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Surgical Treatment of Polyostotic Craniomaxillofacial Fibrous Dysplasia Associated With Acromegalia

The McCuneAlbright Syndrome

Franco, Jéferson Martins Pereira Lucena DDS; Rêgo, Roberto Dias DDS, MSc; Gomes, George Matos Ferreira DDS; Santos, Daniel Facó da Silveira DDS, MSc; Santos, Eliardo Silveira DDS, PhD

doi: 10.1097/SCS.0000000000005498
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Introduction: The McCune–Albright syndrome (MAS) is a complex congenital disorder caused by the embryonic post-zygotic somatic activating mutations in the GNAS1 gene. In such syndrome, phenotypes are heterogeneous and comprised polyostotic/monostotic fibrous dysplasia, café au lait macules, and hyperfunctioning endocrinopathies as the excess growth hormone. Likewise, acromegaly, as a manifestation of the endocrine hyperfunction, is unusual and affects about 20% of patients with MAS.

Case Presentation: This research study describes a case of a 31-year-old female subject presenting polyostotic fibrous dysplasia with severe facial involvement, along with acromegaly and the MAS. The case was satisfactorily managed by surgical re-alignment and presented no clinical signs of relapse in a 12-year follow-up period. Finally, a literature review was conducted to discuss the standard protocols and the controversies when treating such cases.

Conclusion: Patients with craniomaxillofacial fibrous dysplasia associated with acromegaly may present significant facial deformities that can be satisfactorily treated by cosmetic treatment, especially in patients with psychological problems and severe social acceptance.

Department of Oral and Maxillofacial Surgery, Hospital Geral de Fortaleza—HGF, University of Fortaleza, Fortaleza, CE, Brazil.

Address correspondence and reprint requests to Jéferson Martins Pereira Lucena Franco, DDS, Department of Oral and Maxillofacial Surgery, Hospital Geral de Fortaleza—HGF, Rua Paulo Morais, 321, Papicu, Fortaleza, Ceará, Zip Code: 60175-175, Brazil; E-mail: jefersonlucenaodonto@hotmail.com

Received 26 June, 2018

Accepted 21 February, 2019

The authors state that they have no conflict of interest.

© 2019 by Mutaz B. Habal, MD.