Takagi Yumiko, Sato Teruhiko, Morio Yoshiteru, Kumasaka Toshio, Mitani Keiko, Miyamoto Hideaki, Iwakami Shin-Ichiro, Kodama Yuzo, Onuma Emi, Seyama Kuniaki, Takahashi Kazuhisa
Department of Respiratory Medicine, Juntendo University School of Medicine, Tokyo.
Intern Med. 2010;49(5):439-45. doi: 10.2169/internalmedicine.49.2714. Epub 2010 Mar 1.
A 30-year-old Japanese woman with lymphangioleiomyomatosis (LAM) developed a left chylothorax and chylous ascites. A pleuro-peritoneal communication was confirmed by a scintigram with (99)mTc-labeled macroaggregated-albumin injected into the peritoneal cavity. Video-assisted thoracic surgery revealed a protruding papillary lesion on the left diaphragm. Chyle was oozing into the pleural cavity through this lesion. Histopathological analyses demonstrated that the protrusion was a diaphragmatic LAM lesion and that LAM-associated lymphangiogenesis enabled communication between the pleural and peritoneal cavities through lymphatic vessels. This case demonstrated a new mechanism for chylous pleural effusion in LAM and illustrates the significance of LAM-associated lymphangiogenesis.
一名30岁患有淋巴管平滑肌瘤病（LAM）的日本女性出现了左侧乳糜胸和乳糜性腹水。通过向腹腔注射（99）mTc标记的大颗粒白蛋白闪烁扫描证实了胸膜-腹膜交通。电视辅助胸腔镜手术显示左侧膈肌有一个突出的乳头状病变。乳糜通过该病变渗入胸腔。组织病理学分析表明，该突出物是膈肌LAM病变，并且LAM相关的淋巴管生成使得胸膜腔和腹膜腔通过淋巴管相通。该病例展示了LAM中乳糜性胸腔积液的一种新机制，并说明了LAM相关淋巴管生成的重要性。
