Asymptomatic facial disfigurement-case reports of monostotic and polyostotic fibrous dysplasia

Abstract

Fibrous Dysplasia is a developmental dysplastic disorder of bone in which the normal bone matrix is replaced by fibroblastic proliferation. Von Rechlinghausen was the first author who described it in 1891 and Lichtenstein was the person who introduced the term fibrous dysplasia. It can be monostotic (70%) or polyostotic (30%). The craniofacial localization occurs in 10%-25% of cases in monostotic forms and in 50% of cases in polyostotic forms. Almost all patients with extensive polyostotic forms of the disease have skull involvement, while most patients with craniofacial bone involvement have the monostotic form of the disease. Fibrous dysplasia essentially affects children and young adults, with no sex preference. Diagnosis is not always straightforward because the functional symptomology is often absent or not specific. Only symptomatic and/or gross forms are considered for treatment.