儿童复杂性淋巴管异常的影像学
本文阐述经淋巴结造影剂注射结合动态对比增强MR淋巴管造影可深入揭示儿童复杂性淋巴管异常的表型，并强调支持治疗、局部治疗及分子靶向治疗的多学科整合，影像科医生在基因型-表型匹配、个体化治疗和疗效监测中起关键作用。
要点
- 经淋巴结造影剂注射联合动态对比增强MR淋巴管造影是评估儿童复杂性淋巴管异常表型的关键技术。
- 影像科医生需与儿科、介入、外科、遗传等多学科协作，实现基因型-表型匹配的个体化诊疗。
- 靶向分子治疗适用于突变驱动的复杂性淋巴管异常，影像可用于监测治疗反应。
关键影像表现/征象
- 经淋巴结注射对比剂可显示淋巴管，动态对比增强MR淋巴管造影有助于表征复杂性淋巴管异常的淋巴管形态和引流。
临床应用与阅片要点
- 对儿童复杂性淋巴管异常，可采用经淋巴结造影剂注射联合动态对比增强MR淋巴管造影评估淋巴管解剖和功能表型。
- 影像评估应结合基因型与表型，支持多学科团队制定局部治疗（引流、栓塞）和系统治疗（含靶向分子治疗）方案。
- 影像可用于监测治疗反应，尤其对突变驱动的复杂性淋巴管异常接受靶向治疗者。
局限性
- 本文为综述，摘要未报告具体研究设计、样本量、中心数或证据分级，结论主要基于叙述性总结。
以上解读依据论文摘要（全文未开放获取或许可不允许翻译转载）。
英文原文（摘要）· 点击展开
The technique of intranodal contrast injection to visualize the lymphatic vessels, along with the use of dynamic contrast-enhanced MR lymphangiography, has led to important insights into the phenotype of patients with complex lymphatic anomalies. The pediatric patient with a complex lymphatic anomaly benefits from an integration of therapy across modalities, including supportive strategies (diet), localized therapy (drainage, embolization), and systemic therapy including targeted molecular therapy in mutation-driven complex lymphatic anomalies. A multidisciplinary collaboration, involving pediatricians, pharmacotherapeutic experts, pediatric radiologists, interventional radiologists, surgeons, geneticists, and nutritionists, is essential to tailor diagnosis to genotype and phenotype, personalize treatment, and monitor response.
引用
- Klein WM, Biko DM, Gerwins P, et al. Imaging of complex lymphatic anomalies in children. Pediatr Radiol. 2026. PMID: 42024244. doi:10.1007/s00247-026-06608-z
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。