摘要
目的报告6例肺淋巴管肌瘤病(PLAM)的高分辨率CT(HRCT)特征性表现,结合病理所见进行分析,以期提高对该病的诊断水平。方法6例PLAM患者选择3个层面(即主动脉弓、右肺门及右膈上2cm层面)对无气胸侧每个病例3个肺野进行重点观察。结果PLAM的HRCT特征为气胸、胸腔积液及两肺弥漫含气小囊。未进展至肺间质纤维化者其含气小囊具有特征性表现;进展至肺间质纤维化者,含气小囊形态不规则,并出现周围肺大泡、泡性肺气肿及网格状蜂窝肺。本组6例均无纵隔淋巴结增大,仅1例伴腹膜后淋巴结肿大融合成团。结论PLAM未进展至肺间质纤维化者,其HRCT具有一定的特征性,CT可诊断此病。PLAM进展至肺间质纤维化后,则应与肺间质纤维化终末期(蜂窝肺)、肺组织细胞增生症、结节性硬化症等疾病鉴别,确诊需病理活检。
Objective To summarize the characteristic high-resolution CT （HRCT）findings of six patients with pulmonary lymphangiomyomatosis（PLAM）binding pathology,and to improve the diagnosis ability of the disease. Methods HRCT findings of six cases of PLAM were evaluated retrospectively. 18 pulmonary fields in the non-pneumothorax-side were observed focally in 3 layers （aortic arch layer,hilum of right lung layer and 2 cm superior right phrenic layer）. Results The characteristic of HRCT in PLAM is pneumothorax, hydrothorax and pneumatic saecules fill with both lungs.The pneumatic saccules have characteristic image.But in cases having progressed to pulmonary interstitial fibrosis,the form of pneumatic saccules were not regular,jumbled within cireumambient pneumatocele, phlyctenular emphysema,and netlike honeycomb lung. No enlargement of mediastinal lymph node appeared in the six cases.Tumefaction of retroperitoneal lymph node confluencing to mass appeared in only one case.Conclusion In cases of PLAM without pulmonary interstitial fibrosis have definite character of HRCT. The diadynamic value is high enough to diagnosis or suspect this case exceedingly.Difficulty is greater to diagnose with HRCT in case of PLAM with pulmonary interstitial fibrosis. Differential diagnosis should be done with diseases such as telophase of pulmonary intorstitial fibrosis （honeycomb-lung）,lung histiocytosis X and tuberous sclerosis,ere.Pathologic biopsy is necessary to make final diagnosis.
出处
《实用医学影像杂志》
2008年第3期143-145,共3页
Journal of Practical Medical Imaging
关键词
肺淋巴管肌瘤病
高分辨率CT
病理
活检
Pulmonary Lymphangiomyomatosis（PLAM）
High-resolution CT（HRCT）
Pathology
Biopsy
