Abstract：
Intestinal lymphangiectasia (IL) is a protein-losing enteropathy (PLE) that occasionally leads to gastrointestinal bleeding (GIB). We encountered a 41-year-old female with a 9-year history of duodenal IL with PLE and GIB that progressively worsened. Despite a diet, supplemented with medium-chain triglycerides, antiplasmin therapy, oral corticosteroids, octreotides, sirolimus, and repeated endoscopic hemostasis, her symptoms remained uncontrolled, leading to blood transfusion dependence. Lymphangiography revealed significant leakage from abnormal abdominal lymph vessels into the duodenal lumen. The patient subsequently underwent an abdominal-level lymphaticovenous anastomosis combined with local venous ligation. This approach resulted in a dramatic improvement and sustained resolution of both the PLE and GIB. More than 6 months after surgery, the patient remained free of symptoms and blood transfusion dependence.
摘要：
肠淋巴管扩张症（IL）是一种蛋白质丢失性肠病（PLE），偶尔会导致胃肠道出血（GIB）。我们遇到了一名41岁的女性，有9年的十二指肠IL病史，PLE和GIB逐渐恶化。尽管有节食,补充中链甘油三酯，抗纤溶酶治疗,口服皮质类固醇,奥曲肽,西罗莫司,反复内镜下止血,她的症状仍然不受控制，导致输血依赖。淋巴管造影显示，异常的腹部淋巴管明显泄漏到十二指肠腔中。患者随后接受了腹部水平的淋巴静脉吻合术和局部静脉结扎。该方法导致PLE和GIB两者的显著改进和持续的分辨率。手术后6个月以上，患者仍然没有症状和输血依赖。
