肺淋巴管肌瘤病8例影像学分析

The Imaging Study of Pulmonary Lymph Angioleio Myomatosis in Eight Cases

目的：探讨肺淋巴管肌瘤病（PLAM）的影像表现。方法：回顾分析本院8例经病理证实的PLAM的肺部及腹部影像表现，包括X线胸片、CT和腹部B超。结果：8例成年女性患者胸部高分辨率CT（high resolution CT，HRCT）的典型表现为：肺内多发小囊腔影，约5～20mm大小，薄壁（〈2mm），其中5例两肺广泛分布，2例肺内多发，1例散在分布。气胸6例，乳糜胸4例，其中2例气胸并积液；腹部CT和B超发现：腹膜后多发淋巴结增大2例，其中1例合并卵巢畸胎瘤和子宫肌瘤。1例合并卵巢囊肿和大量腹水；子宫肌瘤3例，其中1例合并右肾囊肿；肝内胆管多发结石1例；腹部未见异常2例。结论：HRCT表现为肺内多发小囊腔影是PLAM的特征性表现，PLAM多并发有腹部多脏器病变。

Objective： To evaluate the imaging features of pulmonary lymphangioleiomyomatosis （PLAM）. Methods： The pulmonary and abdominal imaging data of 8 pathologically proven cases with PLAM, including X-ray film, CT scans and abdominal ultrasound imaging, were retrospectively analyzed. Results： Among these 8 female adults, the data of high resolution CT （HRCT） was typically featured by multiple small pulmonary cysts, about 5 to 20 millimeters in diameter and with thin walls （less than 2 millimeters）. In 5 of the 8 cases, pulmonary cysts distributed throughout the lungs; 2 cases showed multiple and one showed localized lesions. Among them, 6 cases were complicated with pneumothorax, 4 with chylothorax, 2 cases with pneumothorax and pleural effusion. Abdominal CT scan and US found 2 cases with multiple retroperitoneal lymph node enlargement, and one of which was complicated with ovarian teratoma and uterine leiomyoma. One case had ovarian cyst and massive ascites. Three cases were found with uterine leiomyoma, and one of them had cyst in right kidney. One case had multiple stones in the intrahepatic bile ducts. Two cases showed negative abdominal examination. Conclusion： The multiple small pulmonary cysts were the typical features of PLAM in HRCT. PLAM may often be complicated with multi-organ diseases in the abdomen.