Surgical Treatment of Extensive Fibrous Dysplasia in the Craniofacial Region –  a Case Report

Authors: F. Kurinec 1;  B. Rudinský 2;  L. Marinčák 3
Authors‘ workplace: Otorinolaryngologická klinika FNsP Nové Zámky 1;  Neurochirurgická klinika FNsP Nové Zámky 2;  CEIT Biomedical Engineering, s. r. o., Košice 3
Published in: Cesk Slov Neurol N 2016; 79/112(6): 723-727
Category: Case Report


Fibrous dysplasia is a rare non-malignant bone disorder. The bone affected by this disorder is replaced by abnormal fibrous connective tissue. This normal fibrous tissue weakens the bone, making it abnormally fragile and prone to a fracture. Fibrous dysplasia may affect one solitary bone (monoostotic disease) or the disorder can be widespread, affecting multiple bones (polyostotic disease). Fibrous dysplasia is usually diagnosed in children or young adults but mild cases may go undiagnosed until adulthood. The case report describes management and surgical treatment of extensive fibrous dysplasia in a 29-year-old man.

Key words:
fibrous dysplasia – craniofacial – surgical treatment

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.


1. 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.

2. Lustig LR, Hol­liday MJ, McCarthy EF, et al. Fibrous dysplasia involv­ing the skull base and temporal bone. Arch Otolaryngol Head Neck Surg 2001;127(10):1239– 47.

3. Edgerton MT, Pers­ing JA, Jane JA. The surgical treatment of fibrous dysplasia. Ann Surg 1985;202(4):459– 79.

4. Cai M, Ma LT, Xu GZ, et al. Clinical and radiological observation in a surgical series of 36 cases of fibrous dysplasia of the skul­l. Clin Neurology Neurosurgery 2012;114(3):254– 9. doi: 10.1016/ j.clineuro.2011.10.026.

5. Mihál V, Michálková K, Ehrmann J, et al. Fibrózní dysplazie lebky. Pediatr Praxi 2013;14(1):58– 9

6. Martinez-Lage JL. Bony reconstruction in the orbit region. Ann Plast Surg 1981;7:464– 79.

7. Lee JS, Fitzgibbon E, Butman JA, et al. Normal vision despote nar­row­ing of the optic canal in fibrous dysplasia. N Engl J Med 2002;347(21):1670– 6.

8. Diah E, Mor­ris DE, Lo I, et al. Cyst degeneration in craniofacial fibrous dysplasia: clinical presentation and management. J Neurosurg 2007;107(3):504– 8.

9. Michael CB, Lee AG, Patrinely JR, et al. Visual loss as­sociated with fibrous dysplasia of the anterior skull base. Case report and review of the literature. J Neurosurgery 2000;92(2):350– 4.

10. Chen YR, Chang CN, Tan YC, et al. Craniofacial fibrous dysplasia an update. Chang Gung Med J 2006;29(6):543– 9.

11. Hart ES, Kel­ly MH, Bril­lante B, et al. Onset, progres­sion, and plateau of skeletal lesions in fibrous dysplasia and the relationship to functional outcome. J Bone Miner Res 2007;22(9):1468– 74.

12. Sas­sin JF, Rosenberg RN. Neurological complications of fibrous dysplasia of the skul­l. Arch Neurol 1968;18(4):363– 9.

13. Pham TQ, Chua B, Gorbatov M, et al. Optical coherence tomography findings of acute traumatic maculopathy fol­low­ing motor vehicle accident. Am J Ophtalmol 2007;143(2):348– 50.

14. Cutler CM, Lee JS, Butman JA, et al. Long-term outcome of optic nerve encasement and optic nerve decompres­sion in patiens with fibrous dysplasia: risk factors for blindness and safety of observation. Neurosurgery 2006;59(5):1011–7.

15. Kel­ly MH, Bril­lante B, Col­lins MT. Pain in fibrous dysplasia of bone:age-related changes and the anatomical distribution of skeletal lesions. Osteoporos Int 2008;19(1):57– 63.

Paediatric neurology Neurosurgery Neurology

Article was published in

Czech and Slovak Neurology and Neurosurgery

Issue 6

2016 Issue 6

Most read in this issue
Forgotten password

Enter the email address that you registered with. We will send you instructions on how to set a new password.


Don‘t have an account?  Create new account