Surgical treatment of fibrous dysplasia of bone in McCune-Albright syndrome

Seven patients affected by McCune-Albright syndrome with polyostotic fibrous dysplasia were operated on either for fracture fixation or for correction of bone deformity. Ten femurs, three tibiae, and one humerus were stabilized by intramedullary titanium nailing with a modified unreamed femoral nail (UFN) and standard unreamed tibial (UTN) and humeral (UHN) nails. The age of the patients at operation ranged from 8 to 30 years, with an average of 17 years, and the length of follow-up ranged from 8 months to 4 years, with an average of 2 years. Before operation, each patient had suffered from a minimum of three fractures to a maximum of 12, with an average of six fractures. The femur was the most affected skeletal segment. Before surgery, two patients were wheelchair-dependent owing to their severe lower limb bone deformities. At follow-up, all the patients were painless and all were able to walk even though one had to wear a bilateral long leg brace and another had to ambulate with the aid of two crutches. No patient had had additional fractures after surgery. The main intra-operative technical problems of this demanding surgical procedure were: a) reaming of a new medullary canal through the fibrodysplastic bone; b) the amount of blood loss in femoral surgery that ranged from 800 to 2,500 ml.