Division of Plastic Surgery, Department of Surgery, University of Pennsylvania, Philadelphia, Pennsylvania.
Section of Interventional Pulmonology, Division of Pulmonary and Critical Care, Department of Medicine, University of Pennsylvania, Philadelphia, Pennsylvania.
Ann Thorac Surg. 2022 Mar;113(3):e219-e221. doi: 10.1016/j.athoracsur.2021.05.039. Epub 2021 Jun 9.
Noonan syndrome is a disorder characterized by central and peripheral lymphatic conducting anomalies, leading to chylothorax, chylous ascites, and metabolic derangement. Novel imaging methods, including dynamic contrast magnetic resonance lymphangiography and intranodal lymphangiography, have allowed for increased visualization of lymphatic pathology. Severe pulmonary insufficiency and chylothoraces developed in a 61-year-old man with Noonan syndrome. Dynamic contrast magnetic resonance lymphangiography and intranodal lymphangiography demonstrated central thoracic duct (TD) occlusion. The patient's condition significantly improved after a microsurgical TD-venous anastomosis assisted by TD catheterization for imaging guidance, resulting in decompression of the lymphatic system and resolution of the pulmonary symptoms.
努南综合征是一种以中央和外周淋巴管异常为特征的疾病，导致乳糜胸、乳糜性腹水和代谢紊乱。新的成像方法，包括动态对比磁共振淋巴管造影术和淋巴结内淋巴管造影术，使得淋巴管病理的可视化程度增加。一名 61 岁的努南综合征患者出现严重的肺功能不全和乳糜胸。动态对比磁共振淋巴管造影术和淋巴结内淋巴管造影术显示中央胸导管（TD）阻塞。在 TD 导管介入成像引导下进行显微 TD-静脉吻合术，使患者的病情得到显著改善，从而使淋巴系统减压，肺部症状得到缓解。
