【摘要】 目的 分析肺淋巴管肌瘤病(PLAM)的CT特点。方法 回顾性分析14例病理证实为PLAM患者的高分辨率CT(HRCT)及临床表现,并结合国内外文献,分析该病的临床及CT特征。结果 14例均为女性,就诊时均以不同程度活动后呼吸困难为主要表现。在常规CT扫描上见两肺散在或广泛均匀分布的透亮影及囊状影,囊壁较模糊;HRCT上表现为均匀清晰的薄壁囊腔,囊腔直径数毫米至25 mm,壁厚1～2 mm,囊气腔被肺组织环绕,血管位于囊腔周围,囊腔中央未见小叶核心组织,病变大小不一,分布无规律性。6例患者有肺外CT表现:1例伴颅内多发硬化、肝肾血管平滑肌脂肪瘤以及肝多发血管瘤,3例伴纵隔及肝肾血管平滑肌脂肪瘤,2例伴腹膜后淋巴管肌瘤病。结论 PLAM的CT可见双肺弥漫分布薄壁囊腔,壁厚大致均匀;HRCT典型表现结合临床资料对PLAM的早期诊断及鉴别诊断具有重要意义。更多还原

【Abstract】 Objective To explore the CT features of pulmonary lymphangiomyomatosis(PLAM).Methods Clinical and high resolution CT(HRCT)data of 14 patients with pathologically proved PLAM were analyzed retrospectively.The clinical and CT features were summarized by combining the literatures.Results All 14 cases were female.They all presented with dyspnea in different degree after the activity.Scattered or widely distributed translucent and cystic lesions with indistinct walls in bilateral lungs were seen on routine CT images.HRCT showed homogeneous clear thin-walled cysts with diameter ranging from several millimeters to 25 mm,wall thickness of 1-2 mm,and surrounded by normal lung tissue.Meanwhile,blood vessels were found around the cysts,and there were no central lobular cores.The cysts were different sizes and irregular distribution.6 patients had extra-pulmonary CT manifestations:1 case with intracranial multiple sclerosis,hepatic and renal angiomyohpomas,and hepatic multiple hcmangiomas,3 cases with mediastinal,hepatic and renal angiomyolipomas,and 2 cases with retroperitonea lymphangioleiomyomatosis.Conclusion The CT of PLAM is characterized by the diffuse distribution of thin-walled cystic cavities and the wall thickness is generally uniform.The typical manifestations of HRCT combined with clinical data have great values in the early diagnosis and differential diagnosis.更多还原