[Polyostotic fibrous dyplasia: a case report of a diffuse form with hemimelic predominance]

Fibrous dysplasia is an uncommon condition characterized by the presence of mesenchymatous tissue in bone. There are various risks. We describe the clinical and radiological features observed in a patient with fibrous polyostotic fibrous dysplasia and discuss risks. A 37-year-old man suffered from bone pain and multiple fractures without endocrine disorder since the age of 10 years. At admission in 1998, he presented limb deformities and hyperchromic spots on the thorax. Calciuria was low and alkaline phosphatase was 1274 IU/ml. Endocrine tests were normal. Radiographs showed polyostotic defects in the right hemibody and in the skull. They also showed a right subtrochanteric fissure. CT scan of the face and skull did not demonstrate nerve compression. Histology analysis identified fibrous dysplasia. Vitamin and calcium supplementation and preventive measures were instituted. No deformity led to surgical correction despite the early beginning. Polyostotic fibrous dysplasia is a congenital disease. Radiological aspects are variable. There is a risk of deformities, fractures, osteomalacia (as in our case), neurological compression, and finally a risk of sarcomatous transformation. Recently introduced biphosphonate therapy appears to provide effective pain relief and probably satisfactory prevention of fractures.