Prevalence of Different Forms and Involved Bones of Craniofacial Fibrous Dysplasia

Abstract

Background: The purposes of this study were to analyze the prevalence of various craniofacial bones involved in patients with craniofacial fibrous dysplasia (CFD) and to demonstrate the most common form and bone involvement in patients with CFD for surgeons.

Methods: To address the research purpose, the authors designed and performed a systematic review with meta-analysis. A comprehensive electronic search without date was performed in August 2013. Data extracted from the previously published literature were analyzed with STATA 11.0 software.

Results: Relevant data were extracted from 18 studies (487 total participants) and revealed that in CFD patients, the prevalence of the monostotic type was 56% (95% confidence intervals [CI]: 0.42-0.70; P = 0.000) and polyostotic type was 47% (95% CI: 0.31-0.63; P = 0.000); McCune-Albright syndrome was a relatively rare form (7%; 95% CI: 0.02-0.12; P = 0.006). Subgroup analyses indicated that the maxilla was most commonly involved (28%; 95% CI: 0.42-0.70; P = 0.000) in monostotic CFD, followed by the orbital (27%; 95% CI: -0.23-0.76; P = 0.298), mandibular (25%; 95% CI: 0.16-0.35; P = 0.000), frontal (22%; 95% CI: 0.09-0.34; P = 0.001), and temporal bones (12%; 95% CI: 0.03-0.21; P = 0.012). The prevalence of maxilla involvement in polyostotic CFD patients was as high as 30% (95% CI: 0.18-0.42; P = 0.000).

Conclusion: This meta-analysis found that monostotic and polyostotic forms of CFD shared similar prevalence rates. Furthermore, the maxilla was found to be the most commonly involved bone in both monostotic and polyostotic CFD.