Foresti V, Casati O, Zubani R, Villa A
3rd Medical Department, Fatebenefratelli-Oftalmico Hospital, Milan, Italy.
Respiration. 1990;57(6):398-401. doi: 10.1159/000195879.
We report on a male patient with tuberous sclerosis (adenoma sebaceum and digital fibromas, renal angiomyolipomas and subependymal brain calcifications), who presented with chylothorax. Chest CT scan did not show pulmonary parenchymal alterations, but only a moderate enlargement of the paracaval mediastinal lymph nodes. Tuberous sclerosis with lung involvement presents clinical, radiologic and pathologic manifestations similar to those of lymphangioleiomyomatosis. The pulmonary manifestations of tuberous sclerosis and lymphangioleiomyomatosis have been observed almost exclusively in women, and it has been suggested that they represent opposite ends of a spectrum of presentations of the same entity. Based on these considerations the formation of a chylothorax in a male with tuberous sclerosis constitutes an extremely rare finding.
我们报告了一名患有结节性硬化症（皮脂腺瘤和指纤维瘤、肾血管平滑肌脂肪瘤和室管膜下脑钙化）的男性患者，该患者出现了乳糜胸。胸部CT扫描未显示肺实质改变，仅腔静脉旁纵隔淋巴结中度肿大。结节性硬化症累及肺部时，其临床、放射学和病理学表现与淋巴管平滑肌瘤病相似。结节性硬化症和淋巴管平滑肌瘤病的肺部表现几乎仅在女性中观察到，有人认为它们代表同一实体的一系列表现的相反两端。基于这些考虑，患有结节性硬化症的男性出现乳糜胸是极为罕见的发现。
