Long-Term Follow-Up of a Patient with McCune-Albright Syndrome: A Case Report

McCune-Albright综合征患者的长期随访:病例报告

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Abstract

Background/Objectives: McCune-Albright syndrome (MAS) is a rare disease characterized by the triad of fibrous dysplasia (FD), café au lait skin macules, and hyperfunctioning endocrinopathies. Although there are many case reports of MAS, few have discussed long-term oral management. We describe the long-term follow-up of an MAS patient over 15 years. Case Presentation: A male patient aged 13 years and 7 months was referred to our department with a chief complaint of difficulty with toothbrushing. He was diagnosed with MAS at 9 years, and bisphosphonate therapy was started. We continued to review the patient periodically and extracted several primary teeth with no adverse effects such as the medication-related osteonecrosis of the jaw (MRONJ). We evaluated the changes in FD using facial photographs, and facial asymmetry worsened over time until the age of 19, when surgery was performed. Although improvement was observed after surgery, there was a tendency for recurrence up to the age of 25 years. Conclusions: Continuous dental support over 15 years has prevented oral disease and minimized the need for surgical procedures such as tooth extractions, which are factors in MRONJ. The worsening of FD on the left side caused facial asymmetry until the age of 25 years; however, the asymmetry may have stabilized with the development of FD on the right side and with age-related changes. It is important for dental professionals to provide MAS patients with appropriate oral health instruction and oral management, taking changes in FD into consideration.

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