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Case 94 - SAPHO syndrome

from Section 12 - Tumors/Miscellaneous

Published online by Cambridge University Press:  05 July 2013

D. Lee Bennett
Affiliation:
University of Iowa
Georges Y. El-Khoury
Affiliation:
University of Iowa
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Summary

Imaging description

In adults the most frequent site for the SAPHO syndrome is the sternoclavicular region (Figure 94.1). About 65–90% of patients show involvement of the sternoclavicular region. With time all the components of the anterior chest wall can become involved particularly the sternoclavicular, upper costosternal, costochondral, and manubriosternal junctions. Radiographically the most common feature is hyperostosis, characterized by chronic periosteal reaction and cortical thickening leading to bone hypertrophy (Figure 94.1). This may be associated with enthesopathy leading to ligamentous ossification, bony bridging, and joint ankylosis. Soft tissue edema and thickening may be seen adjacent to the involved bones and joint. When the disease is advanced these changes are readily identified by radiography, but early changes of sternoclavicular hyperostosis require the use of CT for a definitive diagnosis. CT and MRI may be required for detecting serious complications of sternoclavicular hyperostosis resulting from bony overgrowth which can lead to subclavian vein obstruction and superior vena cava syndrome.

On rare occasions extra-axial SAPHO can occur in the long bones (Figure 94.2), pelvis, scapula, and tarsal bones.

Bone scintigraphy is a sensitive technique for detecting SAPHO lesions throughout the skeleton in early stages of the disease (Figure 94.1 and Figure 94.2 A). The bull horn sign in the upper anterior chest wall is diagnostic of SAPHO.

Magnetic resonance imaging demonstrates the changes due to arthritis and the bone marrow edema. MRI can also be useful in the follow-up of patients to determine active from chronic inactive lesions.

Type
Chapter
Information
Pearls and Pitfalls in Musculoskeletal Imaging
Variants and Other Difficult Diagnoses
, pp. 249 - 254
Publisher: Cambridge University Press
Print publication year: 2013

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References

Boutin, RD, Resnick, D.The SAPHO syndrome: an evolving concept for unifying several idiopathic disorders of bone and skin. AJR Am J Roentgenol 1998;170:585–591.CrossRefGoogle ScholarPubMed
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Davies, AM, Marino, AJ, Evans, N et al. SAPHO syndrome: 20-year follow-up. Skeletal Radiol 1999;28:159–162.CrossRefGoogle ScholarPubMed
Earwaker, JWS, Cotton, A.SAPHO: syndrome or concept? Imaging findings. Skeletal Radiol 2003;32:311–327.CrossRefGoogle ScholarPubMed
Kasperczyk, A, Freyschmidt, J.Pustulotic arthroosteitis: spectrum of bone lesions with palmoplantar pustulosis. Radiology 1994;191:207–211.CrossRefGoogle ScholarPubMed
Nachtigal, A, Cardinal, E, Bureau, NJ et al. Vertebral involvement in SAPHO syndrome: MRI findings. Skeletal Radiol 1999;28:163–168.CrossRefGoogle ScholarPubMed
Sartoris, DJ, Schreiman, JS, Kerr, R et al. Sternocostoclavicular hyperostosis: a review and report of 11 cases. Radiology 1986;158:125–128.CrossRefGoogle ScholarPubMed
Suh, J-S, Shin, K-H, Park, K-W.Hyperostotic and osteosclerotic changes of the tarsal navicular, associated with pustulosis palmaris and plantaris. Skeletal Radiol 1996;25:377–380.CrossRefGoogle ScholarPubMed

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  • SAPHO syndrome
  • Edited by D. Lee Bennett, University of Iowa, Georges Y. El-Khoury, University of Iowa
  • Book: Pearls and Pitfalls in Musculoskeletal Imaging
  • Online publication: 05 July 2013
  • Chapter DOI: https://doi.org/10.1017/CBO9781139031141.096
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  • SAPHO syndrome
  • Edited by D. Lee Bennett, University of Iowa, Georges Y. El-Khoury, University of Iowa
  • Book: Pearls and Pitfalls in Musculoskeletal Imaging
  • Online publication: 05 July 2013
  • Chapter DOI: https://doi.org/10.1017/CBO9781139031141.096
Available formats
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  • SAPHO syndrome
  • Edited by D. Lee Bennett, University of Iowa, Georges Y. El-Khoury, University of Iowa
  • Book: Pearls and Pitfalls in Musculoskeletal Imaging
  • Online publication: 05 July 2013
  • Chapter DOI: https://doi.org/10.1017/CBO9781139031141.096
Available formats
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