肺淋巴管肌瘤病的临床及多排螺旋CT表现

Clinical manifestations and multi-detector CT features of pulmonary lymphangiomyomatosis

目的探讨肺淋巴管肌瘤病(PLAM)临床及多排螺旋CT(MDCT)影像表现,提高对该病的认识和诊断正确率。方法回顾性分析2016年1月至2018年12月期间平顶山市平煤神马医疗集团总医院6例经病理证实的PLAM患者的临床、MDCT影像资料,对其临床及MDCT影像表现进行总结、分析。结果6例PLAM患者均为生育期女性,年龄34~53岁,平均(44.67±7.47)岁。所有患者临床表现主要为不同程度的劳力性呼吸困难,3例患者行肺功能检查显示为阻塞性通气功能障碍;其中其MDCT影像表现为双肺散在多发薄壁囊腔,囊腔分布无特异性,囊腔直径0.3~3.6 cm,囊壁厚度<2 mm,囊壁光滑且厚度均匀,部分囊壁边缘可见血管走行。结论PLAM患者多表现为进行性劳累性呼吸困难,肺功能检查多为阻塞性通气功能障碍,其MDCT影像表现较有特征性,其主要表现为肺内散在的形态相对均匀的薄壁囊腔,MDCT是该病的重要检查方法。

Objective To analyze the clinical manifestations and multi-detector CT(MDCT)features of the pulmonary lymphangiomyomatosis(PLAM),to improve the recognition and diagnostic accuracy of PLAM.Methods The clinical and MDCT imaging data of 6 patients confirmed as PLAM by pathology in Pingdingshan Pingmei Shenma Medical Group General Hospital from Jan.2016 to Dec.2018 were retrospectively analyzed.The clinical manifestations and MDCT features were summarized and analyzed.Results All the 6 patients with PLAM were women of childbearing age,ranging from 34 to 53 years old,with an average of(44.67±7.47)years.The main clinical manifestations included laborious dyspnea to varying degrees in all the 6 patients and obstructive ventilation dysfunction in 3 patients(shown by pulmonary function examination).The MDCT images showed multiple thin-walled cysts scattered in the lungs(with no specific distribution).The cysts had a diameter of 0.3-3.6 cm and a thickness of<2 mm,with smooth walls and uniform thickness,and blood vessels near the margin of some cysts.Conclusion Most patients with PLAM manifested progressive exhaustive dyspnea and obstructive dyspnea(in pulmonary function examination).MDCT showed scattered thin-walled cysts with relatively uniform morphology in the lungs,which was an important examination method for the diagnosis of PLAM.