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Year: 2013  Vol. 17   Num. Suppl. 1  - Print:
Nathalia Soares Campos, Fbio de Rezende Pinna, Fernando de Andrade Balsalobre, Leandro Parilla Foltran, Richard Louis Voegels, Vanessa Mika Kinchoku

McCune Albright Syndrome is a classical clinical triad: Osseous fibrous dysplasia with variant polyostotic, coffee with milk spots (cafe au lait), and precocious puberty. It is a rare illness with a prevalence varying from 1/100,000 to 1/1,000,000. Osseous head and neck involvement is present in 50%-100% of the polyostotic forms. Diagnosis is based on suggestive clinical discoveries and helped by imaging and biopsy. In this case report, we will report on an 11-year-old female patient, who presented with the complete clinical triad. Fibrous dysplasia seems to be the most common component of the syndrome and is more clinically relevant to changes in its definition. The areas that are most affected by dysplasia are the proximal femur and skull base, often manifested by facial asymmetry. For this reason, otolaryngologists provide many of the diagnoses for this condition, so they should be able to diagnose, propose a multidisciplinary approach, and treat it. Computed tomography is the best method to reveal skull base lesions, which have the appearance of ground glass. In most cases, surgery is not necessary, but it is imperative to have continuous observation and imaging of dysplasia, as well as regular evaluation of the cranial nerves. The use of bisphosphonates remains controversial, but they were chosen for the patient in question and had satisfactory results. Objective of this report is to describe a rare case of McCune-Albright syndrome with the complete diagnostic triad and our experience in the diagnosis, monitoring, and treatment of this case.



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