Cleft and Craniofacial Center
Hasbro Children's Hospital

Fibrous Dysplasia

Fibrous dysplasia is a rare bone disorder that occurs when abnormal scar-like fibrous tissue develops where there should be normal bone. This condition can affect different bones of the body, including the face and skull. This fibrous tissue causes the bone to grow abnormally and can lead to fractures, facial deformities and, in rare cases, even blindness. 

The abnormal bone may shift the facial features causing the face, eyes or jaw to appear uneven. The craniofacial team at Hasbro Children’s Hospital specializes in the care of children with fibrous dysplasia affecting the head and neck areas (craniofacial fibrous dysplasia).

Fibrous dysplasia is often first identified in young children and teenagers. This disorder typically involves only one area of the body. However, multiple sites of fibrous dysplasia can sometimes be seen. In these situations, the child most likely has a genetic condition called McCune-Albright syndrome. In such situations, it would be important for these children to be evaluated by a genetic specialist. 

Symptoms of Fibrous Dysplasia

  • Facial swelling, deformity, and asymmetry
  • Shifting facial structure
  • Nasal obstruction
  • Jaw and bite misalignment making it difficult to chew and swallow
  • Vision and hearing problems due to the abnormal bone growth pressing on adjacent nerves
  • Facial fractures
  • Pain
  • Endocrine problems that can cause early puberty or a thyroid disorder
  • Brown skin patches called café-au-lait patches

Testing and Diagnosis

This genetic disorder develops early in pregnancy and is not inherited from parents. Patients with fibrous dysplasia will not pass it on to their children. 

Patients may be referred to other specialists, including geneticist, endocrinologist, or orthopedics, for additional testing and evaluation.

Imaging studies, include X-rays, bone scans and computed tomography (CT) scans.  These can be used to construct 3D images of the abnormal bone growth and then used for surgical planning and intervention.

Treatment

There is no cure for fibrous dysplasia. Your child may be offered surgery based on the location of the abnormal bone growth to relieve pain, improve facial appearance, prevent fractures or to remove large fibrous growths that are pressing on adjacent structures and causing additional problems. Your surgeon will discuss the best way to remove or debulk (reduce a portion of the abnormal bone) while minimizing visible scarring. Surgery may be performed by a team of specialists that include plastic surgery, neurosurgery, otolaryngology (ENT), and ophthalmology. If the abnormal bone involves the jaw and malocclusion, an abnormal alignment of the jaw, occurs, surgery may be combined with orthodontics, also known as braces, to reposition the abnormally placed bones.

Follow-Up Treatment for Fibrous Dysplasia

The abnormal bone may continue to grow, and some patients will need more than one surgery. The fibrous dysplasia will be monitored through imaging, so that the craniofacial team can monitor your child’s development. You may also need to follow-up with an endocrinologist.

Learn more about fibrous dysplasia from the National Organization for Rare Disorders