The Facial Osteoplasty for Polyostotic Fibrous Dysplasia in a Patient With McCune-Albright Syndrome: A Case Report

Abstract

Fibrous dysplasia is a developmental anomaly that affects bone maturation and remodeling, generating replacement of medullary and cortical bone by a disorganized and immature fibro-osseous matrix, which makes the sufferer susceptible to bone pain, skeletal deformities, and pathological fractures. This is a condition that, when associated with cutaneous hyperpigmentation and endocrinological disorders, forms the classic triad of McCune-Albright syndrome, a rare multisystemic pathology formed by postzygotic somatic mutations of the GNAS gene. Fibrous dysplasia can even cause loss of vision, hearing, or difficulty breathing. The severity of these manifestations is associated with the type of treatment, which can be conservative or surgical. The surgical approach is adapted to each particularity and it aims mainly to resolve functional restrictions or correct aesthetic deformities through bone plasty. The present work aims to report the case of a McCune-Albright syndrome patient with deforming craniofacial fibrous dysplasia that triggers respiratory compromise. After clinical and tomographic evaluation, it was decided to remove and reshape the bone affected in the maxilla through the Weber-Ferguson approach and the mandible through the modified Newman approach. The case progressed satisfactorily, with an improvement in the respiratory condition and a reduction in facial asymmetry.