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Fibrous dysplasia is a condition characterized by abnormal bone growth. It is a benign fibro-osseous disease process in which normal bone is replaced by fibrous tissue and immature bone. Fibrous dysplasia may involve one bone (monostotic form) or, less commonly, multiple bones (polyostotic form). Individuals with the polyostotic form of fibrous dysplasia, early puberty, endocrine problems and skin pigment changes may have McCune-Albright syndrome.
Fibrous dysplasia is a rare congenital condition. It is associated with a spontaneous gene mutation affecting the cells that make bone. This is not an inherited condition, meaning that it has not been passed to a child by a parent. The cause of the disease is unknown.
Although the disease is present at birth, diagnosis is not usually made until late childhood or the teen years. Signs and symptoms of the polyostotic form of fibrous dysplasia may be noted earlier. The bones most commonly affected are the femur, tibia, pelvis, ribs, skull, facial bones and humerus. Typical presentation is one of painless enlargement of the involved bone or pathologic fracture of an extremity. When the craniofacial skeleton is involved, the maxilla or frontal bones are most frequently affected. This may cause facial asymmetry. Depending on which bone(s) is involved, the disease may cause problems with vision, hearing, eating, speech or cranial nerve function. It is rare, but malignancy (cancerous cells) may develop in bones affected by fibrous dysplasia.
Examination of your child's skull, face and body will help guide your child's doctor towards a correct diagnosis. A CT scan, x-ray and/or an MRI will be used to diagnose the changes in the craniofacial skeleton that are associated with fibrous dysplasia. A bone biopsy may confirm the diagnosis.
A thorough evaluation by the Craniofacial Team will be undertaken. Your child may also see a team of specialists to help diagnose and treat his or her particular condition(s). Together we will create a comprehensive treatment plan for your child.
The main concerns in the early treatment planning for children with fibrous dysplasia have to do with vision, breathing and hearing. The timing of any surgery is determined by the severity and presentation of your child's particular symptoms. If there are no symptoms, then conservative treatment with observation and close follow up is recommended. There is no cure for fibrous dysplasia.
Medications known as bisphosphonates have been used for chronic pain in adult patients. If the disease progresses to cause functional or psychosocial concern, then surgery may be recommended. The type of surgery recommended will depend on symptoms, but may include debulking the tissue or completely resecting the tissue with reconstruction using bone grafts.