Clinical characteristics of craniomaxillofacial fibrous dysplasia

Bone Recontouring by Guided Surgical Navigation Integrating “Mirroring” Computational Planning in the Management of Severe Fronto-orbital Asymmetry in Fibrous Dysplasia

“…FD occurs in a single bone (monostotic) in almost 70% of cases or in multiple bones (polyostotic) in the remaining 30% of cases. [1][2][3] It may also be associated with extraskeletal features, such as café-au-lait skin macules and endocrinopathic disorders seen in McCune-Albright syndrome. [1][2][3] In most cases, the diagnosis is based on clinical presentation, typically characterized by a progressive skeletal deformity, and on radiologic findings, with the classic ground-glass appearance of the lesions.…”

“…[1][2][3] It may also be associated with extraskeletal features, such as café-au-lait skin macules and endocrinopathic disorders seen in McCune-Albright syndrome. [1][2][3] In most cases, the diagnosis is based on clinical presentation, typically characterized by a progressive skeletal deformity, and on radiologic findings, with the classic ground-glass appearance of the lesions. [1][2][3][4] Depending on the location and extent of the bone lesions, it can also cause pathologic fractures, pain, and nerve compression.…”

“…[1][2][3][4] Depending on the location and extent of the bone lesions, it can also cause pathologic fractures, pain, and nerve compression. [1][2][3] Although FD typically develops during the skeletal growth period and gradually stops, it can progress in adulthood, particularly in the polyostotic forms. [1][2][3] Facial asymmetry, which generally involves the zygomatic region, represents by far the most common sequela of the craniofacial disease.…”

Effects of BMP‐2 on the osteogenic differentiation of bone marrow stem cells in fibrous dysplasia

“…Overproduced fibrous tissue replaces normal bone (Remoli et al., ), which can lead to pain, misshapen bones and fractures of monostotic or polyostotic bones. While the effects of FD are usually seen in the long bones, the skull also can be affected by FD, which causes changes in the shape of the face and hearing or vision loss (Bowers, Taussky, & Couldwell, ; Wu et al., ; Yuksel, Gurbuz, Okay, & Kabalar, ).…”

“…While the effects of FD are usually seen in the long bones, the skull also can be affected by FD, which causes changes in the shape of the face and hearing or vision loss (Bowers, Taussky, & Couldwell, 2014;Wu et al, 2014;Yuksel, Gurbuz, Okay, & Kabalar, 2017). FD is caused by random somatic mutation of the GNAS gene, which encodes the alpha subunit of stimulatory G proteins (Gsα) (Bianco et al, 1998) with constitutive activation of adenylyl cyclase and overproduction of cyclic adenosine monophosphate (cAMP) (Burke, Collins, & Boyce, 2017).…”