Craniofacial Fibrous Dysplasia

Ricalde, Pat; Magliocca, Kelly R.; Lee, Janice S.

doi:10.1016/j.coms.2012.05.004

Cited by 51 publications

(74 citation statements)

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“…Approximately 70% of patients with polyostotic fibrosis dysplasia and 10% of patients with the monostatic form have facial lesions [10]. The upper jaw is more frequently affected than the lower jaw and often demonstrates unilateral lesions in the posterior area [4,11]. In many cases involving lesions in the jaw area, the initial examination is accidental and occurs in the context of dental radiographic examinations in patients in the second and third decade of life [12].…”

Section: Discussionmentioning

confidence: 99%

“…In the appendicular skeleton the disease often becomes apparent in older patients due to fractures, deformations, or pain [4]. Approximately 70% of patients with polyostotic fibrosis dysplasia and 10% of patients with the monostatic form have facial lesions [10].…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…While fibrous dysplasia accounts for approximately 7% of all benign osseous tumors, McCune-Albright syndrome occurs very rarely, with a prevalence of 1/100,000 to 1/1,000,000 [3]. In 70-80% of the cases the disease manifests in a monostatic form in the femur, tibia, and ribs, and in 20-30% as a polyostotic form [4].The pathology is a mutation of the GNAS gene on chromosome 20 in early embryogenesis [5]. This gene encodes a G protein, which leads to dysregulation of the adenylyl cyclase [6].…”

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Various Radiographic Appearances of Fibrous Dysplasia in the Mandible - A Case Report

Angermair¹,

Fretwurst²,

Semper-Hogg³

et al. 2017

Dentistry

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and sclerotic areas. These variable radiographic presentations impede radiographic diagnostics and require for an experienced practitioner to plan correct patient management Therefore, the present case report demonstrates the strongly deviating clinical and radiological manifestations of monostotic and polyostotic forms of fibrous dysplasia (FD) in the facial area and its impact on dental and surgical therapy. Presentation of Case Patient 1A 14-year-old male patient was admitted to the Department of Oral-and Craniomaxillofacial Surgery presenting a painless distension in the anterior lower jaw which appeared approximately 4 month before dental visit. Clinical diagnostics revealed an inconspicuous, pale reddish gingiva in the concerned region. In the right incisor region, a hard, non-compressive, non-displaceable swelling of 4 × 3 cm (Figure 1) was detectable. The patient demonstrated a conservative adequately restored juvenile dentition and good oral hygiene behavior with slightly soft plaque caused by orthognathic multiband brackets. The teeth showed neither loosening nor percussive sensitivity. In Keywords: Fibrous dysplasia; Craniofacial fibrous dysplasia;McCune-Albright syndrome; Cone beam computer tomography; Radiographic diagnostics IntroductionFibrous dysplasia (FD) is a rare, congenital malformation of the bone tissue. The disease occurs as a monostotic form, in which only a single bone is affected, and a polyostotic form involving multiple skeletal locations. Fibrous dysplasia may also occur within the context of McCune-Albright Syndrome, a severe polyostotic disease in which endocrine disorders (e.g., puberty praecox, hyperthyreodism, Cushing's disease) and pigmentation of the skin ("cafe au lait" spots) are present [1,2]. While fibrous dysplasia accounts for approximately 7% of all benign osseous tumors, McCune-Albright syndrome occurs very rarely, with a prevalence of 1/100,000 to 1/1,000,000 [3]. In 70-80% of the cases the disease manifests in a monostatic form in the femur, tibia, and ribs, and in 20-30% as a polyostotic form [4].The pathology is a mutation of the GNAS gene on chromosome 20 in early embryogenesis [5]. This gene encodes a G protein, which leads to dysregulation of the adenylyl cyclase [6]. Overproduction of cyclic adenosine monophosphate (cAMP) leads to a deficient differentiation of bone stem cells [7] and finally to the replacement of the healthy bone matrix by proliferating bone marrow stromal cells. This leads to the formation of the characteristic fibrous lesions [8,9]. Histological lesions are comprised of irregular trabecular formation within a fibrous stroma with bone marrow stromal cells without differentiation in adipocytes and osteoblasts. The bony matrix is replaced by proliferating soft tissue and shows an irregular woven structure that is described to resemble "Chinese character" pattern [8].These changes in the bony structure lead to a characteristic radiological manifestation of the disease in juvenile patients where a "ground glass"-like radiolucency enables a tenta...

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

mentioning

confidence: 99%

See 2 more Smart Citations

Various Radiographic Appearances of Fibrous Dysplasia in the Mandible - A Case Report

Angermair¹,

Fretwurst²,

Semper-Hogg³

et al. 2017

Dentistry

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“…The cause of this disorder is a gene mutation that prevents differentiation of cells within the osteoblastic lineage. [1] FD when confined to the bones within the craniofacial skeleton, it is known as craniofacial FD" (CFD). [2] It is a lesion that grows slowly and is often identified incidentally with routine imaging, particularly for monostotic disease, or when gradual swelling and facial asymmetry become noticeable.…”

Section: Introductionmentioning

confidence: 99%

Fibrous dysplasia of the craniofacial region - A brief understanding of the disease

Deepthi¹,

Jayanth²,

Begum³

et al. 2016

JCRI

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Face is the perception of a person's mind and is an important channel of a person's identity. Any abnormality in the face leads to decreased self-esteem of a person. Many syndromes and congenital disorders affect this region of the human body. One such condition which greatly affects the appearance of the face and also sometimes causing functional disturbances is fibrous dysplasia of the craniofacial region. A proper understanding of the disease is required for its appropriate management. This article helps to understand the etiology, pathogenesis, clinical, histologic, radiologic features, and various treatment options available for its management.

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Generalized Radiopacities of the Craniofacial Skeleton

Omami

Wiggins

2018

JAMA Otolaryngol Head Neck Surg

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An adolescent girl presented with a history of multiple, slowly growing masses of the face and head. The lesions were first reported when she was 5 years of age and gradually increased in size. However, her medical history was otherwise unremarkable. On clinical examination, the lesions were hard in consistency with normal overlying skin. There was significant bilateral exophthalmos (the left eye was more pronounced), with visual changes, and dental malocclusion. However, there were no clinical signs of endocrinopathy. A skeletal survey showed no involvement of other bones. The patient's serum calcium, phosphate, alkaline phosphatase, thyroid, and parathyroid hormone levels were within normal limits. A computed tomographic (CT) examination of the craniofacial skeleton was performed (Figure).

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Craniofacial Fibrous Dysplasia

Cited by 51 publications

References 56 publications

Various Radiographic Appearances of Fibrous Dysplasia in the Mandible - A Case Report

Various Radiographic Appearances of Fibrous Dysplasia in the Mandible - A Case Report

Fibrous dysplasia of the craniofacial region - A brief understanding of the disease

Generalized Radiopacities of the Craniofacial Skeleton

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