目的 分析肺淋巴管肌瘤病(PLAM)的临床特征及高分辨率CT(HRCT)表现,进一步提高对该病的认识.方法 回顾性分析3例经病理证实并行HRCT检查的LAM患者资料,分析其胸部HRCT特点.结果 3例均以进行性呼吸困难为主要临床表现,1例伴有反复自发性气胸,1例伴乳糜胸及腹膜后淋巴管扩张.HRCT示双肺弥漫囊样影,分布均匀,大小形态较一致,部分囊腔有融合,病变直径为2.0～20.0mm的薄壁囊腔,壁厚1.5～2.0mm,病变早期囊腔之间可见正常肺组织.结论 PLAM主要临床特征是进行性呼吸困难,常伴有乳糜胸、气胸、腹膜后淋巴管扩张、淋巴管瘤等,HRCT对该病的早期诊断、鉴别诊断具有重要价值.%Objective To discuss clinic and high resolution computed tomograghy (HRCT) features in pulmonary lym-phangiomyomatosis (PL AM) and to make a further understanding of this disease. Methods 3 cases of PL AM with pathologically proved were reviewed retrospectively. HRCT findings were analyzed. Results The main clinic manifestations of all 3 cases were progressive dyspnea. One case was with accompanied repeatedly spontaneous pneumothorax and the other with chylothorax and lymphangiectasis in subsequence peritoneum. HRCT demonstrated suffusion cysts in both lungs, the cysts were well-distributed, size and shape was conformity, part cysts was fused. The thin walled cysts were 2 - 20 mm in diameter and 1.5- 2. 0 mm in wall thickness in all patients. In the early stage of the disease, lung tissue between cysts appeared normal. Conclusion The main clinic manifestations of PL AM are progressive dyspnea, frequently with chylothorax, pneumothora and lymphangiectasis or lymphangioma behind peritoneum etc. HRCT is a valuable modality in the early diagnosis of PLAM.
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