Jacob Samuel, Ali Mojahid, El-Sayed Ahmed Magdy M, Itkin Maxim, Narula Tathagat, Pham Si, Erasmus David
Department of Cardiothoracic Surgery, Mayo Clinic, Jacksonville, FL, USA.
Center for Lymphatic Disorders, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA, USA.
SAGE Open Med Case Rep. 2020 May 6;8:2050313X20921332. doi: 10.1177/2050313X20921332. eCollection 2020.
Lymphangioleiomyomatosis is a rare systemic disorder of unknown etiology that affects young women almost exclusively. Chylous effusions are known to be associated with lymphangioleiomyomatosis and may be difficult to treat. We present the case of a 37-year-old female who received bilateral lung transplantation for lymphangioleiomyomatosis complicated by refractory chylothorax and chylous ascites, ultimately controlled through repeated, open surgical procedures and percutaneous lymphatic embolization interventions. The combined surgical and interventional radiological approach, while not novel in their own right, suggests that a multi-modal interventional approach may be required in refractory cases.
淋巴管平滑肌瘤病是一种病因不明的罕见系统性疾病,几乎仅累及年轻女性。乳糜性积液已知与淋巴管平滑肌瘤病相关,且可能难以治疗。我们报告一例37岁女性患者,她因淋巴管平滑肌瘤病合并难治性乳糜胸和乳糜性腹水接受了双侧肺移植,最终通过反复的开放手术和经皮淋巴管栓塞干预得以控制。手术和介入放射学联合方法本身虽并非新颖,但提示在难治性病例中可能需要多模式介入方法。