Five patients with isolated Madelung’s deformity were reviewed with an average follow-up of 34 years after surgery. All the patients were female and their average age at surgery was 12.7 years, whereas average age at follow-up was 53 years. The deformity was bilateral in 4 patients and unilateral in 1.

At diagnosis deformity, pain and limitations of the range of motion were present in all the wrists except 2, which were painless but presented marked functional impairment. In all the patients the typical radial deviation of the hand, was observed, with dorsal prominence of the distal end of the ulna. The x-rays showed, in anteroposterior view, the V-shaped arrangement of the first carpal row, with the lunate at the apex of the V and the marked obliquity of the articular surface of the radius toward the ulnar side. In the lateral view, the articular surface of the radius was markedly angulated anteriorly, the ulna was subluxated posteriorly and carpal bones were translated anteriorly. In no patient did we observe growth disturbance of the other bones or deformities typical of osteochondrodysplasias. In some cases the deformity progressed rapidly, whereas in the others the progression was slow. Surgical correction was sought by both the family and the patients mainly for functional reasons, although cosmetic improvement was also expected.

The operation consisted of closing-wedge osteotomy of the distal radial metaphysis and either shortening osteotomy or resection of the distal ulna. At the operation all the patients had passed the adolescence growth spurt, although in 5 out of the 9 wrists growth plates were still open.

At follow- up, all the patients were satisfied with the results of the operations and the range of motion of the wrists was improved. Some residual radiographic abnormalities were present in 4 wrists, but all patients were mostly pleased with the absence of pain and improvement of wrist cosmesis. No radiographic osteoarthritis was present in any of the operated wrists, although 4 of the 5 patients were over 55 years of age.

Conclusions: An association of distal radial wedge-closing osteotomy and either distal ulnar hand resection or shortening distal ulnar osteotomy to correct Madelung’s deformity gave very good results in line with other reports. The limited number of our cases may be compensated by the very lengthy long-term follow-up of our series that showed how the results of these corrective operations do not deteriorate in time; moreover no radiographic osteoarthritis was present even almost 50 years after the operation.

Fibrous dysplasia is a genetic not hereditary disease of the skeleton, most frequently located at the femoral bone. The extension and the radiographic morphology of the femoral lesion are variable, and the prognosis is unpredictable. The purpose of this study is to propose a radiographic classification of the femoral lesion, related to the prognosis and the natural history of the disease.

The authors reviewed the radiographic examinations of 25 patients affected by fibrous dysplasia of bone with femoral involvement, who were followed-up at least two years after diagnosis. Eleven patients had bilateral localization of the disease, for a total of 36 affected femurs. A radiographic classification of the femoral lesion was proposed based on the localization, the extension and the type of the lesion.

The authors classify the femoral lesions as lytic, lytic with surrounding sclerosis, lytic and sclerotic, sclerotic and complex. They observed a prevalence of the lytic lesions and involvement of the proximal part of the femur. Many lesions (40%) expanded with age, and the sclerotic component also increased. The lytic lesions worsened with age, causing severe deformity of the femur. However the mostly sclerotic lesions turned out to be stable at follow-up. The worsening of the lesion was not related to the sex of the subjects; 77% of the worsened lesions were observed in patients younger than 20 years of age, while 66% of the stable lesions were found in patients older than 20 years.

In conclusion, this study demonstrated that the prognosis of the femoral lesion in fibrous dysplasia is related to the type of lesion, as per the radiographic classification here in described. Prognosis is also related to the age of the patient and the presence of a sclerotic component, which suggested a greater stability of the lesion.

OP-1 is a bone morphogenetic protein which induces bone formation. For this reason, this protein is used to treat congenital pseudoarthrosis and post-traumatic non-union as well as to improve healing of bone grafting in orthopaedic surgery. In the present study we report the results of treatment in 10 patients in whom OP-1 was used to improve bone healing. Four patients were operated because of post-traumatic non-union, one because of congenital pseudo-arthrosis and four because of spinal arthrodesis, whereas one had a tibial lenghthening with poor bone formation. At follow-up, ranging from 6 to 16 months after the operation, we observed satisfactory bone formation in all cases. Although the number of our cases is limited, our results confirm the effectiveness of OP-1 in the treatment of post-traumatic non-union and spinal arthrodesis.