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

Abstract

BackgroundFibrous 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.MethodsData 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.ResultsAnalysis 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.ConclusionOur 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.Electronic supplementary materialThe online version of this article (10.1186/s13023-018-0805-7) contains supplementary material, which is available to authorized users.

The Modified West China Hospital Radiographic Classification for Fibrous Dysplasia in Femur: A Retrospective Analysis of 238 Patients

“…And the indication for surgical treatment in type II-V patients included the following: mechanical/ weight-bearing bone pain, hip and/or knee stiff, walking with a limp, fracture, and severe LLD [10]. Therefore, type II was treated with internal xation (IF) following simple curettage [4]. Type III was treated with the IF following single or multiple level valgus osteotomies and simple curettage [5,6].…”

“…Primitive bone has failed to transform into the mature lamellar bone and realign in response to mechanical stress [3]. The lower extremity is frequently affected by deformity, fracture, leg length discrepancy (LLD), and limping [4][5][6][7]. Given the numerous surgical options and complicated deformities in the femur, it can be di cult for orthopedic surgeons to evaluate the severity of deformities and select an appropriate treatment strategy.…”

“…The surgical strategy is aimed at bone pain relief [9], restoring normal femoral alignment [5], gait normalization [6], LLD [5], and preventing pathological fracture [4,10] for FD patients. Based on our early experience in assessing deformities and pairing treatment strategies [6,11,12], we modi ed our previous classi cation into ve types following corresponding treatment options, which were as follows: including proximal femur bone loss, neck-shaft angle, femoral shaft deformity, genu valgum, and arthritis of hip.…”

Clinical and translational pharmacological aspects of the management of fibrous dysplasia of bone

“…Traditionally, the main therapeutic approach to the management of FD/MAS is a surgical one, ranging from curettage of FD lesions in the early 60s , to customized blade plates more recently . The notion of using pharmacological agents in the management of FD/MAS has evolved in the 70s, based on the accumulation of evidence for increased bone resorption in FD lesions.…”

18F-NaF PET/CT IMAGING IN FIBROUS DYSPLASIA OF BONE

“…(4)(5)(6) Surgical treatment is the mainstay approach for fractures and deformities; however, outcomes are frequently unsatisfactory due to postoperative FD regrowth in the craniofacial skeleton, (7,8) and implants that are incapable of providing adequate long-term support in weight-bearing bones, particularly in growing children. (9)(10)(11) Because of the wide phenotypic variability between patients, accurate assessment of skeletal disease burden is a critical component of the evaluation, management, and study of FD. (1) Radiographs and computed tomography (CT) scans can provide anatomical characterization of individual FD lesions ( Fig.…”