Fibrous dysplasia is a disorder where normal bone and marrow is replaced with fibrous tissue, resulting in formation of bone that is weak and prone to. Polyostotic fibrous displasia: A case report. Displasia fibrosa poliostótica: presentación de un caso. Visits. Download PDF. Carlos Francisco Meneses. Transcript of DISPLASIA FIBROSA POLIOSTOTICA. CAUSAS MUTACIONES genéticas EN EL GEN GNAS(GEN DEFECTUOSO).
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Fibrous dysplasia FD is a non-neoplastic tumor-like congenital process, manifested as a localised defect in osteoblastic differentiation and maturation, with the replacement of normal bone with large fibrous stroma and islands of immature woven bone.
Fibrous dysplasia has a varied radiographic appearance. If asymptomatic, it does not require treatment. The remainder of this article concerns itself with skeletal fibrous dysplasia.
For a discussion of craniofacial fibrous dysplasia and cherubismplease refer to the respective articles. In polyostotic form, patients usually present by 10 years old. There is no recognised gender predilection 9. The condition is often an incidental finding and is usually painless.
Alternatively, it ifbrosa present due to bony expansion or remodelling. Morbidity may arise from compression and displacement of adjacent structures. This is particularly true in craniofacial fibrous dysplasia, where the content of the orbit or cranial poliostotiva may be compressed.
Fibrous dysplasia is due to developmental dysplasia and focal arrest in normal osteoblastic activity secondary to a non-hereditary mutation which results in the presence of all of the components of normal bone with a lack of normal differentiation into their mature structures.
Macroscopically, lesions are intramedullary and well circumscribed with abnormal whitish-grey color. Microscopically it manifests as large fibrous matrix displawia scattered curvilinear irregularly shaped trabeculae of immature, inadequately mineralized bone 6.
There is no rimming by osteoblasts differentiating feature from cemento-ossifying fibroma. It is usually asymptomatic until 2 nd fibrossa rd decade but can be seen throughout adulthood 6. After puberty, the disease becomes inactive, and monostotic form does not progress to polyostotic form. As expected this presents earlier, typically in childhood mean age of 8 years with two-thirds symptomatic by the age of Ribs are the most common site of monostotic fibrous dysplasia.
Fibrous dysplasia is the most common cause of a benign expansile lesion of a rib see rib lesions. MRI is not particularly useful in differentiating fibrous dysplasia from other entities as there is marked variability in the appearance of the bone lesions, and they can often resemble a tumor or more aggressive lesions.
Demonstrates increased tracer uptake on Tc 99 bone scans lesions remain metabolically active into adulthood. Usually, no treatment is required as the bone lesions usually do not progress beyond puberty.
If a mass effect is severe, then surgical decompression may be considered. The monostotic form does not transform or progress into the polyostotic form Not surprisingly, bone affected by fibrous dysplasia is weaker than normal and thus susceptible to pathological fractures.
It should be noted that many reported cases may relate to previous treatment with radiation therapy 6. Due to the variability of the appearance of fibrous dysplasia the potential differential is very long but will be significantly influenced by the dominant pattern.
Fibrous dysplasia of bone
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About Blog Go ad-free. Cases and figures Imaging differential diagnosis. Poliosotica it at Google Books – Find it at Amazon. Related Radiopaedia articles Bone tumours The differential diagnosis for bone tumours is dependent on the age of the patient, with a very different set of differentials for the pediatric patient.
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Orphanet: Displasia fibrosa poliostotica
Case 10 Case Case 12 Case Case 13 Case Case 14 Case Case 15 Case Case 16 Case Case 17 Case Case 28 Case Mazabraud syndrome with ABC Case Mazabraud syndrome with ABC. Case 31 Case Case 32 Case poliostotic Paget’s disease Paget’s disease.
Unicameral bone cyst Unicameral bone cyst.
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