Fibrous dysplasia of the skull base

Fibrous dysplasia is a localized disorder of bone characterized by abnormal proliferation of fibrous tissue interspersed with normal or immature bone, and is clinically associated with endocrine dysfunction, abnormal pigmentation, and precocious puberty in females. While fibrous dysplasia involving the temporal bone has been described in a variety of case reports, its impact upon the broader skull base is not as well documented in the literature. This report documents 20 patients with fibrous dysplasia involving the skull base, representing our past 15 year experience with the disorder. Ten cases involved a combination of the maxillary, ethmoid, frontal sinuses and orbit, 7 Cases involved the temporal bone, 1 case involved the sphenoid wing, 1 case involved the pterygomaxillary fossa, and 1 case had widespread involvement throughout the skull base. This report reviews the clinical and pathologic aspects of fibrous dysplasia involving the skull base in this series of patients. Further, we will discuss the surgical indications and treatment outcome for this disorder.