Fibrous dysplasia is a bone disease that destroys normal bone and replaces it with fibrous bone tissue. This tissue is not hard like normal bone, but is soft and stringy, making it more fragile and prone to break.
Fractures occur in about 50 percent of patients with fibrous dysplasia. One or more bones can be affected and most often it is a long bone, such as the femur (thigh bone).
Patients with fibrous dysplasia have an increased risk of developing malignant tumors such as osteosarcoma, fibrosarcoma, chondrosarcoma and malignant fibrous histiocytoma. However, that increased risk is very low — only about 1 percent of cases.
Fibrous dysplasia occurs in childhood, usually between the ages of 3 and 15. Males are more often diagnosed with fibrous dysplasia than females, except one specific type of fibrous dysplasia — McCune-Albright syndrome — which is more common in females.
There are two forms of fibrous dysplasia:
Fibrous dysplasia is also known as inflammatory fibrous hyperplasia or idiopathic fibrous hyperplasia.
The cause of fibrous dysplasia is unknown, but researchers have ruled out hereditary origins.
At The Children’s Hospital of Philadelphia (CHOP), diagnostic evaluation begins with a thorough medical history and physical examination of your child.
Clinical experts use a variety of diagnostic tests to diagnose fibrous dysplasia, including:
At CHOP, treatment of fibrous dysplasia is based on your child’s symptoms and results of diagnostic testing.
In some cases, fibrous dysplasia can be treated with a class of drugs called bisphosphonates, which prevent bone loss and can reduce pain.
If the thigh bone is fractured due to fibrous dysplasia, surgical treatment is almost always necessary.
Though surgery for fibrous dysplasia is highly effective, we understand that any surgery can be a stressful experience for children and families. At CHOP, we offer a wealth of resources about how to prepare your child for surgery and what to expect during surgery.
Additionally, we employ numerous best practices before, during and after surgery to decrease the risk of infection and increase positive outcomes. See safety in surgery for details about safety protocols at The Children's Hospital of Philadelphia.
Surgical treatment for fibrous dysplasia consists of:
After surgery for fibrous dysplasia, your child should expect to stay overnight in the Hospital.
At The Children's Hospital of Philadelphia, we offer ongoing support and services for patients and families at our Main Campus and throughout our CHOP Care Network. Our team is committed to partnering with parents and referring physicians to provide the most current, comprehensive and specialized care possible for your child.
If your child had surgical treatment for fibrous dysplasia, he or she will return for check-ups one to two weeks after surgery, then again at three months, six months, one year and two years post-surgery.
Follow-up is important in fibrous dysplasia to prevent deformities as a result of the disease and check for recurrence. In 50 percent of cases, fibrous dysplasia will recur.
During follow-up visits, your child’s physician will likely perform a physical exam and order X-rays of the affected area until your child reaches skeletal maturity. Your child may also be referred to an endocrinologist for periodic testing of any thyroid abnormalities.
If your child is diagnosed with McCune-Albright syndrome, a subset of fibrous dysplasia, your child will also be monitored for any spine curvatures such as scoliosis.
Outcomes for children treated for fibrous dysplasia at CHOP have been excellent. Because the disease often recurs, continued clinical monitoring is extremely helpful in long-term management of the condition.
To make an appointment with the Division of Orthopedic Surgery at The Children's Hospital of Philadelphia, call 215-590-1527 or contact us online.
Reviewed by: Jason L. Katz, PA-C, MHS, ATC-R, and John P. Dormans, MD, FACS
Date: January 2013