Clinical guidelines for the management of craniofacial fibrous dysplasia.
Fibrous dysplasia (FD) is a non-malignant condition 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 woven bone. The phenotype is variable and may be isolated to a single skeletal site or multiple sites and sometimes is associated with extraskeletal manifestations in the skin and/or endocrine organs (McCune-Albright syndrome). The clinical behavior and progression of FD may also vary, thereby making the management of this condition difficult with few established clinical guidelines. This paper provides a clinically-focused comprehensive description of craniofacial FD, its natural progression, the components of the diagnostic evaluation and the multi-disciplinary management, and considerations for future research.
Department of Oral & Maxillofacial Surgery, University of California San Francisco, San Francisco, CA, USA.
J S
JS
Lee
E J
EJ
FitzGibbon
Y R
YR
Chen
H J
HJ
Kim
L R
LR
Lustig
S O
SO
Akintoye
M T
MT
Collins
L B
LB
Kaban
D000172:Acromegaly / Q000473:pathology; D000293:Adolescent; D002648:Child; D004164:Diphosphonates / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D005357:Fibrous Dysplasia of Bone / Q000175:diagnosis / Q000188:drug therapy* / Q000473:pathology; D006801:Humans; D010256:Paranasal Sinuses / Q000473:pathology; D010346:Patient Care Management / Q000379:methods*; D014057:Tomography, X-Ray Computed / Q000379:methods; D014076:Tooth Diseases / Q000473:pathology
D004164:Diphosphonates