McCune Albright syndrome (MAS) is a rare disease with a broad spectrum of clinical manifestations that may include café-au-lait macules, hyperfunctioning endocrinopathies and fibrous dysplasia. The latter is an uncommon and debilitating skeletal disorder that leads to fractures, deformity, functional impairment and pain. Since bone imaging provides essential data for diagnosis, prognosis evaluation and follow-up, the radiologist plays a crucial role at all steps of MAS management – and sometimes becomes the first health professional to assess non-skeletal associated conditions in routine exams. The present study proposes a practical approach for imaging McCune Albright syndrome. The main findings on various imaging modalities will be discussed according to their topography, bringing together relevant information to enhance the diagnostic, prognostic and follow-up procedures. Case material from our institution will be used to illustrate the following topics: 1. Brief review of MAS pathophysiology; 2. Clinical features and differential diagnosis of MAS; 3. MAS and polyostotic fibrous dysplasia; 4. Craniofacial fibrous dysplasia: imaging features, prognosis and follow-up; 5. Role of various imaging methods in non-skeletal manifestations.
SPR 2020 Annual Meeting & Postgraduate Course
Neto Miguel José,