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.