a working group of Italian stakeholders with expertise in and knowledge of the care of adults with haemophilia A analysed literature for AAV-based gene therapy and drafted a list of statements that were circulated to a panel of Italian peers. During two rounds of voting, panel members voted on their agreement with each statement to reach a consensus. RESULTS: The Delphi process yielded 40 statements regarding haemophilia A gene therapy, across five topics: (1) organisational model; (2) multidisciplinary team; (3) patient engagement; (4) laboratory surveillance; and (5) patient follow-up and gene therapy outcomes. The consensus was reached for all 40 statements, with the second round of voting needed for five statements. CONCLUSION: Use of the hub-and-spoke organisational model and multidisciplinary teams are expected to optimise patient selection for gene therapy, as well as the management of dosing and patient follow-up, patient engagement, laboratory surveillance, and patient expectations regarding outcomes. This approach should allow the benefits of AAV-based gene therapy for haemophilia A to be maximised.

The lesser trochanter "Sling fixation technique" in proximal intramedullary nailing of unstable intertrochanteric fractures: A polymer-based cerclage wiring.

Introduction: Lesser trochanter (LT) fixation to restore the continuity of the posteromedial fragment using a cerclage wire has been reported to provide a more stable reduction in the treatment of unstable femoral neck fractures with LT involvement. However, LT fixation is not commonly performed due to the complications associated with the traditional monofilament cerclage metal wires. In this study, we investigate the clinical and radiological outcomes of a new technique for LT fixation in conjunction with Intramedullary (IM) fixation with nail and screws when compared to isolated IM fixation. The Sling Fixation Technique involves a new wiring technique and the use of a polymer-based cerclage cable characterized by iso-elastic properties that is hypothesized to provide better clinical and radiographic outcomes. Patients and methods: This prospective observational study included 30 patients who underwent proximal IM nailing from July 2019 to April 2020. Thirty consecutive patients (30) were assigned to 2 different treatment groups. Fifteen (15) patients were treated with the IM Nailing and Sling Fixation Technique and 15 with IM nailing only. Patients were comparable for demographic features, fracture pattern, age, gender and associated medical comorbidities. Clinical outcomes were analyzed in terms of time needed to achieve complete weight bearing, Harris hip score (HHS), Barthel Index (BI) and Radiographic Union Scale for Hip (RUSH). The follow-up period was one year. Results: Time for fracture healing and to achieve weight-bearing in the "Sling" group were shown to be significantly shorter than in the IM group. The Sling group showed furthermore better HHS, BI and RUSH scores when compared to the IM group at 1, 3, 6, and 12 months from the surgery. No complications such as wiring breakage, cut-outs, breakage or pullout of the fixation screws were observed; one case of heterotrophic ossification was reported. Conclusion: In cases of unstable intertrochanteric fractures of the proximal femur with lesser trochanter involvement, the Sling fixation technique using an isoelastic polymer-based wire showed superior results in terms of stability and consequently better clinical and radiographic outcomes than IM nailing only.

Early outcomes using a 'kinematic retaining' total knee replacement - A multicentre prospective study at two years follow-up.

BACKGROUND: Although predictable implant longevity in total knee replacement (TKR) is now established, work continues to satisfy the demands of patients who seek full restoration of the painless function of the native knee following TKR. This prospective study examines the early clinical outcomes of 156 patients implanted with a novel 'kinematic-retaining' (KR) implant. METHODS: 156 Physica KR TKRs were implanted for primary osteoarthritis at three European centres. Patients were reviewed up to two years using radiographic, clinical and functional evaluations. RESULTS: Of the 137 patients retained at two years' follow up, none had been revised. Within 6 post-operative months, 51.7% and 79.9% had excellent clinical and functional KSS values respectively, increasing to 81.8% and 88.3% beyond two years. Mean KSS improvement was 34.8 (from 48.6 to 83.4). All KOOS sub-scores improved significantly with total KOOS improving from a mean of 35.5 (SD ±13.0) to 86.5 (±13.7) at two years post-operatively. Pain and sports KOOS sub-scores improved rapidly during the early post-operative periods, with sustained improvements beyond this. Mean OKS improved by 44.1 (±5.1) at two years. VAS satisfaction scores improved significantly at all time points beyond six weeks. Mean FJS-12 was 75.7 at two years, with no significant effects of age or gender. No progressive adverse radiographic features were noted. CONCLUSIONS: Early clinical and radiographic outcomes of this kinematic-retaining knee prosthesis are promising, with improvements in clinical parameters similar to, or exceeding those published in other contemporary TKR designs. LEVEL OF EVIDENCE: II, Multicentre Prospective cohort study.

Varus morphology and its surgical implication in osteoarthritic knee and total knee arthroplasty.

BACKGROUND: Knee varus alignment represents a notorious cause of knee osteoarthritis. It can be caused by tibial deformity, combined tibial-femoral deformity and/or ligament imbalance. Understanding malalignment is crucial in total knee arthroplasty to restore frontal plane neutral mechanical axis. The aim of this study was to determine which factor contributes the most to varus osteoarthritic knee and its related surgical implications in performing a total knee arthroplasty. METHODS: We retrospectively evaluated 140 patients operated for total knee arthroplasty due to a varus knee. Full-leg hip to ankle preoperative X-rays were taken. Radiological parameters recorded were: mechanical axis deviation, hip-knee-ankle, anatomical-mechanical angle, medial neck shaft angle, mechanical lateral distal femoral angle (mLDFA), medial proximal tibial angle (MPTA), joint line convergence angle (JLCA), lateral proximal femoral angle, lateral distal tibial angle (LDTA), femoral bowing, and length of tibia and femur. We also determined ideals tibial and femoral cuts in mm according to mechanical alignment technique. A R2 was calculated based on the linear regression between the predicted values and the observed data. RESULTS: The greatest contributor to arthritic varus (R = 0.444) was MPTA. Minor contributors were mLDFA (R = 0.076), JLCA (R = 0.1554), LDTA (R = 0.065), and femoral bowing (R = 0.049). We recorded an average of 7.6 mm in lateral tibial cut thickness to restore neutral alignment. CONCLUSIONS: The radiological major contributor to osteoarthritic varus knee alignment is related to proximal tibia deformity. As a surgical consequence, during performing total knee arthroplasty, the majority of the correction should therefore be made on tibial cut.

Current Practice of Italian Association of Revision Surgery Members in the Treatment of Unified Classification System Type B Periprosthetic Femoral Fracture Around Hip Arthroplasty: A Cross-Sectional Survey.

Introduction: Periprosthetic femoral fracture around hip arthroplasty are growing in the world, nevertheless management and treatment options for fractures "around the stem" are still debated due to lack of high-level studies. Materials and method: A 85-item survey were fill out by 40 Italian Orthopedic Surgeon member of SIOT (Società Italiana di Ortopedia e Traumatologia) and AIR (Associazione Italiana Riprotesizzazione) to assess their current opinion in the management of type B periprosthetic femoral fractures. Responses were summarized using proportions, and further stratified by practice type, case volume, surgeon age, and fellowship training. Results: Vancouver/UCS fracture classification showed a good interobserver agreement (k value = .76). ORIF were the treatment of choice for UCS type B1 fractures (100%), revision stem for B2 (85%) and B3 (100%). Locked plates were preferred to cable plate and cerclage without a plate for B1 fractures (50% vs 40% vs 10%); revision with modular stem was preferred to monoblock stem for B2 fractures (50% vs 35%) and B3 (75% vs 15%). Responders tended to postpone at 1-month weight-bearing in patients with B1 fractures. Regarding postoperative pharmacological treatment there was absolute lack of consensus. Discussion: The primary finding of our survey confirmed the preference of ORIF for B1 fractures and stem revision for B2 and B3 fractures. However, there is no definitive operative technique for all UCS B fractures. Surgeons tended to favor locked plating over cable plating, although only slightly. This general lack of consensus coincides with the inconclusive evidence that currently exists in the literature, which demonstrates both favorable and unfavorable outcomes for both techniques. Conclusions: The absence of complete homogeneity among participants showed the need for prospective randomized studies to set up stronger guidelines for classification, management, surgical treatment, rehabilitation, and pharmacological support of periprosthetic femoral fractures.

Trabecular titanium cups in acetabular revision arthroplasty: analysis of 10-year survivorship, restoration of center of rotation and osteointegration.

AIMS: In case of severe bone loss, acetabular revision can be challenged using extra porous pure trabecular titanium (TT) revision cups designed to ensure enhanced iliac and ischiatic purchase. Aim of the study is to report on the clinical and radiological results of a TT acetabular component, evaluating functional outcome, restoration of the hip center of rotation and osteointegration. METHODS: 85 patients, who underwent acetabular revision with a TT revision cup system between October 2009 and December 2018, were included in a retrospective study. Clinical outcome were assessed with Harris Hip Score (HHS). The hip rotation center was measured using the Pierchon method on the AP pelvis film. Loosening of the cup was determined according to the Kosashvili modification of Gill's criteria. Kaplan- Meier survivorship curve was performed. Results The mean follow-up was 6.12 years. The average HHS improved from 54.7 points to 89.7 points (p < 0.05). Two acetabular components (2.3%) were re-revised after a mean of 5.6 years, for aseptic loosening and for infection, with a progressive radiolucency and a > 5 mm vertical migration, respectively. The radiographic evaluation of the position of the hip rotation center revealed a statistically significant difference (p < 0.05) between the pre- and post-operative values. The hip rotation center was correctly restored within 5% of the reference Pierchon values in a percentage of 85.4% relative to horizontal parameters and within 8% in a percentage of 66.7% relative to vertical parameters. 5-year and 10-year survivorships were, respectively, 100% and 88%. CONCLUSIONS: In case of severe bone loss, TT revision cup system allows for good restoration of center of rotation and osteointegration showing good 10-year survival rate.

Diagnosis and treatment of chronic synovitis in patients with haemophilia: consensus statements from the Italian Association of Haemophilia Centres.

Although synovitis is recognized as a marker of joint disease activity, its periodic assessment is not included in routine clinical surveillance of patients with haemophilia (PwH). In order to evaluate the current knowledge and to identify controversial issues, a preliminary literature search by the Musculoskeletal Committee of the Italian Association of Haemophilia Centres (AICE) has been conducted. Statements have been established and sent to the Italian AICE members to collect their level of agreement or disagreement by a Delphi process. Thirty-seven consensus recommendations have been drafted. We found a general agreement on the indication to consider the presence of synovitis as a marker of joint disease activity in PwH. Accordingly, there was agreement on the indication to search for synovitis both in patients reporting joint pain and in asymptomatic ones, recognizing ultrasound as the most practical imaging technique to perform periodic joint screening. Interestingly, after detection of synovitis, there was agreement on the indication to modify the therapeutic approach, suggesting prophylaxis in patients treated on demand and tailoring treatment in patients already under prophylaxis. Whereas the need of an early consultation with a physiotherapist is recommended for PwH affected by chronic synovitis, the exact timing for an orthopaedic surgeon consultation is currently unknown.

A three-dimensional measurement method on MR arthrography of the hip to classify femoro-acetabular impingement.

PURPOSE: (1) To investigate correlations between different types of FAI and the ratio of acetabular volume (AV) to femoral head volume (FV) on MR arthrography. (2) To assess 2D/3D measurements in identifying different types of FAI by means of cut-off values of AV/FV ratio (AFR). MATERIALS AND METHODS: Alpha angle, cranial acetabular version, acetabular depth, lateral center edge angle, AV, and FV of 52 hip MR arthrography were measured. ANOVA test correlated different types of FAI with AFR. ROC curves classified FAI by cut-off values of AFR. Accuracy of 2D/3D measurements was calculated. RESULTS: ANOVA test showed a significant difference of AFR (p value < 0.001) among the three types of FAI. The mean values of AFR were 0.64, 0.74, and 0.89 in cam, mixed, and pincer types, respectively. Cut-off values of AFR were 0.70 to distinguish cam types from mixed and pincer types, and 0.79 to distinguish pincer types from cam and mixed types. Cut-off values identified 100%, 73.9%, and 55.6% of pincer, cam, and mixed types. 2D and 3D classifications of FAI showed accuracy of 40.4% and 73.0%. CONCLUSIONS: 3D measurements were clearly more accurate than 2D measurements. Distinct cut-off values of AFR discriminated cam types from pincer types and identified pincer types in all cases. Cam and mixed types were not accurately recognized.

Proximal femoral replacement: A salvage treatment of cephalomedullary nails' mechanical failures in the elderly population.

BACKGROUND: The use of proximal femoral replacements (PFR) has been recently described for catastrophic internal fixation failures. PFR is an attractive treatment option because it is technically straightforward and allows for immediate mobilization of the patient. The aim of the study was to determine the survivorship, functional outcome and complications' rate in a group of elderly patients who underwent proximal femoral replacement as a salvage treatment after femur cephalomedullary nails' mechanical failures. METHODS: We evaluated 21 patients who underwent salvage of a failed cephalomedullary nail by using a single design PFR at our institution between 2014-2017. A cemented stem was used in all cases. Radiographs were assessed for fractures, sign of loosening, presence of heterotopic ossification and leg length discrepancy. Functional evaluation was performed through Harris Hip Score (HHS), FIM™ and Time Up and Go test (TUG). Kaplan-Meier estimator was used to determine the overall implants' survival. RESULTS: The average age at the time of surgery was 83years. The mean follow-up was 3.1years. We recorded 3 dislocations of which 2 required a revision. No case of septic or aseptic failure was reported. Two patients died respectively at 11 and 14 months after surgery. At the last follow-up the mean HHS, FIM™, and the TUG improved significantly (p<0.05). CONCLUSION: Immediate weight bearing, good functional outcomes, low complications' and one-year mortality rate make the proximal femur replacement with megaprostheses a potential first line treatment of intertrochanteric/subtrochanteric fixations' failures among elderly, osteoporotic, frail patients. Dislocation is the most common complication to bear in mind within the first six months after surgery.

Joint replacement for the management of haemophilic arthropathy in patients with inhibitors: A long-term experience at a single Haemophilia centre.

INTRODUCTION: The association between haemophilia and the so-called 'inhibitors', alloantibodies against the infused factor able to neutralize its clotting activity, is a very rare condition. Those sporadic patients suffer of an even more severe arthropathy and performing primary or revision arthroplasty become truly challenging. Literature about this topic is scarce, consisting in small case series, high rates of complications and mid-term follow-ups. AIM: The purpose of this study is the assessment of the long-term outcomes of primary and revision arthroplasty performed in a population of patients with inhibitors, the more consistent to date reported at a single haemophilia centre. METHODS: We reviewed the records of 18 patients with inhibitors (26 procedures) between 1999 and 2017, divided in two groups. Group A [primary total Knee-Hip arthroplasty (TKA-THA)]: 13 patients underwent 19TKA and 2THA; and B (revision): 5 subjects underwent 3rTKA and 2rTHA. All patients received the same haematological prophylaxis (rFVIIa). Haemophilic Joint Health score and VAS, and X-rays were recorded pre- and postoperatively. The survival rate of all primary implants was assessed. RESULTS: The median follow-up was 12.2 years (3-21) for group A, 8.6 years (4-12) for B. Few complications have been reported; the overall survival rate was 94.7% at 15 years. All patients reported satisfaction, pain reduction and improved functional ability. CONCLUSION: Primary and revision TKA/THA in haemophilic subjects and inhibitors may be nowadays considered safe and effective if performed in dedicated multidisciplinary centres. The use of continuous infusion of rFVIIa showed an adequate haemostatic effect and low rate of complications. As expected, revisions are more prone to complications compared to primary arthroplasty.