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Fibrous dysplasia is a very rare [2] nonhereditary genetic disorder where normal bone and marrow is replaced with fibrous tissue, resulting in formation of bone that is weak and prone to expansion. As a result, most complications result from fracture , deformity, functional impairment, pain, and the impingement of nerves. [ 3 ]
The clivus is a shallow depression behind the dorsum sellae of the sphenoid bone. [3] It slopes gradually to the anterior part of the basilar occipital bone at its junction with the sphenoid bone. Synchondrosis of these two bones forms the clivus. The clivus extends inferiorly to the foramen magnum. [3]
It is usually diagnosed by x-ray or MRI, when investigating another problem. [1] The tumor presents as a well defined radiolucent lesion, with a distinct multilocular appearance, sometimes looking like a "soap bubble". [5] If small and no symptoms, then biopsy is not needed. [1]
Fibrous dysplasia causes bone thinning [13] and growths or lesions in one or more bones of the human body. These lesions are tumor-like growths that consist of replacement of the medullary bone with fibrous tissue, causing the expansion and weakening of the areas of bone involved. Especially when involving the skull or facial bones, the lesions ...
It comprises a majority of the cases of fibrous dysplasia (approximately 70–80%). [2] It is a rare bone disease characterized by the replacement of normal elements of the bone by fibrous connective tissue, [3] which can cause very painful swellings and bone deformities, and make bone abnormally fragile and prone to fracture. [4]
All patients with known or suspected McCune–Albright syndrome should undergo a screening evaluation for fibrous dysplasia. [18] Nuclear medicine tests such as technetium-99 scintigraphy are the most sensitive way to detect fibrous dysplasia lesions. [19] CT scan of the skull is the most useful test to evaluate craniofacial fibrous dysplasia ...
Fibrodysplasia ossificans progressiva, a rare disease in which fibrous tissue becomes ossified; Fibromuscular dysplasia, a disease characterized by the fibrous thickening of the renal artery; Fibrous dysplasia, a disease that causes growths or lesions in one or more bones of the human body
The lesion is usually identified as a well demarcated, expansile mass with an ossified rim at the periphery. Calcifications are noted throughout. MRI shows a variable finding depending on T1 or T2 weighted images, dependent on the amount of bone to fibrous connective tissue ratio. [2]