McCUNE-ALBRIGHT SYNDROME ASSOCIATED WITH ACROMEGALY: A CASE OF FACIAL DISFIGURATION

Abstract

McCune-Albright syndrome is characterized by the triad of fibrous dysplasia, cafe-au-lait spots, and hyperfunctioning endocrinopathies. About 20% of the patients affected by the syndrome have acromegaly. A 36-year-old male patient with McCune-Albright syndrome and acromegaly described a complaint of severe headache and body pain when he was diagnosed with a syndrome by an endocrinologist. He was referred to treatment by maxillofacial surgery with craniofacial involvement associated with fibrous dysplasia, amaurosis in the right eye due to pathologic occlusion of the optic canal, severe facial disfiguration, dental diastemas, and alveolar borders of altered contours. Image exams revealed polyostotic fibrous dysplasia and pituitary adenoma. The patient underwent osteoplasties on the face with the aid of virtual planning. After the surgeries, he had significant improvement of the masticatory function and aesthetics with a balance of the proportions of a third of the face and a better social life.