Fibrous Dysplasia of Temporal Bone in 14-months Old Boy

Česká verzia

Authors: G. Pavlovčinová; J. Jakubíková; A. Jovankovičová
Authors place of work: Detská ORL klinika LF UK a DFNsP, Bratislava prednostka kliniky prof. MUDr. J. Jakubíková, CSc.
Published in the journal: Otorinolaryngol Foniatr, 62, 2013, No. 4, pp. 221-224.
Category: Case History

Summary

Introduction:
Fibrous dysplasia is a rare benign disorder of bone characterized by slow and progressive replacement of normal bone with fibrous tissue. It can affect one bone (monostotic form), or multiple bones (polyostotic bone). Also it can be a part of Jaffe-Lichtenstein syndrome or McCune-Albright syndrome. The monostotic form is typically diagnosed between 20-30 years, while polyostotic has an earlier onset, in children under 10 years.

Case report:
In our work we describe 14-months old boy who presented with painless bulging of left temporal bone. Based on clinical signs, X-ray and CT scan we did not definitive diagnosis, and we decided for surgery. The diagnosis of monostotic form of fibrous dysplasia of temporal bone was confirmed by histology.

Conclusion:
In case of uncertain diagnosis the definitive diagnosis is provided by histology. Surgical treatment should be as conservative as possible and should take care to maintain function, prevent complications a provide reconstruction of affected area.

Keywords:
fibrous dysplasia, temporal bone, monostotic type, child

Zdroje

1. Abdel-Wanis, M. E., Tsuchiya, H.: Melatonin deficiency and fibrous dysplasia: might a relation exist?. Med. Hypotheses, 59, 2002, s. 552-554.

2. Albright, F., Butler, M., Hampton, A., Smith, P.: Syndrome characterized by osteitis fibrosa disseminata, areas of pigmentation and endocrine dysfunction with precious puberty in females. N. Engl. J. Med., 216, 1937, s. 727-746.

3. Černá, H., Macháč, J., Šlapák, I.: Fibrózní dysplázie a cholesteatom. Otorinolaryng. a Foniat., 57, 2008, s. 102-104.

4. Faves, M. J., Vokes, T. J.: Pagets disease and other dysplasias of bone. In Harrisons principles of Internal Medicine 16th edition. McGraw-Hill, New York, 2005.

5. Feller, L., Wood, N. H., Khammissa, R. A., Lemmer, J., Raubenheimer, E. J.: The nature of fibrous dysplasia. Head and Face Medicine., 2009, 5, s. 22-26.

6. Chinski, A., Beider, A., Cohen, D.: Fibrous dysplasia of temporal bone. Int J. Ped. Otorhinolaryngol., 47, 1999, s. 275-281.

7. Chrapurlat, R. D., Meunier, P. J.: Fibrous dysplasia of bone. Baillieres Clin Rheumatol., 14, 2000, s. 385-398.

8. Chrobok, V., Brtková, J.: Fibrózní dysplazie spánkové kosti (kazuistika). Choroby hlavu a krku, 1998, 7, s. 18-21.

9. Kaissi, A. A., Klaushofer, K., Grill, F.: Outward bulging of the right parietal bone in connection with fibrous dysplasia in an infant: case report. Cases Journal., 2008, 1, s. 347-350.

10. Krempaská, S., Kovaľ, J.: Cholesteatóm vonkajšieho zvukovodu. Otorinolaryng. a Foniat., 55, 2006, 2, s. 122-127.

11. Levine, P. A., Wiggins, R., Archibald, R. W., Britt, R.: Ossifying fibroma of the head and neck: Involvement of the temporal bone – an unusual and challenging site. Laryngoscope., 91, 1981, s. 720-725.

12. Lichtenstein, L.: Polyostotic fibrous dysplasia. Arch. Surg., 36, 1938, s. 874-898.

13. Lustig, L. R., Holliday, M. J., McCarthy, E. F., Nager, G. T.: Fibrous dysplasia involving the skull base and temporal bone. Arch. Otolaryngol. Head Neck Surg., 127, 2001, s. 1239-1247.

14. Markalous, B., Charvát, F. a kol.: Zobrazení hlavy, paranazální dutiny, lební báze a obličejová část. Maxdorf, Praha, 2000.

15. McCune, D., Bruch, H.: Osteodystrophia fibrosa: repost of a case in which the condition was combined with precious puberty, multiple pigmentation of the skin and hyperthyroidism. AJDC. 52, 1937, s. 745-748.

16. Megerian, C. A., Sofferman, R. A., McKenna, M. J., Eavey, R. D., Nadol, J.B. Jr.: Fibrous dysplasia of the temporal bone: Ten New Cases domonstrating the Spectrum of otologic Sequelae. Am. J. Otol., 16, 1995, 4, s. 408-419.

17. Nager, G. T.: Fibrous dysplasia In pathology of the ear andtemporal bone. Md Wiliams and Wi lkins, Baltimore, 1993.

18. Nager, G. T., Kennedy, D., Kopstein, E.: Fibrous dysplasia: a review of the disease and its manifestations in the temporal bone. Ann. Otol. Rhinol. Laryngol., 91, 1982, 92, s. 1-52.

19. Nager, G. T. , Holiday, M. J.: Fibrous dysplasia of the temporal bone: updatue with case reports. Ann. Otol. Rhinol. Laryngol., 93, 1984, s. 630-633.

20. Neville, B. W., Damm, D. D., Allan, C. M., Bouquot, J. E.: Bone pathology. In Oral and Maxillofacial Pathology 2nd edition. WB Saunders Company, 2002.

21. Poruban, D., Chynoranský, M., Kurill, E.: Fronto-orbito-zygomatiko-maxilárna fibrózna dysplázia – kazuistika. Stomatológ., 9, 1999, 3, s. 37-39.

22. von Recklinghausen, F.: Die Fibrose oder deformierende ostits, die Osteomalacie and die osteoplastische Carcinose in ihren gegenseitigen Beziehungen. Berlin, Germany, 13, 1981.

23. Slow, I. N., Friedman, E. W.: Osteogenic sarcoma arising in a preexisting fibrous dysplasia: report of case. J. Oral Surg., 29, 1971, s. 126-129.

24. Warner, D. R., Weinstein, L. S.: A mutation in the heterotrimeric stimulatory guanine nucleotide binding protein alpha-subunit with impaired receptor mediated activation because of elevated GTPase activity. Proc. Natl. Acad. Sci. USA, 96, 1999, s. 4268-4272.