More than 80 % of craniofacial FD cases are diagnosed within the first two decades of life. Most cases are originally identified in children and adolescents. The pathologic process in this anatomic site is not always strictly limited to one bone, but may extend by continuity across suture lines to involve adjacent bones, thus the commonly used notation monostotic in these cases is not always accurate, and the term craniofacial fibrous dysplasia is preferred. Monostotic FD occurs in the craniofacial skeleton, particularly the maxilla and mandible, in 25 % of the cases. The craniofacial skeleton may be involved in either of the two types of FD. Polyostotic FD is less common and a few of these cases may also be associated with skin pigmentation and endocrine abnormalities, a condition known as the McCune Albright’s syndrome which is more common in female patients. The disease may affect multiple bones (polyostotic) or a single bone (monostotic). Four main groups of BFOLs will be addressed.įibrous dysplasia (FD) is a dysplastic skeletal anomaly in which normal bone is distorted and replaced by poorly organized and inadequately mineralized immature bone and fibrous tissue. This review will discuss the clinical, microscopic and radiologic aspects of the more important types of BFOL of the craniofacial complex with updated information on underlying genetic and molecular pathogenic mechanisms of disease. Reactive, neoplastic, developmental and dysplastic pathologic processes are included under the rubric of BFOL and treatment varies from disease to disease. Some BFOL of the craniofacial complex are unique to that location whereas others are encountered in bones from other regions. Whereas some are diagnosable histologically, most require a combined assessment of clinical, microscopic and radiologic features. Benign fibro-osseous lesions of the craniofacial skeleton (BFOL) are a variant group of intraosseous disease processes that share similar microscopic features characterized by hypercellular fibroblastic stroma containing various combinations of bone or cementum-like tissue and other calcified structures.
0 Comments
Leave a Reply. |
AuthorWrite something about yourself. No need to be fancy, just an overview. ArchivesCategories |