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.

Introduction: The purpose of this study is to demonstrate that definitive surgery (extraperiosteal excision) is required in patients with osteofibrous dysplasia (OFD) due to the risk of recurrence and co-existent adamantinoma. The possible link with adamantinoma is controversial with some authors believing that they are part of one histological process. This therefore provides difficulty in recommending treatment options.

Methods: Using our database 22 cases of OFD were identified. Management was diverse.

Results: Definitive (extraperiosteal) surgery, in the majority of our patients, was localized excision for small lesions (less than 50% of the bony circumference) and segmental excision followed by reconstructive surgery for more extensive ones. Seven patients had a “shark-bite” excision and a further seven were treated with fibula autografting. Of the latter group, one required further excision and bone transport due to recurrence of OFD. Five underwent bone transport & distraction osteogenesis and one had a proximal tibial replacement. Nine initially underwent curettage, but eight recurred. No recurrences occurred following localized extraperiosteal excisions and bone transport. There were three confirmed cases of adamantinoma.

Discussion: In view of the risk of association of OFD with adamantinoma, and to some extent the continuous morbidity of OFD if left untreated, we believe that radical extraperiosteal excision is indicated in most if not all cases of OFD.