儿童局限性硬皮病的MRI评估：浅层与深层组织受累及临床实践影响
回顾性纳入44例确诊儿童局限性硬皮病患者，25年间行MRI检查，由两名儿科放射科医师独立评估预设的浅层和深层影像特征。结果显示浅层受累占95.5%，深层受累占59.1%，活动性深层炎症占54.5%，MRI与临床活动度、损伤或亚型无相关性，但MRI在95.3%的病例中有临床价值，34.9%的病例影响了管理。
要点
- 儿童局限性硬皮病MRI常见浅层受累（95.5%）和深层受累（59.1%），后者常临床隐匿。
- MRI可发现临床未怀疑的双侧病变（20.6%下肢研究），并影响34.9%病例的管理。
- 建议采用简化标准化MRI协议并常规增强，以优化诊断产出，但需注意皮肤增厚评估受体素大小和化学位移伪影限制。
英文原文（摘要）· 点击展开
BACKGROUND: Pediatric morphea (also known as localized scleroderma) is an inflammatory disorder that can involve not only the skin but also deeper tissues such as fascia, muscle, and bone. Clinical examination often underestimates the depth and activity of disease, creating a need for objective imaging tools to guide management. Despite increasing clinical reliance on MRI, data describing MRI findings and clinical utility in pediatric morphea remains limited.
OBJECTIVE: To characterize superficial and deep tissue abnormalities on MRI in patients with pediatric morphea, assess correlations with clinical disease activity and subtype, evaluate the clinical impact of MRI on management, and propose an optimized MRI protocol.
MATERIALS AND METHODS: This retrospective study included 44 pediatric patients with confirmed morphea who underwent MRI over 25 years. Two pediatric radiologists independently evaluated each MRI for predefined superficial and deep imaging features, and interobserver agreement was calculated. Clinical data, including disease activity, subtype, treatment, and MRI utility, were extracted from electronic medical records. Associations between MRI findings and clinical variables were assessed using Fisher's exact test.
RESULTS: Superficial involvement was present in 42/44 (95.5%) of patients, and deep involvement in 26/44 (59.1%). Active deep inflammation was identified in 24/44 (54.5%). No correlation was found between MRI findings and clinical activity, damage, or morphea subtype. Bilateral disease was present in 7/34 (20.6%) of lower extremity studies, most clinically unsuspected. MRI was clinically useful in 41/43 (95.3%) of cases and helped with management in 15/43 (34.9%). Enhancement was considered useful in 17/25 (68.0%) of cases. MRI assessment of skin thickening was limited by voxel size and chemical shift artifact.
CONCLUSION: MRI is a highly valuable, independent tool for evaluating patients with pediatric morphea, frequently identifying clinically occult deep involvement and influencing management. A simplified, standardized MRI protocol with routine contrast may optimize diagnostic yield.
引用
- Reyes Recasens MP, Pope EI, Sibbald C, et al. Pediatric morphea: MRI evaluation of superficial and deep tissue involvement and impact in clinical practice. Pediatr Radiol. 2026. PMID: 42176060. doi:10.1007/s00247-026-06647-6
供执业医师工作参考，不替代临床判断；数值与分级以指南原文为准。