Radiography
Findings
Characteristic radiographic findings include the following:
- In the skull, the lytic phase (osteoporosis circumscripta) typically involves the frontal or occipital bones (see Image 5) and progresses to a mixed pattern with multifocal sclerotic patches in the intermediate stage of the disease, referred to as a cotton wool appearance (see Image 6).
- The vertebral bodies typically become enlarged (see Image 7) with a prominent cortical margin (picture frame vertebrae) or become densely sclerotic (see Image 8), mimicking lymphoma or metastatic disease (ivory vertebra).
- In the pelvis, typical findings include thickening of the iliopectineal line in early stages, progressing to patchy sclerosis and lucency in later stages (see Image 9).
- Weakening of the pagetic acetabular bone may lead to protrusio acetabuli and insufficiency fracture (see Image 10).
- In the long bones, early involvement consists of lysis of the subarticular bone, which advances along the diaphysis with the characteristic shape of a blade of grass (see Image 11). Long bones are affected first in the epiphyseal region, with the exception of the tibia, where Paget disease frequently begins in the tubercle (see Image 12). Later stages of disease show development of enlarged, sclerotic, deformed bones with thickened coarse trabeculae (see Image 13). The weakened femur and tibia eventually may become bowed under the stress of weight bearing. Insufficiency fractures may occur, characteristically involving the convex cortical surface (see Image 14). Conversely, Looser zones of osteomalacia typically occur on the concave cortical surface.
Computed Tomography
Findings
Cross-sectional MRI and CT demonstrate enlarged bones with trabecular coarsening and increased cortical thickness (see Images 15-16). The anatomy is well demonstrated by cross-sectional imaging in complex structures, such as the spine, where spinal or nerve root compression may be an issue (see Image 17). Cross-sectional imaging also helps delineate the pathology in complicated Paget disease, which includes nerve or spinal cord compression, as well as basilar invagination at the skull base and osseous encroachment involving cranial nerve foramina. Secondary sarcomatous development also is better evaluated with cross-sectional imaging. Additionally, should biopsy be indicated for the diagnosis of sarcoma, CT typically is the guidance modality of choice.
Magnetic Resonance Imaging
Findings
See CT Scan section.
Nuclear Imaging
Findings
Skeletal scintigraphy is useful. Radionuclide bone scans are more sensitive than radiographs for the diagnosis of Paget disease. Additionally, bone scans help survey the different sites of involvement with polyostotic disease (see Image 18). Characteristically, a marked uptake of radiopharmaceutical in the involved bones is observed (see Image 19). However, late-stage involvement may not reveal intense radiopharmaceutical uptake, and osteoporosis circumscripta may demonstrate only a peripheral rim of increased uptake. Scintigraphy tends to follow the physiologic activity of disease and may monitor treatment. Polyostotic Paget disease often can be distinguished from multiple metastatic lesions, although occasional difficulties occur. Perform radiographic correlation when this situation arises. Furthermore, the diagnosis of fracture or sarcoma may be challenging, often requiring multimodality correlation.
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References
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Further Reading
Keywords
osteitis deformans, Paget's disease
Imaging: Paget Disease