The results of a functional, clinical and radiological study of 30 children (60 hips) with whole-body cerebral palsy were reviewed at a mean follow-up of 10.2 years (9.5 to 11). Correction of windsweep deformity of the hips was performed by bilateral simultaneous combined soft-tissue and bony surgery at a mean age of 7.7 years (3.1 to 12.2). We were able to recall 22 patients; five had died of unrelated causes and three were lost to follow-up. Evaluation involved interviews with patients/carers and clinical and radiological examination. The gross motor functional classification system was used to assess overall motor function and showed improvement in seven patients. Of the 12 patients thought to have pain pre-operatively, only one had pain post-operatively. Improved handling was reported in 18 of 22 patients (82%). Those with handling problems were attributed by the carers to growth of the patients. All patients/carers considered the procedure worthwhile. The range of hip movements improved, and the mean windsweep index improved from 50 pre-operatively to 36 at follow-up. The migration percentage and centre-edge angle were assessed on plain radiographs. Radiological containment improved, the mean migration percentage improved from 50 pre-operatively to 20 at follow-up and the mean centre-edge angle improved from −5° to 29°. No statistical difference was noted between the three-year and ten-year follow-up results, indicating that the improvements in clinical and radiological outcome had been maintained.
Osteofibrous dysplasia is an unusual developmental condition of childhood, which almost exclusively affects the tibia. It is thought to follow a slowly progressive course and to stabilise after skeletal maturity. The possible link with adamantinoma is controversial and some authors believe that they are part of one histological process. We retrospectively reviewed 16 patients who were diagnosed as having osteofibrous dysplasia initially or on the final histological examination. Their management was diverse, depending on the severity of symptoms and the extent of the lesion. Definitive (extraperiosteal) surgery was localised ‘shark-bite’ excision for small lesions in five patients. Extensive lesions were treated by segmental excision and fibular autograft in six patients, external fixation and bone transport in four and proximal tibial replacement in one. One patient who had a fibular autograft required further excision and bone transport for recurrence. Six initially underwent curettage and all had recurrence. There were no recurrences after localised extraperiosteal excision or bone transport. There were three confirmed cases of adamantinoma. The relevant literature is reviewed. We recommend extraperiosteal excision in all cases of osteofibrous dysplasia, with segmental excision and reconstruction in more extensive lesions.