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Questions and Answers
What is fibrous dysplasia?
Which form of fibrous dysplasia is more common?
At what age is the monostotic form of fibrous dysplasia usually asymptomatic?
What percentage of cases does the polyostotic form of fibrous dysplasia account for?
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What is the genetic mutation linked to fibrous dysplasia?
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How is the diagnosis of fibrous dysplasia mainly based?
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When is histological confirmation indicated in cases of fibrous dysplasia?
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What is the aim of management for fibrous dysplasia?
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Is treatment usually required for fibrous dysplasia?
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What are the complications of fibrous dysplasia?
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Study Notes
- Fibrous dysplasia is a benign medullary fibro-osseous process that affects any bone.
- It can occur in a monostotic form involving only one bone or a polyostotic form involving multiple bones.
- The monostotic form is far more common accounting for 70-80% of cases and is usually asymptomatic until the 2nd to 3rd decade.
- The polyostotic form accounts for 20-30% and presents earlier, typically in childhood.
- Fibrous dysplasia is linked to postzygotic activating missense GNAS mutations that encode the alpha subunit of the stimulatory G-protein.
- The diagnosis of fibrous dysplasia is mainly based on clinical and typical radiographic features.
- Histological confirmation is indicated in cases with atypical imaging appearance or in isolated monostotic lesions with clinical symptoms or other concerning features.
- Management aims to establish the extent of the disease and the maintenance of bone quality via dietary measures and exercise.
- The prognosis is excellent and usually, no other treatment is required.
- Complications include pathological fractures and sarcomatous dedifferentiation, which is more common in the polyostotic form.
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Description
Test your knowledge on fibrous dysplasia, a benign medullary fibro-osseous process that affects bones. This quiz covers the different forms of the condition, its causes, diagnosis, and management. Learn about the typical clinical and radiographic features, as well as the potential complications associated with fibrous dysplasia. Test yourself on this topic and gain a better understanding of this condition.
