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Fibrous Dysplasia of the Craniofacial Bones

  • Wen-Sen Lai and Jih-Chin Lee
Published/Copyright: August 1, 2013
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Journal of Osteopathic Medicine
From the journal Journal of Osteopathic Medicine Volume 113 Issue 8

An elderly woman presented with a history of slow and progressive facial asymmetry since childhood. Physical examination revealed painless right facial enlargement involving the soft tissue and bony structures of the maxilla and mandible (image A). A plain radiograph revealed hazy radiopacities with a ground glass appearance (image B), suggestive of polyostotic fibrous dysplasia (FD). Transoral excisional biopsy revealed fibrous stroma in which spicules of disconnected woven bone were noted, confirming the diagnosis of polyostotic FD. The patient was treated with 70 mg of alendronate once weekly to improve bone density, decrease the diameter of the lesion, and decrease pain scores. At 30-month follow-up, no other complaints were reported, and the craniofacial FD was smaller.

Craniofacial FD masses are almost exclusively unilateral; the maxilla and mandible are most frequently affected followed by the frontal, parietal, and occipital bones.1 Although approximately 1% of craniofacial FD cases carry a risk of malignant transformation, it typically follows a benign and indolent course of bone malformation.2 Bisphosphonate therapy may be useful in treating patients for refractory pain. Immediate surgical care may be necessary for patients with rapid growth, new sensory changes, or auditory or visual disturbances.3

From the Department of Otorhinolaryngology—Head and Neck Surgery, Tri-Service General Hospital, National Defense Medical Center in Taipei, Taiwan. Dr Lai is also associated with the Department of Otolaryngology–Head and Neck Surgery, Taichung Armed Forces General Hospital in Taichung, Taiwan
Address correspondence to Jih-Chin Lee, MD, Department of Otorhinolaryngology—Head and Neck Surgery, Tri-Service General Hospital, No 325, Sec 2, Chenggong Rd, Neihu District, Taipei City 114, Taiwan (Republic of China). E-mail:

  1. Financial Disclosures: None reported.

References

1 Wei YT Jiang S Cen Y . Fibrous dysplasia of skull. J Craniofac Surg.2010;21(2):538-542. doi:10.1097/SCS.0b013e3181d0243f.10.1097/SCS.0b013e3181d0243fSearch in Google Scholar PubMed

2 Leet AI Collins MT . Current approach to fibrous dysplasia of bone and McCune-Albright syndrome. J Child Orthop.2007;1(1):3-17. doi:10.1007/s11832-007-0006-8.10.1007/s11832-007-0006-8Search in Google Scholar PubMed PubMed Central

3 Lee JS Fitzgibbon EJ Chen YR et al. . Clinical guidelines for the management of craniofacial fibrous dysplasia. Orphanet J Rare Dis.2012;7(suppl 1):S2. doi:10.1186/1750-1172-7-S1-S2.10.1186/1750-1172-7-S1-S2Search in Google Scholar PubMed PubMed Central

Received: 2012-11-28
Revised: 2013-01-07
Accepted: 2013-01-23
Published Online: 2013-08-01
Published in Print: 2013-08-01

© 2013 The American Osteopathic Association

This work is licensed under the Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

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https://doi.org/10.7556/jaoa.2013.027
Creative Commons
BY-NC-ND 4.0

Articles in the same Issue

  1. Abstracts
  2. 57th Annual AOA Research Conference—Abstracts, 2013
  3. Editorial
  4. Search for a New Editor in Chief
  5. Osteopathic Research Conference 2013: “From Bench to Bedside”
  6. Letters to the Editor
  7. The Effect of OMT on Postoperative Medical and Functional Recovery of Coronary Artery Bypass Graft Patients
  8. Response
  9. Relighting the Fire in Our Bellies
  10. Original Contribution
  11. Mathematical Analysis of the Flow of Hyaluronic Acid Around Fascia During Manual Therapy Motions
  12. Exercise During Pregnancy: The Role of Obstetric Providers
  13. Evidence-Based Clinical Review
  14. Caring for Patients With Chronic Pain: Pearls and Pitfalls
  15. Medical Education
  16. Incorporating Simulation Technology Into a Neurology Clerkship
  17. Case Report
  18. Conservative Approach to Tardive Dyskinesia–Induced Neck and Upper Back Pain
  19. Clinical Images
  20. Fibrous Dysplasia of the Craniofacial Bones
  21. In Your Words
  22. One of Ours
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