Preserving growth following limb-salvage surgery of the upper
limb in children remains a challenge. Vascularized autografts may
provide rapid biological incorporation with the potential for growth
and longevity. In this study, we aimed to describe the outcomes
following proximal humeral reconstruction with a vascularized fibular
epiphyseal transfer in children with a primary sarcoma of bone.
We also aimed to quantify the hypertrophy of the graft and the annual
growth, and to determine the functional outcomes of the neoglenofibular
joint. We retrospectively analyzed 11 patients who underwent this procedure
for a primary bone tumour of the proximal humerus between 2004 and
2015. Six had Ewing’s sarcoma and five had osteosarcoma. Their mean
age at the time of surgery was five years (two to eight). The mean
follow-up was 5.2 years (1 to 12.2).Aims
Patients and Methods
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.
