Eutsler Eric P, Horton Daniel B, Epelman Monica, Finkel Terri, Averill Lauren W
Nemours Children's Health System/Alfred I. duPont Hospital or Children, 1600 Rockland Road, Wilmington, DE, 19803, USA.
Mallinckrodt Institute of Radiology, Washington University School of Medicine, St. Louis, MO, USA.
Pediatr Radiol. 2017 Apr;47(4):442-449. doi: 10.1007/s00247-016-3765-x. Epub 2017 Jan 14.
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.
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.
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).
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.
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.
青少年局限性硬皮病是一组自身免疫性疾病，临床上常以皮肤硬化区域为特征。除皮肤和皮下组织的表面变化外，青少年局限性硬皮病可能累及深部软组织、骨骼和关节，除了外观改变外，还可能导致功能障碍和疼痛。
有文献记录了成人局限性硬皮病（筋膜、肌肉、肌腱、骨骼和关节）深部受累的一系列表现，但关于儿童该疾病的文献有限。我们旨在记录儿童青少年局限性硬皮病浅表和深部受累的肌肉骨骼磁共振成像（MRI）表现谱，并评估各种MRI序列检测这些表现的效用。
两位放射科医生回顾性评估了14例青少年局限性硬皮病患儿四肢的20份MRI研究。每个成像序列还被给予主观评分0（无用）、1（有点用）或2（对检测这些表现最有用）。
在65%的成像四肢中检测到深部组织受累。在50%的成像四肢中可见筋膜增厚和强化。轴位T1、轴位T1脂肪抑制（FS）对比增强和轴位液体敏感序列被评为最有用。
筋膜增厚和强化是青少年局限性硬皮病患儿四肢MRI中最常见的深部组织表现。由于皮肤、皮下组织和筋膜的异常在受累肢体中往往呈纵向分布，成像方案应包括轴位T1、轴位液体敏感和轴位T1-FS对比增强序列。
