肺淋巴管肌瘤病的胸部影像学表现

目的探讨肺淋巴管肌瘤病（PLAM）的影像学表现。方法报告3例PLAM病例,结合文献资料对该病病因、临床特点及影像学表现进行讨论。结果 3例患者均为育龄期女性,首发症状均为活动后呼吸困难,其中1例合并气胸,1例合并气胸及乳糜胸,1例合并肺外淋巴管肌瘤。结合文献资料,本病常发于育龄期女性,临床表现依次为呼吸困难、咯血、气胸、乳糜胸等。本病X线片常呈非特异改变,CT较具特异性,主要表现为多发均匀分布囊状影,伴有间隔线,呈网格状改变,同时出现液气胸和淋巴结肿大,肺外也可有多发相关改变。肺功能测定常伴有阻塞性通气功能障碍。结论 CT检查对PLAM具有诊断价值。对育龄期妇女发生的渐进性呼吸困难、咯血、自发性气胸应及时进行胸部CT检查,并应常规进行腹部和盆腔影像学检查以了解患者是否合并肺外淋巴管平滑肌瘤。

Imagine diagnosis of pulmonary lymphangiomyomatosis

Objective To explore the imaging features of the Pulmonary lymphangiomyomatosis（PLAM）.Methods Three cases of chest film and CT were confirmed by pathology,the imaging material and clinical data were analyzed,along with a review of the related literature.Results The three cases all were female of childbearing ages,imaging appearance is extensive honeycomb and cystic airspace patter,of which one case were complicated with chylothorax,one with recurrent pneumothorax and chylous effusion.The number of the cysts was increasing with the progress of the disease,with some cysts being coalesced together to form bullae or cause pneumothorax.Whereas,according to the literature,changes of the abdominal lesions were accompanied,too,such as the renal hamartoma and retroperitoneal lymphadenopathy.Conclusion PLAM should be suspected in women of childbearing age if there were recurrent pneumothorax and unknown etiological dyspnea,hemoptysis or chylothorax.Lung function test and CT should be immediately performed for an early diagnosis.