Clinical Practice Guidelines and Modern Management Approaches for Craniofacial Fibrous Dysplasia

Professor Shiao-Pieng LEE

Tri-Service General Hospital

National Defense Medical Center

 

Fibrous dysplasia (FD) is one of the most perplexing diseases of osseous tissue caused by post-zygotic, activating mutations of the GNAS gene that results in inhibition of the differentiation and proliferation of bone-forming stromal cells and leads to the replacement of normal bone and marrow by fibrous tissue and immature woven bone. FD comprises approximately 2.5% of all bone tumors and nearly 7.5% of benign bone pathology. It is more common in children and young adults with equal gender predilection.

 

FD frequently affects the craniofacial complex, including the maxilla and the mandible. This benign tumor-like condition can cause a variety of signs and symptoms depending on the extensive type and specific location of the lesions. The most common presenting features included facial deformity, malocclusion, intraoral bulging, and dental anomalies. There is no consensus regarding the best surgical treatment (conservative or radical) for craniofacial FD. The prognosis for patients with craniofacial FD is usually favorable, although the spontaneous malignant transformation is reported.

 

The rarity of craniofacial FD, its variable clinical behavior, and progression often leads to misdiagnosis and inadequate therapeutic approaches, thereby making treating this condition difficult with few established guidelines for clinicians. Accordingly, this talk is mainly aimed at a comprehensive review of the current concept, recent developments, and modern management of this disease and the sharing of experience in TSGH. I will then suggest the clinical practice guidelines for craniofacial FD that are tailored to the individual patient and founded on clinical, radiological, and pathoanatomical information.