Fox U, Lucani G
General Surgery Department, Ospedale Santa Corona, Milan, Italy.
Lymphology. 1993 Jun;26(2):61-6.
Disorders of the intestinal lymphatic transport system are rare and typically associated with protein losing enteropathy (PLE). Hypoproteinemia caused by intestinal lymphangiectasia is often associated with lymphedema of the legs and occasionally with chyluria, chylometrorrhea and chylous ascites. This article examines the varied presentations of lymphangiectasia syndromes including its pathophysiology. Diagnosis is based on signs and symptoms, specific laboratory findings, and confirmed by contrast small bowel series, lymphography and best of all laparoscopy. We describe 12 patients with PLE secondary to primary intestinal lymphangiectasia (1980-1991). Treatment was non-operative (dietary) in 8 patients and surgical in 4 including segmental resections of the jejunum in two, lymphatic-mesenteric venous anastomosis in one, and peritoneal-venous (LeVeen) shunt in one with overall satisfactory results.
肠道淋巴转运系统疾病较为罕见，通常与蛋白丢失性肠病（PLE）相关。由肠道淋巴管扩张引起的低蛋白血症常伴有腿部淋巴水肿，偶尔还伴有乳糜尿、乳糜性子宫出血和乳糜性腹水。本文探讨了淋巴管扩张综合征的各种表现及其病理生理学。诊断基于体征和症状、特定的实验室检查结果，并通过小肠造影、淋巴管造影，最好是腹腔镜检查来确诊。我们描述了12例继发于原发性肠道淋巴管扩张的PLE患者（1980 - 1991年）。8例患者采用非手术（饮食）治疗，4例采用手术治疗，其中2例行空肠节段切除术，1例行淋巴管 - 肠系膜静脉吻合术，1例行腹膜 - 静脉（LeVeen）分流术，总体效果满意。
