Figure 5

Serial images of a woman who presented at 9-year old with MAS and extensive fibrous dysplasia complicated by growth hormone excess. A&B) At presentation, she had a history of failure to thrive, airway obstruction, and was blind in the left eye at the time of presentation. Due to the airway obstruction in the nose and displacement of the tongue by the mandibular lesions, she underwent extensive contouring of the nasal bones, maxilla, and mandible with excellent results and patent airway. C-E) Over time, this patient’s lesions continued to grow but eventually stabilized by age 17 years. F-J) The patient 5 years after the second surgery. She has improved facial contours and symmetry though she continues to have pronounced orbital asymmetry. Her airway remains stable. She graduated magna cum laude from college.F) The 3D model of the patient demonstrates the enlargement of the maxilla, mandible, and blockage of the nasal cavity by the FD at age 17 years. G) The left mandible was significantly contoured to more normal proportions. H) Aggressive contouring of the left maxilla as well as the opening of the occluded nasal cavity. I) The nasal trumpet (green) was necessary to maintain a patent passageway while healing from surgery. J&K) Intraoperative view of the surgically removed fibrous dysplastic bone.