Craniofacial fibrous dysplasia is a specific type of fibrous dysplasia, a chronic condition where fibrous, scar-like tissue grows in place of normal bones, causing bone deformities and pain. Craniofacial fibrous dysplasia, sometimes called facial fibrous dysplasia or fibrous dysplasia of the skull, affects the facial and/or skull bones, either one bone (monostotic) or multiple bones (polyostotic).

Some people with fibrous dysplasia develop hormonal problems due to a condition called McCune-Albright syndrome, which causes early onset of puberty and skin spots.

Symptoms

Monostotic fibrous dysplasia affects one bone and is active while the child is growing, becoming inactive after puberty, while polyostotic fibrous dysplasia affects multiple bones and may remain active for life.

Symptoms affecting the skull include:

  • Facial asymmetry
  • Shifts in facial structure
  • Nasal airway obstruction
  • Jaw and bite misalignment
  • Visual and hearing problems

Additionally, children with craniofacial fibrous dysplasia may experience symptoms related to bone weakness in other parts of the body, including fractures or deformities, pain, or lesions in the bones. Children with polyostotic fibrous dysplasia may also experience endocrine gland problems, such as early puberty, thyroid disorders and related issues, as well as unusual skin patches or spots, called café-au-lait pigmentation.

Diagnosis

Fibrous dysplasia usually occurs in children ages 3 to 15 and is sometimes not diagnosed until adulthood. Diagnosis is done through a thorough physical exam that may include X-rays and CT scans, as well as bloodwork and hormonal tests if McCune-Albright syndrome symptoms are present.

Treatment

Treatment options typically involve specialists from plastic surgery, neurosurgery, endocrinology, and ophthalmology based on the specific needs of your child. Depending on which facial and/or skull bones are affected, surgery may be the recommended treatment.

Surgery is typically performed with limited incision approaches to reduce the appearance of scars. For small and accessible areas of the skull and/or face, bone is usually removed and reconstructed using a combination of bone grants, plates and screws. For larger areas, bone may be reduced to a normal size by using a high-speed burr. For patients with facial and/or jaw deformities, a combination of surgery, orthodontics and facial reconstructive surgery is available, and our team of specialists at the North Carolina Cleft and Craniofacial Center provide comprehensive care through reconstruction, surgery, therapy and future checkups.