Sherrier R H, Chiles C, Roggli V
Department of Radiology, Duke University Medical Center, Durham, NC 27710.
AJR Am J Roentgenol. 1989 Nov;153(5):937-40. doi: 10.2214/ajr.153.5.937.
Lymphangioleiomyomatosis, a rare disease occurring in women of childbearing age, is characterized by proliferation of smooth muscle in pulmonary lymphatic channels and mediastinal and abdominal lymph nodes. Chest radiographs typically reveal interstitial disease with normal lung volume. Pneumothorax and pleural effusions may be present. CT scans in eight patients with biopsy-proved pulmonary lymphangioleiomyomatosis were reviewed. The prominent feature of the disease was multiple thin-walled cysts throughout the lungs, best visualized on scans made with 1.5-mm collimation. Mediastinal and/or retrocrural lymphadenopathy, often not appreciated on the chest radiograph, was present in four of eight patients. CT can suggest a diagnosis of lymphangioleiomyomatosis when diagnosis by clinical presentation and chest radiographs is uncertain.
淋巴管平滑肌瘤病是一种发生于育龄女性的罕见疾病，其特征为肺淋巴管以及纵隔和腹部淋巴结内平滑肌增生。胸部X线片通常显示肺间质病变且肺容积正常。可能出现气胸和胸腔积液。回顾性分析了8例经活检证实为肺淋巴管平滑肌瘤病患者的CT扫描结果。该病的显著特征是全肺多发薄壁囊肿，在层厚1.5毫米的扫描图像上显示最佳。8例患者中有4例存在纵隔和/或膈脚后淋巴结肿大，胸部X线片常未显示。当根据临床表现和胸部X线片难以确诊时，CT可提示淋巴管平滑肌瘤病的诊断。
