肺淋巴管平滑肌瘤病影像学分析

Imaging findings of pulmonary lymphangioleiomyonmatosis

目的探讨肺淋巴管平滑肌瘤病（PLAM）胸部X线片、多排螺旋CT（MSCT）的影像学表现特点及诊断价值。方法回顾性分析经病理证实的4例PLAM的临床和胸部X线片、MSCT表现，并复习文献进行分析。结果PLAM临床主要表现为呼吸困难（4例），咳嗽、咳痰（2例），咯血（2例），反复出现的气胸（2例）和乳糜胸（1例）等。胸部X线片主要表现为弥漫性分布的网状阴影（2例）、蜂窝状透亮区（1例）、气胸（2例）和胸腔积液（1例）等；MSCT特征性表现：2例见双肺弥漫性分布、大小不一、薄壁、囊状透亮区，囊腔直径3-15mm。1例广泛性囊变合并肺间质纤维化并胸膜增厚、粘连，确诊后3个月死亡。余症状缓解出院。结论PLAM是一种罕见的弥漫性肺间质病变，胸部X线片表现缺乏特征；MSCT特征性表现对临床诊断有重要价值。

Objective To explore the chest X-ray and MSCT findings and the diagnostic value of pulmonary lymphangioleiomyomatosis （PLAM）. Methods Four cases of PLAM proven by. pathology were retrospectively analyzed, and the literature was reviewed. Result The clinical manifestations were dyspnoea （4 eases）, cough （2 eases）, haemoptysis （2 cases）,recurrent pneumothorax（2 eases）and chylous effusions （1 case）. The chest radiography presented diffused reticular shadows （2 eases）, honey-comb changes （1 case）, pneumatothorax （2 eases）, and pleural effusion （1 case）. The characteristic MSCT manifestations of 4 cases with PLAM were multiple different-sized and thin-walled cystoid transparent areas, which were diffusely-distributed throughout both lungs, most cysts were of 3-15 mm in diameter. One case combined with extensive pulmonary interstitial fibrosis, pleural thickening and adhesion. Conclusions PLAM is a kind of exceptional chronic diffuse lung interstitial disease.The chest radiography has been lack of characteristic imagines, but MSCT has characteristics of PLAM, and is more valuable for accurate clinical diagnosis of PLAM.