肺淋巴管肌瘤病-高分辨CT表现

Pulmonary lymphangioleiomyomatosis:high-resolution CT findings

目的分析肺淋巴管肌瘤病（PLAM）的高分辨率CT（HRCT）表现，探讨HRCT对该病的诊断价值。方法回顾性分析7例经病理证实的PLAM患者的胸片、胸部常规CT和HRCT表现，所有影像均有2位以上放射科医生共同分析，意见一致。结果胸片诊断PLAM的价值有限，PLAM的CT特征表现为两肺弥漫均匀分布的直径2～10mm含气小囊，HRCT对显示气囊优于常规CT，是诊断本病的必要手段。1例伴有自发性气胸，2例伴乳糜胸，l例合并肺大疱形成。结论PLAM具有特征性的HRCT表现，有经验的放射科医生根据典型HRCT表现可诊断此病，但确诊需病理活检。

Objective To describe the high-resolution CT （HRCT） findings in patients with pulmonary lymphangioleio myomatosis （PLAM） and to investigate its value in diagnosis of this disease. Methods Seven patients with pathologically confirmed PLAM underwent chest X-ray radiography, conventional CT and HRCT examinations. The images were re viewed retrospectively by more than two radiologists and a consensus opinion was reached. Results Chest X-ray radio graphy could not diagnose PLAM. Although the presence of cysts may be suggested at conventional CT, HRCT was supe rior for cyst detection and was essential for diagnosis. Numerous, thin-walled cysts, ranged 2～ 10 mm, distributed dif fusely throughout the lungs, were typical HRCT findings for diagnosis of PLAM. Other findings included spontaneous pneumothorax in 1 case, chylothorax in 2 cases, and bulla in 1 case. Conclusion PLAM has relative typical HRCT find ings. The diagnosis of PLAM can be made on HRCT scan with reasonable certainty by expert radiologists, but biopsy is necessary to make the final diagnosis.