Craniofacial Fibrous Dysplasia presenting with Visual Impairment

Alam, Arshad; Chander, BN

doi:10.1016/s0377-1237(03)80152-1

Cited by 9 publications

(13 citation statements)

References 5 publications

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“…The craniofacial bones are affected in about 10% of cases of monostotic fibrous dysplasia and in 50%-100% of cases of polyostotic fibrous dysplasia. 16,18,19 When only the cranial and facial bones are affected, the term craniofacial fibrous dysplasia is used. Van Tillburg analyzed skull lesions from 144 patients identified in the literature and noted that the frontal bones were most commonly involved, followed by the sphenoid, ethmoid, parietal, temporal, and occipital bones.…”

Section: Epidemiologymentioning

confidence: 99%

Fibrous dysplasia of bone: a clinicopathologic review

Mohan¹

2011

PLMI

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Fibrous dysplasia of the bones is an uncommon congenital skeletal disorder that is found equally in both genders and is not inherited. Its etiology has been linked to an activating mutation of Gsα and the downstream effects of the resultant increase in cAMP. Fibrous dysplasia is categorized as either monostotic or polyostotic, and may occur as a component of McCune-Albright syndrome or the rare Mazabraud syndrome. Long bones, skull bones, and ribs are the most commonly affected bones. The radiological picture is somewhat variable, including a ground-glass appearance, expansion of the bone, and sclerosis surrounding the lesion. Histologically, fibrous dysplasia shows irregularly-shaped trabeculae of immature, woven bone in a background of variably cellular, loosely arranged fibrous stroma. It may be complicated by pathologic fracture, and rarely by malignant transformation. This review examines interesting issues surrounding the etiology of fibrous dysplasia, its clinical and laboratory manifestations, radiological picture, utility of bone biopsy, gross and microscopic pathology, complications, and its differential diagnostic considerations.

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Section: Epidemiologymentioning

confidence: 99%

Fibrous dysplasia of bone: a clinicopathologic review

Mohan¹

2011

PLMI

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“…Gender prevalence of monostotic and polyostotic fibrous dysplasia is equal. In patients with polyostotic disease, the most commonly involved bones are the craniofacial bones, ribs, and metaphysis or diaphysis of the proximal femur or tibia and the lesions are often found on one side of the body the monostotic presentation is more frequent, and lesions enlarge in proportion to skeletal growth [7][8][9].…”

Section: Discussionmentioning

confidence: 99%

“…But some adolescence patients have widespread polyostotic fibrous dysplasia may have severe deformities. The lesions Polyostotic lesions often continue to enlarge after skeletal maturity and causes progressive deformity and increase in pathologic fractures [8][9][10].…”

Section: Discussionmentioning

confidence: 99%

Polyostotic Fibrous Dysplasia of Maxillofacial Region: A Case Report

Agarwal¹

2017

J Interdiscipl Med Dent Sci

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Conditions in which normal bone is replaced with fibrous tissue containing abnormal bone or cementum constitute group of pathologies bone dysplasias. These pathologies should be differentiated from tumours, because the treatments of these pathologies are very different. Types of Fibrous dysplasia are monostotic, polyostotic or may occur as a component of McCune-Albright syndrome and Mazabraud syndrome. The most commonly affected bones are long bones, skull bones and ribs are. We present a case of polyostotic fibrous dysplasia in a 25 years old male patient.

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“…Most common are facial deformities, atypical facial pain and headaches 7 8. Additionally, cranial nerve dysfunction has also been reported including visual impairment and labyrinth involvement with sensorineural hearing loss and dizziness 9–11. In some cases FD can result in sinus obstruction and secondary sinusitis or mucocele formation.…”

Section: Discussionmentioning

confidence: 99%

Olfactory dysfunction as first presenting symptom of cranial fibrous dysplasia

et al. 2013

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Fibrous dysplasia (FD) is a benign bone disorder presenting with a variety of clinical manifestations. This is the first reported case of anosmia as presenting symptom of FD. We present the case of a 72-year-old female patient with a progressive olfactory dysfunction. Clinical examination revealed evidence of chronic rhinosinusitis; therefore the patient was treated with a course of oral corticosteroids. The patient had no improvement in her olfactory ability and imaging studies were ordered. Bony lesions characteristic of craniofacial FD were found, causing obstruction of the central olfactory pathway. This case emphasises the need to conduct further investigations in patients with rhinosinusitis and olfactory dysfunction especially when they present no response to oral steroid treatment.

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Craniofacial Fibrous Dysplasia presenting with Visual Impairment

Cited by 9 publications

References 5 publications

Fibrous dysplasia of bone: a clinicopathologic review

Fibrous dysplasia of bone: a clinicopathologic review

Polyostotic Fibrous Dysplasia of Maxillofacial Region: A Case Report

Olfactory dysfunction as first presenting symptom of cranial fibrous dysplasia

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