Center for Lymphatic Disorders, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA; Department of Radiology, Nemours/AI DuPont Children's Hospital, Wilmington, DE.
Department of Radiology, Nemours/AI DuPont Children's Hospital, Wilmington, DE.
Chest. 2020 Aug;158(2):681-691. doi: 10.1016/j.chest.2020.02.058. Epub 2020 Mar 24.
Pulmonary involvement in lymphatic anomalies (LA) is associated with significant morbidity and mortality. Dynamic contrast-enhanced magnetic resonance lymphangiography (DCMRL) is capable of imaging the lymphatic system in a variety of pulmonary lymphatic disorders.
The objective of this study is to describe the central lymphatic anatomy in patients with LA and pulmonary involvement on DCMRL.
This prospective observational study enrolled 16 patients with LA (mean age, 17 years; range, 6-63 years; ratio of female to male patients, 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.
T2-weighted imaging showed the following: mediastinal soft tissue masses in 10 patients, diffuse pulmonary interstitial thickening in 13 patients, and bone involvement in 15 patients. DCMRL revealed abnormal pulmonary lymphatic flow in 14 of 16 patients. Abnormal pulmonary lymphatic flow originated from the TD in three of 14 patients, the retroperitoneum in six of 14 patients, and both the TD and retroperitoneum in four of 14 patients. In nine of 16 patients, the TD was dilated and tortuous. In two patients the TD was not identified, and in five patients it was normal.
Abnormal pulmonary lymphatic flow/perfusion from the TD or retroperitoneum into the 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 whether mechanical cessation of this abnormal flow can improve pulmonary function and prolong survival in patients with LA.
Clinicaltrials.gov; No.: NCT02744027; URL: www.clinicaltrials.gov.
淋巴管异常（LA）的肺部受累与显著的发病率和死亡率相关。动态对比增强磁共振淋巴造影术（DCMRL）能够在多种肺部淋巴疾病中成像淋巴系统。
本研究的目的是描述 DCMRL 上有 LA 和肺部受累的患者的中央淋巴解剖结构。
这项前瞻性观察研究纳入了 16 名有 LA（平均年龄 17 岁；范围 6-63 岁；男女比例 9:7）且肺部受累的患者。所有患者均接受了 DCMRL。评估了淋巴系统是否存在纵隔肿块、间质性肺病、胸导管（TD）的大小和迂曲以及异常肺淋巴流的存在。
T2 加权成像显示：10 名患者有纵隔软组织肿块，13 名患者有弥漫性肺间质增厚，15 名患者有骨受累。16 名患者中有 14 名存在异常肺淋巴流。14 名患者中有 3 名异常肺淋巴流起源于 TD，6 名来自腹膜后，4 名来自 TD 和腹膜后。16 名患者中有 9 名 TD 扩张且迂曲。2 名患者 TD 无法识别，5 名患者 TD 正常。
在这项研究的大多数患者中，TD 或腹膜后的异常肺淋巴流/灌注进入肺实质。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。未来的研究将确定是否可以通过机械阻断这种异常流量来改善 LA 患者的肺功能并延长其生存时间。
Clinicaltrials.gov；编号：NCT02744027；网址：www.clinicaltrials.gov。
