HRCT扫描对肺淋巴管平滑肌瘤病的肺部结构研究

Lung structural assessment in pulmonary lymphangioleiomyomatosis using HRCT

目的:评价HRCT扫描对肺淋巴管平滑肌瘤病患者肺部结构研究的可行性。方法:收集61例肺淋巴管平滑肌瘤病患者的临床资料,分析HRCT图像表现、分级,不同级别患者肺结构破坏情况。结果:肺淋巴管平滑肌瘤病患者肺部以多发散在囊腔为主要表现,囊腔呈圆形或椭圆形,平均直径(1.22±0.76)cm,囊壁薄,均匀,平均厚度(0.86±0.37)mm。肺部合并气胸27例(44.26%)、胸腔积液19例(31.15%)、肺部实变16例(26.23%)、小叶间隔增厚16例(26.23%)、纤维灶13例(21.31%)、肺部钙化灶9例(14.75%)。肺外主要合并肾错构瘤34例(55.74%)、腹膜后淋巴管平滑肌瘤18例(29.51%)、肝脏错构瘤11例(18.03%)、子宫肌瘤10例(16.39%)、盆腔囊肿9例(14.75%)。CT分级:0级0例、Ⅰ级24例、Ⅱ级18例、3级19例。HRCT平均评分:上肺区域(1.74±0.92)分、中肺区域(1.88±0.97)分、下肺区域(1.84±0.94)分,上、中、下肺区域平均分比较,差异无统计学意义(P>0.05)。不同级别患者肺纤维灶、小叶间隔增厚比较,差异有统计学意义(P<0.05)。结论:HRCT扫描可较好评估肺淋巴管平滑肌瘤病患者的肺部破坏情况,并进行CT分级。

Objective: To evaluate the feasibility of high-resolution CT( HRCT) in studying the lung structure in patients with pulmonary lymphatic leiomyoma( PLL).Methods: The clinical data of 61 PLL patients were reviewed. The HRCT imaging features,classification and structural destruction of the lungs with varying PLL severity in the patients were analyzed. Results: On HRCT,the lungs of PLL patients were featured mostly by multiple,dispersing cysts which were round or oval in shape with a mean diameter of( 1.22±0.76) cm,and thin,even walls measuring( 0. 86 ± 0. 37) mm by thickness. Concomitant pneumothorax was found in 27 patients( 44.26%),pleural effusion in 19( 31.15%),lung consolidation in 16( 26.23%),interlobular septal thickening in 16( 26.23%),fibrosis in 13( 21.31%),and local calcification in 9( 14.75%). Extrapulmonary manifestations included renal hamartoma found in 34 patients( 55. 74%),retroperitoneal lymph node leiomyoma in 18( 29.51%),hepatic hamartoma in 11( 18. 03%),uterine leiomyoma in 10( 16. 39%),and pelvic cysts in 9( 14.75%). CT classification: none patient with Grade 0,24 with Grade I,18 with Grade II,and 19 with Grade III. The mean HRCT score was( 1.74± 0.92) for the upper lung,( 1. 88 ± 0. 97) for the middle lung,and( 1. 84 ±0.94) for the lower lung,with no significant difference between the three regions( P > 0. 05). Pulmonary fibrosis and interlobular septal thickening did not differ significantly among patients with varying disease severity( P <0.05). Conclusion: HRCT scan may be useful for evaluating the lung damage in PLL patients with reliable CT classification.