eMedicine Specialties > Radiology > Musculoskeletal

Paget Disease: Imaging

Author: Mitchell J Kline, MD, Consulting Staff, Department of Diagnostic Radiology, University of Louisville, Clark Memorial and Floyd Memorial Hospitals
Contributor Information and Disclosures

Updated: Dec 12, 2007

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.

More on Paget Disease

Overview: Paget Disease
Imaging: Paget Disease
Follow-up: Paget Disease
Multimedia: Paget Disease
References

References

  1. Paget J. On a form of chronic inflammation of bones (osteitis deformans). Med Chir Tr. 1877;60:37.

  2. Singer FR. Update on the viral etiology of Paget''s disease of bone. J Bone Miner Res. Oct 1999;14 Suppl 2:29-33. [Medline].

  3. Barker DJ. The epidemiology of Paget''s disease of bone. Br Med Bull. Oct 1984;40(4):396-400. [Medline].

  4. Siris ES, Ottman R, Flaster E. Familial aggregation of Paget''s disease of bone. J Bone Miner Res. May 1991;6(5):495-500. [Medline].

  5. Layfield R. The molecular pathogenesis of Paget disease of bone. Expert Rev Mol Med. 2007;9(27):1-13. [Medline].

  6. Guyer PB, Chamberlain AT. Paget''s disease of bone in two American cities. Br Med J. Apr 5 1980;280(6219):985. [Medline].

  7. Barker DJ, Clough PW, Guyer PB. Paget''s disease of bone in 14 British towns. Br Med J. May 7 1977;1(6070):1181-3. [Medline].

  8. Schmorl G. Ueber Ostitis deformans Paget. Virchows Arch. 1932;283:694-751.

  9. Rojas-Villarraga A, Patarroyo PA, Contreras AS, Restrepo JF, Iglesias-Gamarra A. Paget disease of bone in Colombia and Latin America. J Clin Rheumatol. Apr 2006;12(2):57-60. [Medline].

  10. Harrington KD. Surgical management of neoplastic complications of Paget''s disease. J Bone Miner Res. Oct 1999;14 Suppl 2:45-8. [Medline].

  11. Smith J, Botet JF, Yeh SD. Bone sarcomas in Paget disease: a study of 85 patients. Radiology. Sep 1984;152(3):583-90. [Medline].

  12. Deyrup AT, Montag AG, Inwards CY, Xu Z, Swee RG, Krishnan Unni K. Sarcomas arising in Paget disease of bone: a clinicopathologic analysis of 70 cases. Arch Pathol Lab Med. Jun 2007;131(6):942-6. [Medline].

  13. , Sharma H, Mehdi SA, MacDuff E, Reece AT, Jane MJ. Paget sarcoma of the spine: Scottish Bone Tumor Registry experience. Spine. May 20 2006;31(12):1344-50. [Medline].

  14. Hamdy RC. Paget's Disease of the Bone: Assessment and Management. Westport, CT:. Greenwood Publishing Group;1981.

  15. Gebhart M, Vandeweyer E, Nemec E. Paget''s disease of bone complicated by giant cell tumor. Clin Orthop. Jul 1998;(352):187-93. [Medline].

  16. Altman RD, Collins B. Musculoskeletal manifestations of Paget''s disease of bone. Arthritis Rheum. Oct 1980;23(10):1121-7. [Medline].

  17. Tiegs RD. Paget''s disease of bone: indications for treatment and goals of therapy. Clin Ther. Nov-Dec 1997;19(6):1309-29; discussion 1523-4. [Medline].

Further Reading

Keywords

osteitis deformans, Paget's disease

Contributor Information and Disclosures

Author

Mitchell J Kline, MD, Consulting Staff, Department of Diagnostic Radiology, University of Louisville, Clark Memorial and Floyd Memorial Hospitals
Mitchell J Kline, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Radiology, American Roentgen Ray Society, Radiological Society of North America, and Society of Skeletal Radiology
Disclosure: Nothing to disclose.

Medical Editor

Leon Lenchik, MD, Director, Densitometry Minifellowship, Assistant Professor, Department of Radiology, Wake Forest University Medical Center
Leon Lenchik, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, and Radiological Society of North America
Disclosure: Nothing to disclose.

Pharmacy Editor

Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.

Managing Editor

Wilfred CG Peh, MD, MBBS, FRCP(Glasg), FRCP(Edin), FRCR, MHSM, Clinical Professor, Faculty of Medicine, National University of Singapore; Senior Consultant Radiologist, Programme Office, Singapore Health Services
Wilfred CG Peh, MD, MBBS, FRCP(Glasg), FRCP(Edin), FRCR, MHSM is a member of the following medical societies: American Roentgen Ray Society, British Institute of Radiology, International Skeletal Society, Radiological Society of North America, Royal College of Physicians, and Royal College of Radiologists
Disclosure: Nothing to disclose.

CME Editor

Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.

Chief Editor

Felix S Chew, MD, MBA, EdM, Professor, Department of Radiology, Vice Chairman for Radiology Informatics, Section Head of Musculoskeletal Radiology, University of Washington
Felix S Chew, MD, MBA, EdM is a member of the following medical societies: American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.

 
 
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