Fibrous Dysplasia of Temporal Bone in 14-months Old Boy

Czech version

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

Overview

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

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