一名53岁男性在就诊前出现腹部症状和发烧6个月，腹水和下半身水肿2个月。怀疑有心力衰竭、肾衰竭、下腔静脉或门静脉阻塞、肝硬化和恶性肿瘤，但均未出现。我们还根据 α-1 抗胰蛋白酶清除率升高（224 mL/天）怀疑蛋白质渗漏性胃肠病。根据双气囊内窥镜检查结果，我们诊断该患者患有原发性肠道淋巴管扩张症。由于患者的腹水没有得到令人满意的药物控制，因此在丹佛进行了腹腔静脉分流术。结果，腹水量在较长时间内得到了成功控制。

A 53-year-old man presented with abdominal symptoms and a fever for 6 months and ascites and lower body edema for 2 months before visiting our clinic. Heart failure, renal failure, inferior vena cava or portal vein obstruction, cirrhosis, and malignancy were suspected, but none were present. We also suspected protein-leakage gastroenteropathy based on the elevated alpha-1 antitrypsin clearance (224 mL/day). Based on the double-balloon endoscopy findings, we diagnosed the patient with primary intestinal lymphangiectasia. Since the patient's ascites were not satisfactorily controlled medically, a Denver peritoneovenous shunt was placed. As a result, the ascites volume was successfully controlled over an extended period.