Prevalence of Different Forms and Involved Bones of Craniofacial Fibrous Dysplasia

Yang, Liya; Wu, Huanhuan; Lu, Jianjian; Teng, Li

doi:10.1097/scs.0000000000002830

Cited by 41 publications

(28 citation statements)

References 27 publications

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“…Additionally, patients who are diagnosed with "monostotic" FD may not be appropriately evaluated for polyostotic and endocrine disease, making it difficult to determine the relative prevalence of these subtypes. A metanalysis of 18 series including a total of 487 patients diagnosed with craniofacial FD reported that 56% had monostotic disease, 47% had polyostotic disease, and 7% had MAS [2]. It is unclear whether these patients were screened for subclinical endocrine disease, calling into question the generalizability of these findings.…”

Section: Epidemiologymentioning

confidence: 99%

Fibrous Dysplasia of Bone and McCune–Albright Syndrome: A Bench to Bedside Review

et al. 2019

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Fibrous dysplasia is an uncommon mosaic disorder in which bone is replaced by structurally unsound fibro-osseous tissue. It is caused by the sporadic post-zygotic activating mutations in GNAS, resulting in dysregulated Gα S-protein signaling in affected tissues. This manifests on a broad clinical spectrum ranging from insignificant solitary lesions to severe disease with deformities, fractures, functional impairment, and pain. Fibrous dysplasia may present in isolation or in association with hyperfunctioning endocrinopathies and café-au-lait macules, known as McCune-Albright Syndrome. This review summarizes current understanding of pathophysiology in fibrous dysplasia, describes key pre-clinical and clinical investigations, and details the current approach to diagnosis and management.

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Section: Epidemiologymentioning

confidence: 99%

Fibrous Dysplasia of Bone and McCune–Albright Syndrome: A Bench to Bedside Review

et al. 2019

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“…The long delay of the clinical management of the patient's disease would probably responsible for her considerable craniofacial deformation. The maxillary bone was described in the literature as the main site of malignant transformation, followed by the mandible, and zygoma in the case of aggressive dysplasia, [3,4].…”

Section: Epidemiological and Clinical Characteristics Of Aggressive Cmentioning

confidence: 99%

“…The McCune-Albright syndrome is a rare disease characterized by skeletal lesions, skin hyperpigmentations, and hyperfunctional endocrinopathies. It represents less than 7 % of bone dysplasia and its prevalence is estimated at between 1/100,000 and 1/1,000,000 [3,4]. It slowly evolves but might be aggressive in exceptional cases or lead to malignant transformation in 0.9-4% of cases [5,6].…”

Section: Introductionmentioning

confidence: 99%

Imaging of advanced craniofacial fibrous dysplasia associated with McCune-Albright syndrome: A case report

Bile-Gui

Ahoury

Kabas

2020

European Journal of Radiology Open

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A B S T R A C TThe fibrous dysplasia of bone is painless, benign, and slowly progressive bone lesion. It may rarely become aggressive by compression of adjacent organs or malignant transformation. This disease falls within the scope of a McCune-Albright syndrome in less than 7 % of cases. The authors reported the case of a 25-year-old woman, living in the countryside, who suffered from a severe craniofacial fibrous dysplasia which has begun since her childhood with no medical care. Her clinical picture was that of McCune-Albright syndrome, and CT scan revealed advanced dysplasia with endocranial compression.

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“…1 The prevalence of FD of the temporal bone (FDTB) is variable and accounts for up to 89% of craniofacial FD, 2 but only 12% of isolated FDTB. 3 Patients with FD usually present with headache, pain, sensory deficit, functional impairment, and infectious complications. 4 The most common presenting complaint of FDTB is hearing loss (58%) 5 and is usually conductive.…”

Section: Introductionmentioning

confidence: 99%

Middle Ear Implant in a Patient With Fibrous Dysplasia: An Alternative for Hearing Restoration

Alshawi

Alsughayer

Alradhi³

et al. 2020

Ear Nose Throat J

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We propose middle ear implants (MEI) as alternative hearing restoration option for patients with fibrous dysplasia of the temporal bone (FDTB). A 60-year-old man presented with unilateral right-sided conductive hearing loss (CHL) and FDTB. The Vibrant Soundbridge (VSB) MEI was implanted in the right ear. Preoperatively, the right side had a pure tone average (PTA) threshold of 67 dB, speech reception threshold (SRT) = 75 dB HL, and speech discrimination score (SDS) = 54% at 80 dB HL presentation level. Postoperative aided PTA threshold of the right ear was 28 db, aided SRT = 30 db, and SDS = 96% at 65dB HL. After excluding cholesteatoma, VSB provides an alternative hearing restoration technique for adults with FDTB and CHL due to stenosis of the external auditory canal and/or ossicular crowding.

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Prevalence of Different Forms and Involved Bones of Craniofacial Fibrous Dysplasia

Abstract: This meta-analysis found that monostotic and polyostotic forms of CFD shared similar prevalence rates. Furthermore, the maxilla was found to be the most commonly involved bone in both monostotic and polyostotic CFD.

Cited by 41 publications

References 27 publications

Fibrous Dysplasia of Bone and McCune–Albright Syndrome: A Bench to Bedside Review

Fibrous Dysplasia of Bone and McCune–Albright Syndrome: A Bench to Bedside Review

Imaging of advanced craniofacial fibrous dysplasia associated with McCune-Albright syndrome: A case report

Middle Ear Implant in a Patient With Fibrous Dysplasia: An Alternative for Hearing Restoration

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