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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 investigate the characteristics of high resolution CT (HRCT) in pulmonary lymphangioleiomyomatosis (PLAM) and the changes of the images during follow-up. Methods Retrospective analysis of 10 cases of PLAM confirmed by pathological biopsy HRCT follow-up data, combined with domestic and foreign literature summary and discuss the follow-up results. Results All 10 HRCT images showed that the two lungs were diffusely distributed in the center of the lung and the surrounding area. In the follow-up of 10 patients, the number of 6 cases of cysts increased with the cyst diameter increased, followed by a case of follow-up about lung transplantation, 2 cases of lung cysts and diameter no significant change in 1 case of continuous interval 1 Follow-up after the year of progress, no follow-up after 6 months of follow-up. With signs of interstitial hyperplasia and nodules no significant change. 2 cases of pleural effusion, 1 case of absorption, 1 case decreased; 2 cases of pneumothorax, 1 case of absorption, 1 case of recurrent after resuscitation; 2 cases of liquid pneumothorax All absorbed. Complications of abdominal lymphatic drainage, renal pelvis, double ureter, left anterior mediastinal leiomyoma, retroperitoneal lymph nodes in 1 case. Conclusions The number and diameter of lung cysts are changed. The changes of fluid and pneumothorax are the main observation indexes during the follow-up. The HRCT examination is an effective diagnosis and follow-up of PLAM.