Surgical Management of Fibrous Dysplasia and Associated Syndromes

Abstract

Fibrous dysplasia is a benign condition that is characterized by the inability of bone-forming tissue to produce mature lamellar bone, creating a radiolucent lesion of immature bone within the medullary canal of the affected bone. Typically occurring in the long bones, this process can lead to pathologic fractures, debilitating pain, and malignant transformation and may be associated with Mazabraud syndrome, severe polyostotic disease, and McCune-Albright syndrome. Fibrous dysplasia can be treated most effectively if caught early with nonsurgical diphosphonate therapy, which has been shown to improve cortical thickness and reduce pathologic fractures. Conversely, in those with higher disease burden, surgery can prove beneficial, albeit difficult. Current literature suggests that although bone grafting may be an option, it does not necessarily provide long-term stability, particularly for patients younger than 18 years. Studies tracking long-term follow-up showed 75% of various grafts, including but not limited to struts, bone chips, and freeze-dried grafts, had failed to incorporate and had been resorbed. Furthermore, studies showed patients treated with strut allografts found a 54% revision-free survival after 20 years, demonstrating it as a viable option in treatment of proximal femoral disease. Perhaps the most challenging complication, the shepherd's crook, was shown to be treated with 71% revision-free success with angled blade plates and had 91% of patients walking pain-free. Moreover, success has been found with an intertrochanteric valgus osteotomy and fixation with an angled blade plate followed by stabilization with a cephalomedullary nail. Although recent studies have shown promise, additional long-term follow-up will need to be done to confirm improvement of both deformity and functional status.