Computed tomography characteristics of non-syndromic craniofacial fibrous dysplasia.

BACKGROUND: Fibrous dysplasia is a benign fibro-osseous tumor of bones commonly involving the craniofacial region. Computed tomography (CT) imaging study of the disease is useful for evaluation and treatment planning. However, few studies have evaluated such large patient series.
METHODS: A total of 46 patients with complete medical records and CT images was included in this study. All of these patients were non-syndromic, had fibrous dysplasia involving only the craniofacial region, and had no skin pigmentation or other evidence of endocrine problems. Data analyses were performed on the clinical manifestations, time of onset, signs and symptoms, involvement of cranial and facial bones, and CT appearance of the tumors in this patient group.
RESULTS: Painless swelling was the chief clinical problem in 78% of patients, followes by dental malocclusion in 22%. Onset of the disease was reported to have occurred before 6 years of age in 34%, between 6 and 10 years in 27%, and older than 10 years in 39% of patients. Extreme timings such as onset at infancy or older than 20 years of age were also noted. The average number of bones involved was 3.2 bones per patient. Involvement of more than one craniofacial bone occurred in 70% of patients. The maxilla, orbital, and frontal bones were most commonly involved. CT images appeared sclerotic or homogenous in 34%, mixed white and dark or heterogenous in 55%, and cystic in 11%. A correlation between the age of onset of the disease and the number of bones involved was not observed.
CONCLUSIONS: Findings of this study demonstrate that craniofacial fibrous dysplasia displays a wide spectrum of clinical behaviors. CT imaging generally revealed extensive involvement of the tumor in the craniofacial region.