肺淋巴管肌瘤病的HRCT诊断

目的 探讨肺淋巴管肌瘤病（PLAM）高分辨率CT（HRCT）特征性表现,以期提高对该病的认识水平.方法 回顾性分析7例经病理证实的肺淋巴管肌瘤病影像学及临床资料,所有病例均行胸部常规CT及HRCT扫描.结果 常规胸部CT见两肺密度减低,偶见囊状影;HRCT清晰显示两肺弥漫、均匀分布的圆形薄壁囊状阴影,病灶之间的肺组织正常.随着病程进展,囊状阴影有增大、增多趋势,部分融合成肺大泡样改变,亦可成形态怪异的囊腔.结论 HRCT上表现为两肺弥漫、均匀分布的圆形薄壁囊状阴影,病灶之间的肺组织正常,再结合性别、年龄、进行性呼吸困难等临床资料,应考虑PLAM可能.

Diagnosis of pulmonary lymphangioleiomyomatosis by HRCT

Objective To improve the recognition of pulmonary lymphangioleiomyomatosis by investigating the characteristic features of HRCT. Methods The CT and clinical findings in 7 patients with pulmonary lymphangioleiomyomatosis （PLAM） confirmed by pathological assessment, were analyzed retrospectively. Conventional CT and HRCT were performed in all patients. Results HRCT shows numerous cystic changes that are uniformly scattered throughout the lung with normal intervening lung parenchyma and no sparing of any particular lung zones. Irregularly shaped cysts are uncommon until late in the course of disease when there is coalescence of lesions into larger, more bizarre - shaped cystic airspaces. Conclusion HRCT can suggest a diagnosis of pulmonary lymphangioleiomyomatosis by the imaging feature.