Role of dental clinical and imaging exam during McCune Albright Syndrome diagnosis process: Case Report

Abstract

McCune Albright Syndrome (MAS) is a rare disease characterized by the classic triad of early puberty that begins in the embryonic period and mainly affects women. The present study aims to report the case of a 27-year-old patient, melanodermic, referred to the oral and maxillofacial surgery service of the Walter Cantídio University Hospital, complaining of pain in the right mandibular body region. During the anamnesis, the patient reported having had her menarche at the age of 6. Clinically, it was observed body asymmetry, prominence of the right zygomatic, frontal and parietal bones, and expansion of the right hemimandible. Panoramic radiography showed images with ground-glass appearance in the region of the direct hemi-mandible with mixed radiopacity and root resorption, suggestive of MAS. The patient was referred to the gynecologist and endocrinologist, and it was observed that the patient had the presence of cyst in the right ovary. Incisional biopsy was performed on the right maxilla, which showed histopathological characteristics compatible with fibrous dysplasia, with final diagnosis of MAS. In summary, the present study reinforces the importance of a meticulous and interdisciplinary clinical examination allied to the patient's complaint in order to establish an adequate treatment plan.