Extensive Craniofacial Fibrous Dysplasia: An Overview

Abstract

Introduction: 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.Aim: The aim of this article is to study the clinical presenta tion 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.Results: 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.