BACKGROUND Pulmonary involvement in lymphatic anomalies (LA) is associated with significant morbidity and mortality. Recently described Dynamic Contrast Enhanced Magnetic Resonance lymphangiography (DCMRL) is capable of imaging the lymphatic system in a variety of pulmonary lymphatic disorders. RESEARCH QUESTION The objective of this study is to describe the central lymphatic anatomy in patients with LA and pulmonary involvement on DCMRL. STUDY DESIGN AND METHODS This prospective observational study enrolled 16 LA patients (mean age 17 years, range 6-63, F/M-9/7) with pulmonary involvement. All patients underwent DCMRL. The lymphatic system was assessed for the presence of mediastinal masses, interstitial lung disease, size and tortuosity of the thoracic duct (TD) and presence of abnormal pulmonary lymphatic flow. RESULTS T2 weighted imaging showed: Mediastinal soft tissue masses in 10 patients, diffuse pulmonary interstitial thickening in 13 patients, bone involvement in 15 patients. DCMRL revealed abnormal pulmonary lymphatic flow in 14/16 patients. Abnormal pulmonary lymphatic flow originated from the TD in 3/14, retroperitoneum in 6/14, and both TD and retroperitoneum in 4/14. In 9/16 patients, the TD was dilated and tortuous. In two patients, the TD was not identified and in 5 patients, it was normal. INTERPRETATION Abnormal pulmonary lymphatic flow/ perfusion from the TD or retroperitoneum into lung parenchyma occurred in the majority of patients in this study. These findings can explain the interstitial lung disease and chylothorax resulting in deterioration of respiratory function in these patients. Future studies will determine, if mechanical cessation of this abnormal flow can improve pulmonary function and prolong survival in LA patients.

中文翻译：

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淋巴管异常和呼吸功能不全患者的肺淋巴流异常

背景淋巴管异常 (LA) 中的肺部受累与显着的发病率和死亡率相关。最近描述的动态对比增强磁共振淋巴管造影术 (DCMRL) 能够对各种肺淋巴系统疾病的淋巴系统进行成像。研究问题 本研究的目的是描述 DCMRL 中 LA 和肺部受累患者的中央淋巴管解剖结构。研究设计和方法 这项前瞻性观察研究招募了 16 名肺部受累的 LA 患者（平均年龄 17 岁，范围 6-63，F/M-9/7）。所有患者均接受了DCMRL。评估淋巴系统是否存在纵隔肿块、间质性肺疾病、胸导管 (TD) 的大小和弯曲度以及异常肺淋巴流动的存在。结果 T2加权成像显示：纵隔软组织肿块10例，弥漫性肺间质增厚13例，骨受累15例。DCMRL 显示 14/16 名患者的肺淋巴流动异常。3/14 起源于 TD，6/14 起源于腹膜后，4/14 起源于 TD 和腹膜后。在 9/16 患者中，TD 扩张和弯曲。在 2 名患者中，TD 未确定，在 5 名患者中，它是正常的。解释 本研究中的大多数患者发生了从 TD 或腹膜后进入肺实质的异常肺淋巴流动/灌注。这些发现可以解释导致这些患者呼吸功能恶化的间质性肺病和乳糜胸。未来的研究将确定，