Background: McCune-Albright Syndrome (MAS) is a genetic disorder with a triad of
endocrine diseases, café-au-lait macules and fibrous dysplasias. Craniofacial fibrous dysplasia is a
term that is used to describe the fibrous dysplasia, which was localized at the craniofacial skeleton
and is common in MAS patients.
Objective: The objective of this review is to determine the involvement frequency of cranial and facial
bones in patients with MAS and CFD.
Methods: Articles in PubMed was searched with the following details “(mccune[Title/Abstract]
OR albright[Title/Abstract]) OR (“craniofacial fibrous dysplasia”[MeSH Terms] OR (“craniofacial”[
All Fields] AND “fibrous”[All Fields] AND “dysplasia”[All Fields]) OR “craniofacial fibrous
dysplasia”[All Fields])”. The articles in which the authors did not state the involved bones or
did not add any radiographic images were excluded from the study.
Results: 26 cases in 25 articles met the inclusion criteria. Among the 26 cases and our case,
sphenoid and frontal bones were involved in 17 cases, parietal and occipital bones were involved in
15 cases, mandible and ethmoid bone were involved in 14 cases, maxilla-zygoma-temporal and
palate was involved in 13, 11, 6 and 3 cases, respectively. Palate was involved in cases where maxilla
was also involved. Our case was the only case that was evaluated with CBCT.
Conclusion: Routine follow-ups are important since new CFDs can occur in different cranial or facial
bones. 2D imaging techniques may not be able to demonstrate early CFDs; thus, an advanced
imaging technique should be used after MAS diagnosis.