Chen Yee-Shiuan, Memon Pauras
Division of Medical Education, Department of Medicine, Barnes-Jewish Hospital, Saint Louis, Missouri, USA.
BMJ Case Rep. 2019 Jul 11;12(7):e229958. doi: 10.1136/bcr-2019-229958.
This is a rare case of sporadic lymphangioleiomyomatosis (S-LAM) manifesting as refractory chylothorax and chyloperitoneum. A middle-aged woman with unremarkable medical history presented with respiratory failure, abdominal distension and anasarca. She was found to have high-output chylous effusion that required chest tube drainage, as well as chylous ascites. Notably initial chest and abdominal CT did not reveal characteristic pulmonary cysts or the presence of angiomyolipomas suggestive of LAM. An extensive oncologic and infectious work-up was undertaken with negative findings. The chylous effusion was persistent and refractory to thoracic duct embolization, total parenteral nutrition with octreotide, and talc pleurodesis. Diagnosis of S-LAM was confirmed after repeat chest CT showed subtle pulmonary cystic changes, and serum vascular endothelial growth factor-D level was found to be elevated at 834 pg/mL. Patient was started on sirolimus therapy, but lost to follow-up after hospital discharge. Patient died approximately 1 year later.
这是一例罕见的散发性淋巴管平滑肌瘤病（S-LAM），表现为难治性乳糜胸和乳糜腹。一名既往病史无特殊的中年女性出现呼吸衰竭、腹胀和全身水肿。她被发现有高输出量的乳糜性积液，需要胸腔闭式引流，同时伴有乳糜腹水。值得注意的是，最初的胸部和腹部CT未显示特征性肺囊肿或提示LAM的血管平滑肌脂肪瘤。进行了广泛的肿瘤学和感染性检查，结果均为阴性。乳糜性积液持续存在，对胸导管栓塞、使用奥曲肽的全胃肠外营养及滑石粉胸膜固定术均无反应。重复胸部CT显示细微的肺囊性改变，且血清血管内皮生长因子-D水平升高至834 pg/mL后，S-LAM的诊断得以证实。患者开始接受西罗莫司治疗，但出院后失访。患者约1年后死亡。
