Craniofacial polyostotic fibrous dysplasia : Report of a case with review of literature

Fibrous dysplasia is a nonspecifîc hamartomatous skeletal developmental anomaly, characterized by slow progressive replacement of a localized area of bone by an abnormal proliferation of isomorphic fibrous tissue. The exact etiology of it is in ambiguity. The recent concept with etiopathogenesis proposes that post-zygotic mutation in the guanine nucleotide binding protein á-stimulating activity polypeptide-1 gene. The Fibrous dysplasia can be of monostotic type which is common and affects maxilla, and polyostotic type, a rare variety. The incidence of cranial fibrous dysplasia is even rare amongst the primary bone lesions, representing less than one percent. There is no specifie treatment for fibrous dysplasia. Untreated fibrous dysplasias are associated with complications like hearing loss, trismus, and facial disfigurement for which cosmetic surgical treatment is rendered.