肺淋巴管肌瘤病的临床表现及HRCT特点

The Clinic and HRCT Features of Pulmonary Lymphangiomyomatosis

目的探讨肺淋巴管肌瘤病（PLAM）的临床特征及高分辨率CT（HRCT）表现,旨在提高对该病的认识。资料与方法回顾性分析9例经病理证实并行HRCT检查的PLAM患者资料,分析其临床表现、肺功能结果、X线胸片及HRCT特点。结果9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,2例伴乳糜胸。肺功能检查表现为以阻塞性通气功能障碍为主的混合性通气功能障碍和弥散功能降低;X线胸片8例呈弥漫性网状阴影,1例可见蜂窝状囊腔;HRCT扫描均见均匀散在性分布的直径2.0-20mm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,常可伴有咯血、气胸、乳糜胸,疑诊PLAM时应及时行HRCT及肺功能检查,必要时需行肺活检,以明确病理诊断。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 lymphangiomyomatosis（PLAM） and to make a further understanding of this disease. Materials and Methods 9 cases of PLAM with pathologically proved were reviewed retrospectively. Clinic manifestations, pulmonary function results, chest X ray and HRCT findings were analyzed. Results The main clinic manifestations included progressive dyspnea （ n = 9）, repeated pneumothorax （ n = 1 ）, and chylopleura （ n = 2）. Pulmonary function showed mixed ventilation disturbance with obstructive ventilation disturbance mainly. In 8 patients, chest X-ray showed reticular opacities and honeycombing opacities in 1 patient. HRCT demonstrated thin-walled cysts 2 - 20mm in diameter and 1.5 - 2.0mm in wail thickness in all patients, scattering at random in all parts of the lung. In the early stage of the disease, lung tissue between cysts appeared normal. Conclusion The main clinic manifestations of PLAM are progressive dyspnea with repeated pneumothorax, chylopleura and hemoptysis. HRCT is a valuable modality in the early diagnosis of PLAM.