肺淋巴管肌瘤病的CT表现(附5例报告)

目的 探讨肺淋巴管肌瘤病CT表现以期提高对该病的认识水平。资料与方法 对5例经病理证实的肺淋巴管肌瘤病CT资料进行回顾性分析。所有病例均行胸部常规CT及HRCT扫描；其中1例行腹部CT扫描。结果 常规胸部CT见两肺密度减低，偶见囊状影，相应水平HRCT清晰显示全肺囊状影，无分布差异。随病程进展，囊状影数目增多，部分融合成肺大泡或气胸。1例伴右肾错构瘤及后腹膜淋巴结肿大，随访3年腹部病灶无明显改变。结论 常规CT可显示肺淋巴管肌瘤病部分肺部改变，而HRCT全面显示肺部异常，已成为本病的基本影像检查方法；肺外表现系重要的诊断佐证。

CT Findings of Pulmonary Lymphangiomyomatosis:A Report of Five Cases

Objective To discuss CT features of pulmonary lymphangiomyomatosis and to make a further understanding of the disease.Materials and Methods Both thoracic CT and HRCT scanning were performed in 5 cases with pathologically-proved pulmonary lymphangiomyomatosis. Additional abdominal CT scanning was carried out in one case. CT findings were retrospectively analyzed.Results On conventional CT scans, decreased density of both lungs and occasional cystic shadow were seen. On HRCT, multiple thin-walled cysts throughout both lungs were demonstrated. 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. In one case, right renal hamartoma and retroperitoneal lymphadenopathy were accompanied, and no any change of the abdominal lesions was observed during 3 years follow-up. Conclusion HRCT is superior to conventional CT in visualizing cystoid degeneration of pulmonary lymphangiomyomatosis, therefore, it should be used as the first choice for the diagnosis of the disease. Extrapulmonary findings are helpful supplementary evidence for the diagnosis.