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VOLUME 10 , ISSUE 1 ( January-April, 2017 ) > List of Articles


Extensive Craniofacial Fibrous Dysplasia: An Overview

Keywords : Endocrinal,Fibrous dysplasia,Ground glass,Maxillary

Citation Information : Extensive Craniofacial Fibrous Dysplasia: An Overview. Clin Rhinol An Int J 2017; 10 (1):17-21.

DOI: 10.5005/jp-journals-10013-1297

Published Online: 01-04-2017

Copyright Statement:  Copyright © 2017; The Author(s).



Fibrous dysplasia (FD) is a common benign osseous tumor that may affect the craniofacial skeletal. It is a slowly progressing disease, presenting with swelling and pressure effects on adjacent structure. Epistaxis as presenting symptom is rare. The literatures reviewed rarely mention it.


The aim of this article is to study the clinical presentation and management of extensive craniofacial FD along with review of literature, emphasizing ear, nose, and throat/endocrinal and orthopedic checkup and computer tomo graphy evaluation and related hematological workup. Serum alkaline phosphatase was also done to see its correlation to disease activity. Brief overview of management and its related complication is done.


Both patients were presented with 2year history. There was no predisposing factor. Both were from tribal area. They were having slowly progressive nasal obstruction, facial swelling, and proptosis. In Case 1, it was loosening of tooth and facial pain. In Case 2, it was epistaxis which was the presenting complaint of patient. Nasal obstruction, swelling, and epistaxis in teenager male usually suggest angiofibroma. Craniofacial FD may have epistaxis and pain due to cystic degeneration in FD. Both patients had proptosis; however, their vision was normal. Serum alkaline phosphates was marginally raised, thus not signifying active disease. Computed tomography (CT) scan was diagnostic in both cases, with ground glass appear ance. In Case 2, there was cystic degeneration present and CT was useful in diagnosing the disease, to show the extent of lesion and any intracranial extension. Fine needle aspiration cytology was inconclusive and incision biopsy is better to get representative sample. Lateral rhinotomy approach with lip splitting and gingival component gave good exposure. The tumor was totally removed with thin bone at periphery. The tumor was vascular, and bleeding was controlled by anterior nasal packing.

How to cite this article

Sachdeva K. Extensive Cranio facial Fibrous Dysplasia: An Overview. Clin Rhinol An Int J 2017;10(1):1721.

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  1. Clinical guidelines for the management of craniofacial fibrous dysplasia. Orphanet J Rare Dis 2012 May 24;7(Suppl 1):S2.
  2. Fibrous Dysplasia of bone in the McCune-Albright syndrome: abnormalities in bone formation. Am J Pathology 1997 Dec;151(6):1587-1600.
  3. The histopathology of fibrous dysplasia of bone in patients with activating mutations of the Gs alpha gene; site-specific patterns and recurrent histological hallmarks. J Pathol 1999 Jan;187(2);249-258.
  4. Fibrous dysplasia. J Am Acad Orthop Surg 2004 Sep-Oct;12(5):305-313.
  5. Computed tomography characteristics of non-syndromic craniofacial fibrous dysplasia. Chang Gung Med J 2002 Jan;25(1):1-8.
  6. Spontaneous conversion of fibrous dysplasia into osteosarcoma. J Craniofac Surg 2011 May:22(3):959-961.
  7. Fibrous dysplasia of the temporal bone. J Laryngol Otol 1988 Feb;102(2):171-172.
  8. Cyst degeneration in craniofacial fibrous dysplasia: clinical presentation and management. J Neurosurg 2007 Sep;107(3):504-508.
  9. Common fibro-osseous lesions of the paranasal sinuses. Otolaryngol Clin North Am 2006 Jun;39(3):585-600.
  10. The surgical treatment of fibrous dysplasia. With emphasis on recent contributions from cranio-maxillofacial surgery. Ann Surg 1985 Oct;202(4):459-479.
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