Blanchon F, Grivaux M, Muller C, Chantelot J M, Beroud P, Attia M
Service de Pneumologie, Centre Hospitalier Général de Meaux.
Rev Mal Respir. 1989;6(1):75-8.
The authors report a case of lymphangiomatosis (LAM) proven histologically in a 49 year old female. This case benefitted from very detailed CT-scanning (IDM) with characteristic images obtained from the thorax and bilateral renal tumours suggestive of angiomyolipomas. This association seems to be quasi-pathognomic of the disease. The discovery at CT-scanning of cerebral calcification raises a possible connection of LAM with Bourneville's tuberous sclerosis. This CT-scanning study has several points of interest: the early diagnosis of pulmonary lesions which are under evaluated by standard radiography; a complete inventory of the possible localisations of the disease; it enables a possible diagnosis in very frail patients in whom a lung biopsy would be a risk; an ability to assess further progress as there also exists a good correlation between the extent of the disease assessed by TDM and the disturbance in pulmonary function.
作者报告了一例经组织学证实的49岁女性淋巴管肌瘤病（LAM）病例。该病例受益于非常详细的CT扫描（IDM），获得了胸部的特征性图像以及提示血管平滑肌脂肪瘤的双侧肾肿瘤。这种关联似乎是该疾病的准特异性表现。CT扫描发现脑钙化提示LAM与结节性硬化症可能存在关联。这项CT扫描研究有几个有趣之处：能早期诊断标准X线检查评估不足的肺部病变；全面了解该疾病可能的发病部位；对于身体非常虚弱、肺活检有风险的患者能够进行可能的诊断；由于通过TDM评估的疾病范围与肺功能障碍之间也存在良好的相关性，所以能够评估疾病的进一步进展情况。
