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Findings thus far The radiographs frankly do not show anything at all. The MRI is a bit more of an “eye test” than in most of the cases I have presented. In the clivus, there is abnormal, heterogeneous signal in all pulse sequences. I apologize if time was spent looking for more typical cervical spine pathology, but I did not otherwise want to give this case away.
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CT Findings Correlating with the findings of the MRI, there is a hazy opacification or ground glass opacification of the clivus that extends into the right occipital condyle. This is associated with a mild expansion and deformity of the bone. The findings 4 years later demonstrate no interval change although the outside institution exam was done with slightly less technique making the findings slightly less apparent.
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Diagnosis: Fibrous Dysplasia Fibrous dysplasia is a non-neoplastic disorder of bone representing a mixture of fibrous tissue and immature, woven bone. It has a variety of appearances but very frequently presents with a hazy opacification of the bone on radiographs or CT described as ground glass opacification. The lesions can cause expansion of the bone with endosteal scalloping but do not produce periosteal reaction unless there is associated fracture. These medullary lesions at times have a thick rind of sclerotic tissue (not in this example) which is classic when present. The expanded bone can become deformed as in the classic Shepherd’s crook deformity of the femur, and the expanded bone can also narrow or obliterate openings for neurovascular structures. In the example shown in the skull base, one could see how the foramina and passageways for the cranial nerves could be compromised. If the imaging findings are typical, the need for further investigation and biopsy can be obviated.
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Diagnosis: Fibrous Dysplasia, continued As seen here, the findings on MRI are often heterogeneous and at times the lesions may become encystified. An associated lesion, fibrocartilaginous dysplasia, will have punctate/ring and arc type calcifications typical of cartilage lesions. Fibrous dysplasia is a disease of children and young adults. The lesions are often found incidentally but may be associated with pain especially if bone has become weakened and a pathologic fracture precipitated. The process is most often monostotic where it involves the craniofacial bones/skull base, ribs, and long bones of the extremities. The less common polyostotic form involves a similar distribution but also frequently involves the pelvis, spine, and distal aspects of the extremities. The most common complication is fracture or potential fracture requiring curettage/grafting and potential fixation. Malignant transformation is almost unheard of in the monostotic form and extremely rare in the polyostotic form.
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References Fibrous dysplasia for radiologists: beyond ground glass bone matrix. Yevgeniya S. Kushchayeva, Sergiy V. Kushchayev, Tetiana Y. Glushko, Sri Harsha Tella, Oleg M. Teytelboym,3Michael T. Collins, and Alison M. Boyce. Insights Imaging. 2018 Dec; 9(6): 1035–1056.Published online 2018 Nov 27. doi: 10.1007/s13244-018-0666-6 Fibrous dysplasia. Dr Daniel J Bell and Dr Gagandeep Singh et al. Radiopedia
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