肺淋巴管肌瘤病HRCT诊断价值

Pulmonary lymphangioleiomyomatosis:high-resolution CT findings

目的 :回顾性分析肺淋巴管肌瘤病的HRCT特征。方法 :总结 5例经病理证实并行HRCT检查的肺淋巴管肌瘤病 ,2例行支气管镜肺活检 ,2例经胸腔镜 ,1例经皮肺活检确诊。 4例行吸气末HRCT扫描 ,其中 1例表现阴性者加扫呼气末HRCT。分析X线胸片、常规CT以及HRCT表现特征。结果 :X线胸片 3例呈弥漫性网状阴影 ,1例可见蜂窝状囊腔 ,1例表现正常 ;HRCT4例为均匀散在性分布的 10～ 2 0mm大小的薄壁囊腔 ,其中 2例囊腔之间可见正常肺组织 ,1例吸气末HRCT正常 ,而呼气末HRCT可见到“空气潴留征” ,提示有小气道狭窄。肺功能检查均有不同程度的阻塞型通气障碍和弥散功能异常。结论 :尽管本病的最后确诊要依靠肺活检病理证实 ,但其HRCT具有较特征性的表现 ,呼气末HRCT可发现早期小气道狭窄引起的空气潴留 ,从而指导临床有目的进行纤维支气管镜活检 ,有助于早期诊断。

Objective:To evaluate retrospectively the HRCT findings of pulmonary lymphangioleiomyomatosis(LAM).Methods:HRCT examinations and radiographs were obtained in 5 patients with LAM confirmed by transbronchial lung biopsy (n=2),thoracoscopy biopsy (n=2) and open lung biopsy (n=1).The patients were 5 women,ranging in age form 36 to 43 years.The clinical,histopathologic records and CT images were reviewed.Results:The radiographs showed diffuse reticular shadows in 3 patients,honeycomb appearance in 1 and normal appearance in 1.HRCT scans revealed an increase in the interstitial pattern,architectural distortion and cystic lesions in 4 patients.The size of the cysts varied form 10mm to 20mm and normal tissue amogn the pulmonaly cysts was found in 2.One patiets appeared air-trapping on expiratory high-resolution CT but show normal on inspiratory HRCT.No pneumothorax and pleural effusion were found in all patients.Conclusion:Thought the definite diagnosis was made by pathology,LAM has some characteristics features on HRCT.It could not exclude LAM thought HRCT appearing nomal.End expiratory HRCT could show early air-trapping caused be stenosis of small air passages.Whith could guaid the biopsy of fiberbronchoscope and whith will be helpful to make early diagnosis.