CT淋巴管造影对淋巴管肌瘤病淋巴回流障碍的诊断价值

Diagnostic value of CT lymphangiography about lymphatic flow disorder of lymphangioleiomyomatosis

目的探讨CT淋巴管造影（CTL）对淋巴管肌瘤病（LAM）淋巴回流障碍的诊断价值。方法回顾性分析21例临床和病理证实为LAM患者的临床及影像学资料，由2名医师对CTL的图像及其发生率进行分析。结果CTL示：（1）胸部异常征象：肺内多发含气囊腔21例，胸导管出口梗阻15例，纵隔密度增高13例，支气管血管束和小叶核心增厚11例，小叶间隔增厚13例，叶间裂增厚18例，肺脏实变或磨玻璃影7例，胸膜增厚19例，心包积液和心包增厚6例，气胸2例，胸腔积液21例，对比剂异常分布或堆积16例，纵隔和腋窝淋巴结肿大4例。（2）胸外异常征象：腹膜后淋巴管瘤或淋巴管平滑肌瘤16例，对比剂异常分布或堆积21例，腹腔积液10例，肠系膜水肿4例，肝脏或肾脏错构瘤5例，卵巢畸胎瘤1例，子宫肌瘤2例，骨骼内异常密度7例。结论LAM很可能是一种全身性系统性淋巴回流障碍性疾病，肺部囊性变只是LAM全身病变中的一部分。CTL能够明确淋巴回流障碍的部位、范围及程度，为临床诊断及治疗LAM提供了影像学依据。

Objective To explore the diagnostic value of CT lymphangiography （CTL） about lymphatic flow disorder of lymphangioleiomyomatosis （ LAM ）. Methods A retrospective review of the clinical and radiological data of 21 patients with LAM proved by pathology and clinical results. The findings of CTL were analyzed by 2 doctors. Results （ 1 ） The thoracic features of patients with LAM included multiple cysts of lung（ n = 21 ）, thoracic duct outlet obstruction （ n = 15 ）, hyperdense shadow of mediastinum （ n = 13 ）, bronchovaseular bundle and lobular kernel thinkening （ n = 11 ） , interlobular septal thickening （ n = 13 ）, interlobar fissure thickening （ n = 18 ）, consolidation or ground-glass opacity of lung （ n = 7 ）, pleural thickening （ n = 19 ）, pericardium thickening or pericardial effusion （ n = 6 ）, pneumothorax （ n = 2）, pleural effusion（ n = 21 ）, abnormal deposition of contrast medium （n = 16）, mediastinum and axilla lymphadenopathy （n = 4 ）. （2） The other associated extrathoracic abnormalities included retroperitoneal angiolymphoma and（or） lymphangiomyoma （ n = 16 ） , abnormal deposition of contrast medium （ n = 21 ） , abdominal effusion（ n = 10 ）, mesentery lymphedema （ n = 4 ）, renal and hepatic angiomyolipoma （ n = 5 ）, ovary teratoma （n = 1）, hysteromyoma（n =2）, abnormal density of bone（n =7）. Conclusions Multiple organs were involved in the LAM, which suggests that LAM maybe one kinds of systemic diseases with close relation with lymphatic flow disorders. CTL can clearly show the exact location and extent of lymphatic flow disorder , which provides important information for diagnosis and therapy of LAM.