Orthopedic Surgery for Fibrous Dysplasia Treatment & Management
- Author: Bernardo Vargas; Chief Editor: Harris Gellman, MD more...
Medical Therapy
Although there is no specific medical therapy for fibrous dysplasia, studies have shown decreased pain after treatment with bisphosphonates. Bisphosphonates inhibit bone resorption by their action on osteoclasts (see also Future And Controversies ). The most common drug therapy is intravenous pamidronate. An intravenous infusion of pamidronate (total dose of 1 mg/kg/day over 3 days, repeated every 3-6 mo) has been proposed. The total dose must be administered over a 4-hour period. Vitamin D and calcium supplements must be added to this therapy. This therapy in children seems to be safe, but longer follow-up is needed to confirm the absence of collateral effects on the growth plate. An increased growth-plate thickness has been reported in children treated with bisphosphonates.[2]
The PROFIDYS study (Oral Bisphosphonate Effect on Osseous Symptoms in Fibrous Dysplasia of Bone) is a double-blind study evaluating the long-term safety and results of treatment with an oral bisphosphonate (risedronate [Actonel]), which has been ongoing since 2007. The study is evaluating bone pain and the evolution of osteolytic lesions in patients with fibrous dysplasia, and it is expected to be completed by 2013.
Surgical Therapy
- If surgical treatment is required for fibrous dysplasia in long bones, intramedullary nails are recommended.[26]
- This technique provides good stabilization and could prevent deformation.
- Conservative treatment, use of plates, curettage, or bone grafting should be discouraged.[10, 14, 27]
- Deformity-correction surgery is indicated in patients with mechanical axis deviation of the lower limbs.
Intraoperative Details
- The dysplastic bone in fibrous dysplasia can be quite difficult to ream.
- Fibrous dysplasia is associated with a high tendency of bone bleeding during surgery.[10]
Follow-up
- The main role of the follow-up is to prevent deformity as a result of the disease.
- The authors recommend yearly radiographs of the involved area or areas until skeletal maturity.
- Fibrous dysplasia rarely undergoes remission. For this reason, it is appropriate to periodically monitor the disease progression, especially in the skeletally immature patient.
- Once skeletal maturity has been achieved, it is unusual for monostotic fibrous dysplasia to progress.
- Early intervention with internal fixation of involved bones may be important in the prevention of deformity.
- Referral to an endocrinologist for endocrine and metabolic testing is suggested so that endocrine anomalies can be diagnosed and treated.
Complications
- Fracture is the most common complication of fibrous dysplasia. In polyostotic disease, fracture occurs in more than 50% of cases.
- Deformity may occur in weight-bearing bones.
- Malignant transformation occurs in less than 0.5% of cases. It is more likely to occur if polyostotic disease exists or following treatment with radiation therapy. Typically, malignant transformation occurs during the third or fourth decade of life.[12] Benign tumors have also been associated with fibrous dysplasia.[28]
- Patients with McCune-Albright syndrome have a high incidence of scoliosis (probably more than 50%).[2]
Outcome and Prognosis
- The recurrence rate for fibrous dysplasia has been reported to be 21% following curettage and grafting, but if patients are monitored for many years, the rate is probably closer to 100%.
- Unless malignant transformation develops, fibrous dysplasia is not a life-threatening disease. The lesions tend to stabilize as skeletal maturity is reached.
- The majority of the monostotic cases have a good evolution regardless of treatment.
- Polyostotic lesions are very often associated with one or more fractures.[14]
- Malignant transformation develops in a minority of patients (< 0.5%)
Future and Controversies
Major advances have occurred in understanding the molecular basis of fibrous dysplasia. The mutation has been identified, but the actual pathways that lead to abnormal osteoblast differentiation and function are just beginning to be understood.
In the future, effective nonsurgical treatments may be possible. The risk of local recurrence is high, so the decision to treat must be made with informed consent to avoid inappropriate expectations. In general, the goals of surgery should be to stabilize the bone and relieve pain, rather than to excise the involved bone. The condition often is found incidentally, and the need for prophylactic treatment may be difficult to accept for an asymptomatic or minimally symptomatic patient.
The PROFIDYS study (Oral Bisphosphonate Effect on Osseous Symptoms in Fibrous Dysplasia of Bone) is double-blind study evaluating the long-term safety and results of treatment with an oral bisphosphonate (risedronate [Actonel]), which has been ongoing since 2007. The study is evaluating bone pain and the evolution of osteolytic lesions in patients with fibrous dysplasia, and it is expected to be completed by 2013.
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