Fibrous dysplasia: a systematic review

Abstract

Objectives: To evaluate the principal features of fibrous dysplasia (FD) by systematic review (SR) and to compare their frequencies between four global groups.

Methods: The databases searched were the PubMed interface of Medline and LILACS. Only those reports of FD which occurred in a series in the reporting authors' caseload were considered. All cases were confirmed fibro-osseous lesions histopathologically. The SR-included series must also have included radiographs.

Results: Of the 106 reports considered (including the Hong Kong report), 31 reports and a total of 788 cases were included in the SR. 11 SR-included series were in languages other than English. FD affected both genders equally, but was 50% more prevalent in the maxilla. The mean age at first presentation was 24 years. The decade with the greatest frequency was the second, in which males accounted for 63%. The main symptom in 90% of all SR-included cases was swelling (including deformation of the jaws). Not one SR-included case directly involved the ocular apparatus. All cases displayed buccolingual expansion; all mandibular cases exhibited downward displacement of the lower border of the mandible and almost all maxillary cases involved the maxillary antrum. Only 35% of reports included follow-up; 18% of cases recurred or were reactivated. Not one case displayed sarcomatous change.

Conclusions: Long-term follow-up of large series that would have revealed the long-term outcomes of FD was lacking. This is necessary because many cases do not burn out at the end of adolescence, as expected of a hamartoma, but are reactivated.