肺淋巴管平滑肌瘤病的HRCT随访研究

Follow Up Study of Pulmonary Lymphangioleiomyomatosis Using High Resolution CT

目的探讨肺淋巴管平滑肌瘤病(pulmonary lymphangioleiomyomatosis,PLAM)的高分辨率CT(HRCT)特征及随访中影像变化规律。方法回顾分析经病理活检证实的10例PLAM患者的HRCT随访资料,结合国内外文献总结并讨论随访结果。结果 10例HRCT像上均表现为两肺弥漫均匀分布于肺中心及周边区域、大小不等的边界清楚的薄壁含气囊腔,以中等大小囊肿为主。在对10例患者随访中,6例肺囊肿数目增多伴随囊肿直径增大,1例在随访过程中先后行左右肺移植术,2例肺部囊肿数目及直径无显著改变,1例连续间隔1年后随访出现进展,再次随访6个月后无改变。伴随征象中间质增生及类结节影无显著改变。并发症3例肺不张中2例复张、1例范围缩小;2例胸腔积液,1例吸收、1例减少;2例气胸,1例吸收、1例吸收后复发;2例液气胸均吸收。合并症有腹腔淋巴管扩张,右肾双肾盂、双输尿管畸形,左前纵隔血管平滑肌瘤,后腹膜淋巴结肿大各1例。结论肺部囊肿的数目及直径改变,液、气胸改变是随访过程的主要观察指标,HRCT检查是PLAM有效的诊断及随访手段。

Objective To assess the high-resolution computed tomography(HRCT) features of pulmonary lymphangioleiomyomatosis(PLAM).Methods Ten cases(female,average age of 43.8 years)of PLAM proved by pathology were collected in the study.The HRCT findings and follow up results were analyzed retrospectively.Results On HRCT imagings,lesions showed multiple air filled thin walled cysts with varying size and clear boundary distributed throughout both lungs.On follow up imagings,the number and diameter of lung cysts were increased in 6 patients,steady in 2 patients,One patient had lung transplantation during the follow up.Accompanied signs including the thickening septum and ground glass opacities had no change.Complication included atelectasis,pleural effusion,pneumothorax and hydropneumothorax.Among 3 patients with pulmonary atelectasis,the lungs were expanded in 2 patients,partial expanded in 1 patient.Among 2 patients with pleural effusion,the pleural effusion was absorbed in one patient and reduced in the another patient.Among 2 patients with pneumothorax,the pneumothorax was absorbed in one patient and recurred in another patientafter absorption.The hydropneumothorax in 2 patients were absorbed.The other findings included abdominal lymph vessel enlargement,double ureter pelvis and mediastinal vascular leiomyoma,enlarged abdominal lymph nodes.Conclusion HRCT can be a valuable diagnostic tool in PLAM follow up.The change of lung cysts and pleural lesion is the main observing index during HRCT follow up.