PubMed1998
Fibrous dysplasia accounts for approximately 2% of bone tumors. The ribs, proximal femurs and cranio-facial bones represent the majority of bone lesions. Surgery is the mainstay of treatment but the technique is controversial: conservative surgery or removal of dysplastic lesions followed by implantation of autogenous bone graft. The aim of this study was to assess the indications of each method. The medical records of 25 patients with fibrous dysplasia of the cranio-facial bones treated between January 1, 1980 and December 31, 1994 at the Department of Maxillofacial Surgery, Centre Hospitalier Universitaire de Bordeaux, France, were reviewed. Fourteen (56%) patients were women and 11 (44%) men. The median age at the time of diagnosis was 23 years (ranging from 8 to 56 years). The mean follow-up was 8 years. Two patients were unavailable for follow-up after treatment. The primary sites of the tumors were the mandible (n = 19 [76%]), maxilla (n = 1 [4%]) and skull (n = 5 [20%]). For mandibular lesions, the primary treatment always included a correction of deformations and asymmetry, which was the only treatment in 14 cases. Two patients required subsequent surgery to reduce further bone enlargement (1 and 2 years later in the first case and 11 years later in the second) without further problems. In 3 cases a segmental mandibulectomy followed by implantation of autogenous bone graft was required, and no further recurrence was observed. Therefore, the success rate of conservative surgery was 74% initially, and up to 86% after subsequent surgery. Skull lesions, although often very extensive, were remarkably stable and asymptomatic. They were successfully treated 4 times by conservative surgery, mainly for cosmetic reasons. One patient, with an ethmoidal tumor producing a mass effect along the course of the optic nerve, underwent a combined cranio-facial resection. As for the only maxillary tumor, three curettages were performed throughout an 11-year period and there was no evidence of further recurrence 4 years after the last intervention. In all cases, conservative surgery may be recommended as primary treatment of fibrous of the craniofacial bones, providing essential structures like the optic nerve are not at risk. Cosmetic results and local control proved excellent, and a further removal of the tumor remained feasible in the event of a recurrence. Success or failure did not correlate with tumor size, which justifies the use of this technique.