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Pediatric Radiology

, Volume 47, Issue 4, pp 442–449 | Cite as

Musculoskeletal MRI findings of juvenile localized scleroderma

  • Eric P. Eutsler
  • Daniel B. Horton
  • Monica Epelman
  • Terri Finkel
  • Lauren W. AverillEmail author
Original Article

Abstract

Background

Juvenile localized scleroderma comprises a group of autoimmune conditions often characterized clinically by an area of skin hardening. In addition to superficial changes in the skin and subcutaneous tissues, juvenile localized scleroderma may involve the deep soft tissues, bones and joints, possibly resulting in functional impairment and pain in addition to cosmetic changes.

Objective

There is literature documenting the spectrum of findings for deep involvement of localized scleroderma (fascia, muscles, tendons, bones and joints) in adults, but there is limited literature for the condition in children. We aimed to document the spectrum of musculoskeletal magnetic resonance imaging (MRI) findings of both superficial and deep juvenile localized scleroderma involvement in children and to evaluate the utility of various MRI sequences for detecting those findings.

Materials and methods

Two radiologists retrospectively evaluated 20 MRI studies of the extremities in 14 children with juvenile localized scleroderma. Each imaging sequence was also given a subjective score of 0 (not useful), 1 (somewhat useful) or 2 (most useful for detecting the findings).

Results

Deep tissue involvement was detected in 65% of the imaged extremities. Fascial thickening and enhancement were seen in 50% of imaged extremities. Axial T1, axial T1 fat-suppressed (FS) contrast-enhanced and axial fluid-sensitive sequences were rated most useful.

Conclusion

Fascial thickening and enhancement were the most commonly encountered deep tissue findings in extremity MRIs of children with juvenile localized scleroderma. Because abnormalities of the skin, subcutaneous tissues and fascia tend to run longitudinally in an affected limb, axial T1, axial fluid-sensitive and axial T1-FS contrast-enhanced sequences should be included in the imaging protocol.

Keywords

Children Juvenile localized scleroderma Magnetic resonance imaging Morphea Musculoskeletal Scleroderma 

Notes

Compliance with ethical standards

Conflicts of interest

None

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Copyright information

© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  • Eric P. Eutsler
    • 1
    • 2
  • Daniel B. Horton
    • 3
    • 4
  • Monica Epelman
    • 5
  • Terri Finkel
    • 6
  • Lauren W. Averill
    • 1
    Email author
  1. 1.Nemours Children’s Health System/Alfred I. duPont Hospital or ChildrenWilmingtonUSA
  2. 2.Mallinckrodt Institute of RadiologyWashington University School of MedicineSt. LouisUSA
  3. 3.Division of Rheumatology, Department of PediatricsNemours Children’s Health System/Alfred I. duPont Hospital for ChildrenWilmingtonUSA
  4. 4.Department of PediatricsRutgers Robert Wood Johnson Medical SchoolNew BrunswickUSA
  5. 5.Department of Medical ImagingNemours Children’s Health System/Nemours Children’s HospitalOrlandoUSA
  6. 6.Department of PediatricsNemours Children’s Health System/Nemours Children’s HospitalOrlandoUSA

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