Re: Fibrous dysplasia

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Treatment

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.
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%).