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Individualized approach to the surgical management of fibrous dysplasia of the proximal femur.

ABSTRACT: BACKGROUND:Fibrous dysplasia of the proximal femur presents with heterogeneous clinical manifestations dictating different surgical approaches. However, to date there are no clear recommendations to guide the choice of surgical approach and no general guidelines for the optimal orthopedic management of these lesions. The objective of this study was to evaluate treatment outcomes of angled blade plates and intramedullary nails, using as outcome indicators revision-free survival, pain, function and femoral neck-shaft-angle. Based on a review of published literature and our study findings, we propose a treatment algorithm, taking into account different factors, which may play a role in the selection of one surgical approach over another. METHODS:Data were evaluated in thirty-two patients (18 male) from a combined cohort from the Netherlands and Austria, who had a surgical intervention using an angled blade plate (n?=?27) or an intramedullary nail (n?=?5) between 1985 and 2015, and who had a minimal follow-up of one year. The primary outcome was success of the procedure according to the revised Henderson classification. Secondary outcomes, which were assessed at one year and at the end of follow-up included: function (as measured by walking ability), pain and change in femoral neck-shaft angle over time. RESULTS:Analysis of data showed that revision-free survival was 72% after a median follow-up of 4.1 years. Revision was necessary in two patients for structural failure due to a fracture distal to an angled blade plate and in 7 patients due to angled blade plate-induced iliotibial tract pain. At the end of follow-up 91% of all patients had good walking ability and 91% were pain free. There was no significant postoperative change in femoral neck shaft angle. CONCLUSION:Our data show that fibrous dysplasia of the proximal femur can be adequately and safely treated with angled blade plates or intramedullary nails, providing these are used according to specific characteristics of the individual patient. Based on published literature and our own experience, we propose an individualized, patient-tailored approach for the surgical management of fibrous dysplasia of the proximal femur.

SUBMITTER: Majoor BCJ

PROVIDER: S-EPMC5932767 | biostudies-literature | 2018 May

REPOSITORIES: biostudies-literature

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Individualized approach to the surgical management of fibrous dysplasia of the proximal femur.

Majoor Bas C J BCJ Leithner Andreas A van de Sande Michiel A J MAJ Appelman-Dijkstra Natasha M NM Hamdy Neveen A T NAT Dijkstra P D Sander PDS

Orphanet journal of rare diseases 20180502 1

<h4>Background</h4>Fibrous dysplasia of the proximal femur presents with heterogeneous clinical manifestations dictating different surgical approaches. However, to date there are no clear recommendations to guide the choice of surgical approach and no general guidelines for the optimal orthopedic management of these lesions. The objective of this study was to evaluate treatment outcomes of angled blade plates and intramedullary nails, using as outcome indicators revision-free survival, pain, fun ...[more]

PMID: 29720212

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Project description:BackgroundSeveral strategies for the treatment of pathologic proximal femur fractures are practiced but treatment outcomes have not been rigorously compared.Questions/purposesMajor variations in the use of intramedullary fixation, extramedullary/plate-screw fixation, and endoprosthetic reconstruction techniques for pathologic proximal femur fractures in patients with skeletal metastases are reported. The clinical and surgical variables that influence this choice differ among treating surgeons. To characterize the technique preferences and to identify areas of consensus regarding specific clinical presentations, we administered an online survey to the Musculoskeletal Tumor Society (MSTS) membership. We also tested whether responses correlated with the respondents' years in practice and asked about the indications for wide tumor resection and the role of tumor debulking and adjuvant cementation.MethodsA 10-minute, web-based survey was sent via email to 244 MSTS members. The survey queried participants' musculoskeletal oncology training and experience and presented case scenarios illustrating different combinations of four variables that influence decision-making: cancer type, estimated patient survival, fracture displacement, and anatomic region of involvement.ResultsForty-one percent (n = 98) of MSTS members completed the survey. Intramedullary nail fixation (IMN; 45%) and proximal femur resection and reconstruction (34%) were the most commonly recommended techniques followed by long-stem cemented hemiarthroplasty/cemented hemiarthroplasty (15%) and open reduction and internal fixation (7%). Most respondents (56%) recommended use of cementation with IMN. Differences of opinion on recommended treatment were associated with variations in cancer type, fracture displacement, and anatomic region of involvement.ConclusionsOur online survey showed a trend among MSTS members for selecting IMN and arthroplasty-related techniques to treat pathologic fractures of the proximal femur, but major differences in preferred operative technique exist. Prospective studies are needed to develop consistent, evidence-based treatment recommendations.

Project description:Fibrous dysplasia (FD) is a skeletal disease caused by somatic activating mutations of the cyclic adenosine monophosphate (cAMP)-regulating protein, α-subunit of the Gs stimulatory protein (G(s) α). These mutations lead to replacement of normal bone by proliferative osteogenic precursors, resulting in deformity, fracture, and pain. Medical treatment has been ineffective in altering the disease course. Receptor activator of NF-κB ligand (RANKL) is a cell-surface protein involved in many cellular processes, including osteoclastogenesis, and is reported to be overexpressed in FD-like bone cells. Denosumab is a humanized monoclonal antibody to RANKL approved for treatment of osteoporosis and prevention of skeletal-related events from bone metastases. We present the case of a 9-year-old boy with severe FD who was treated with denosumab for a rapidly expanding femoral lesion. Immunohistochemical staining on a pretreatment bone biopsy specimen revealed marked RANKL expression. He was started on monthly denosumab, with an initial starting dose of 1 mg/kg and planned 0.25 mg/kg dose escalations every 3 months. Over 7 months of treatment he showed marked reduction in pain, bone turnover markers (BTMs), and tumor growth rate. Denosumab did not appear to impair healing of a femoral fracture that occurred while on treatment. With initiation of treatment he developed hypophosphatemia and secondary hyperparathyroidism, necessitating supplementation with phosphorus, calcium, and calcitriol. BTMs showed rapid and sustained suppression. With discontinuation there was rapid and dramatic rebound of BTMs with cross-linked C-telopeptide (reflecting osteoclast activity) exceeding pretreatment levels, accompanied by severe hypercalcemia. In this child, denosumab lead to dramatic reduction of FD expansion and FD-related bone pain. Denosumab was associated with clinically significant disturbances of mineral metabolism both while on treatment and after discontinuation. Denosumab treatment of FD warrants further study to confirm efficacy and determine potential morbidity, as well as to determine the mechanism of RANKL in the pathogenesis of FD and related bone marrow stromal cell diseases.

Project description:BackgroundHip fractures are a common and costly health problem, resulting in significant morbidity and mortality, as well as high costs for healthcare systems, especially for the elderly. Implementing surgical preventive strategies has the potential to improve the quality of life and reduce the burden on healthcare resources, particularly in the long term. However, there are currently limited guidelines for standardizing hip fracture prophylaxis practices.MethodsThis study used a cost-effectiveness analysis with a finite-state Markov model and cohort simulation to evaluate the primary and secondary surgical prevention of hip fractures in the elderly. Patients aged 60 to 90 years were simulated in two different models (A and B) to assess prevention at different levels. Model A assumed prophylaxis was performed during the fracture operation on the contralateral side, while Model B included individuals with high fracture risk factors. Costs were obtained from the Centers for Medicare & Medicaid Services, and transition probabilities and health state utilities were derived from available literature. The baseline assumption was a 10% reduction in fracture risk after prophylaxis. A sensitivity analysis was also conducted to assess the reliability and variability of the results.ResultsWith a 10% fracture risk reduction, model A costs between $8,850 and $46,940 per quality-adjusted life-year ($/QALY). Additionally, it proved most cost-effective in the age range between 61 and 81 years. The sensitivity analysis established that a reduction of ≥ 2.8% is needed for prophylaxis to be definitely cost-effective. The cost-effectiveness at the secondary prevention level was most sensitive to the cost of the contralateral side's prophylaxis, the patient's age, and fracture treatment cost. For high-risk patients with no fracture history, the cost-effectiveness of a preventive strategy depends on their risk profile. In the baseline analysis, the incremental cost-effectiveness ratio at the primary prevention level varied between $11,000/QALY and $74,000/QALY, which is below the defined willingness to pay threshold.ConclusionDue to the high cost of hip fracture treatment and its increased morbidity, surgical prophylaxis strategies have demonstrated that they can significantly relieve the healthcare system. Various key assumptions facilitated the modeling, allowing for adequate room for uncertainty. Further research is needed to evaluate health-state-associated risks.

Project description:ObjectiveThe mechanical high modulus structure of the proximal femur could guide clinical surgical treatment and instrument design of proximal femoral fractures. The purpose of this study is to analyze and verify the mechanical structure of the proximal femur.MethodsA total of 375 patients with intertrochanteric fractures were imaged using computed tomography (CT) scans. Patients were grouped according to age and sex. Cortical and medullary cavity parameters (cortical thickness [CTh], cortical mean density [CM], upper-lower diameter length [ULL], and medial-lateral diameter length [MLL]) were measured at eight planes. Six proximal femoral finite element models of different sexes and ages were constructed. To verify the measurement results, Abaqus was used to implement the force load to describe the von Mises stress distribution, and the maximum von Mises stress values of each wall of the proximal femur were compared.ResultsThe CTh values of the lower and upper walls were higher than those of the anterior and posterior walls of the femoral neck (p < 0.05). The CM values of the lower and upper walls were higher than those of the anterior and posterior walls of the subcephalic and middle femoral neck (p < 0.05). The ULL value gradually increased from the subcephalic region to the bottom (p < 0.05). The CTh and CM values of the medial and lateral walls were higher than those of the anterior and posterior walls in the femoral trochanteric region (p < 0.05). The MLL value decreased gradually from the plane 20 mm above the upper edge to that 20 mm below the vertex of the femoral lesser trochanter (p < 0.05). The von Mises stress was concentrated on the upper and lower walls of the femoral neck and on the medial and lateral walls of the femoral trochanteric region. The maximum von Mises stress values of the upper and lower walls were higher than those of the anterior and posterior walls of the femoral neck. The maximum von Mises stress values of the medial and lateral walls were higher than those of the anterior and posterior walls in the femoral trochanteric region, except for the plane 20 mm above the upper edge of the femoral lesser trochanter.ConclusionThe bone mass of the proximal femur presented a triangular high-modulus distribution, which bore the main stress of the proximal femur. The triangular mechanical structure provides a guideline for the surgical strategy and instrument design of the proximal femur.

Project description:Fibroblast growth factor-23 (FGF23) is a phosphate- and vitamin D-regulating hormone derived from osteoblasts/osteocytes that circulates in both active (intact, iFGF23) and inactive (C-terminal, cFGF23) forms. O-glycosylation by O-glycosyl transferase N-acetylgalactosaminyltransferase 3 (ppGalNAcT3) and differential cleavage by furin have been shown to be involved in regulating the ratio of active to inactive FGF23. Elevated iFGF23 levels are observed in a number of hypophosphatemic disorders, such as X-linked, autosomal recessive, and autosomal dominant hypophosphatemic rickets, whereas low iFGF23 levels are found in the hyperphosphatemic disorder familial tumoral calcinosis/hyperphosphatemic hyperostosis syndrome. Fibrous dysplasia of bone (FD) is associated with increased total FGF23 levels (cFGF23 + iFGF23); however, classic hypophosphatemic rickets is uncommon. Our results suggest that it can be explained by increased FGF23 cleavage leading to an increase in inactive cFGF23 relative to active iFGF23. Given the fact that FD is caused by activating mutations in the small G-protein G(s) α that results in increased cyclic adenosine monophosphate (cAMP) levels, we postulated that there may be altered FGF23 cleavage in FD and that the mechanism may involve alterations in cAMP levels and ppGalNacT3 and furin activities. Analysis of blood specimens from patients with FD confirmed that the elevated total FGF23 levels are the result of proportionally increased cFGF23 levels, consistent with less glycosylation and enhanced cleavage by furin. Analysis of primary cell lines of normal and mutation-harboring bone marrow stromal cells (BMSCs) from patients with FD demonstrated that BMSCs harboring the causative G(s) α mutation had higher cAMP levels, lower ppGalNAcT3, and higher furin activity. These data support the model wherein glycosylation by ppGalNAcT3 inhibits FGF23 cleavage by furin and suggest that FGF23 processing is a regulated process that controls overall FGF23 activity in FD patients.

Dataset Information

Individualized approach to the surgical management of fibrous dysplasia of the proximal femur.

Publications

Individualized approach to the surgical management of fibrous dysplasia of the proximal femur.

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