Craniofacial Fibrous Dysplasia Involvements of Mccune-Albright Syndrome: A Review with an Additional Case

Abstract

Background: McCune-Albright Syndrome (MAS) is a genetic disorder with a triad of endocrine diseases, caféau-lait macules and fibrous dysplasias. Craniofacial fibrous dysplasia is a term that is used to describe the fibrous dysplasia which were localized at the craniofacial skeleton and it is common in MAS patients. Objective: The objective of this review is to determine the involvement frequency of cranial and facial bones in the 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 stated the involved bones or did not add any radiographic images were excluded from the study. Results: 26 cases in 25 articles which met the inclusion criteria was found. 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 was 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 which 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.

Clinical Characteristics and Management of Patients With McCune-Albright Syndrome With GH Excess and Precocious Puberty: A Case Series and Literature Review

“…GH excess, which is present in 20~30% of patients with MAS (8,(14)(15)(16), presents with increased growth velocity in children and adolescents, and facial and acral dysmorphia in adults. GH excess is usually accompanied by serious craniofacial FD complications-e.g., visual, hearing, and olfactory injuries (16)(17)(18)(19)(20)(21)(22).…”

Current concepts of craniofacial fibrous dysplasia: pathophysiology and treatment

“…If a rapidly expanding lesion is associated with new localized pain or paresthesia, malignancy and aneurysmal bone cysts should be suspected, and MRI is recommended modality of diagnosis. Aneurysmal bone cysts are benign, rapidly expanding cystic lesions that can cause severe pain and compression sequelae and are best treated with surgery [ 27 ].…”