Fibrous dysplasia (FD) is a benign bone tumor that often affects the face and skull by replacing normal bone with fibrous tissue. There are two types with the most common being monostotic, meaning it affects one area of bone only and grows throughout puberty but then usually stops growing. More rarely when the disease affects multiple bones (polyostotic) the tumor growth may remain active throughout life. Rarely it can be associated with McCune-Albright syndrome, a specific syndrome with polyostotic fibrous dysplasia as well as skin and hormonal changes. Although we do not know exactly what causes FD is it not genetic and generally not passed on through families.
Clinical presentation depends on what bones are involved but most commonly results in asymmetry of the facial areas involved. The most common sites of the involvement of the craniofacial bones are the forehead, followed by eye, then cheek bones this causing asymmetry in these areas. Because of the common involvement of the bones around the eye, there often are visual changes. Diagnosis is usually made by a CT scan which shows classis ground glass opacities.
|TREATMENT & MANAGEMENT|
Treatment is varied depending on the bones involved but includes monitoring by a craniofacial team to observe for appearance changes and check for any visual or other functional changes. Periodic CT scans may be helpful to monitor any progression of the disease. The goal of any treatment is preserve function primarily and secondary to improve appearance. Often if no functional problem we recommend waiting until after puberty years when the disease is less likely to continue to grow. For small areas of involvement we can usually remove all the disease and replace with bone, however for larger areas we may not be able to remove all of the bone and also may need to use a bone substitute to reconstruct the area. It is common for additional procedures need to be done.