Bantel H, Gregor M, Lamberts R
Abteilung Gastroenterologie, Hepatologie und Infektiologie, Universität Tübingen.
Dtsch Med Wochenschr. 1999 Nov 5;124(44):1301-6. doi: 10.1055/s-2007-1024535.
A 32-year-old man had ten years previously undergone several laparotomies for recurrent ileus of the small intestine. They revealed severe intestinal oedema and histology showed hyperplasia of the lymphatic system but the aetiology was unclear. After a 10-year interval free of symptoms he presented with marked hypoproteinaemic oedema and exudative enteropathy the cause of which was to be clarified by exploratory laparotomy with excision of lymph nodes and a small section of small intestine.
Histology revealed intestinal lymphangiectasis with partly hyperplastic lymphoid tissue. Lymphangiography demonstrated several lymph nodes in the region of the aortic bifurcation and renal vessels with a central filling defect. It is thought likely that obstruction to lymphatic flow in this region resulted in oedema of the intestinal wall which caused the recurrent episodes of ileus.
Retrospectively it is assumed that primary intestinal lymphangiectasis was responsible for the initial manifestation of an acute abdomen. For treatment of hypoproteiaemia with human albuminea prot system was implanted. The course was complicated by recurrent inflammation and thrombosis of the port catheter. Because of immune deficiency risk of carcinoma is high in primary intestinal lymphangiectasis.
Primary intestinal lymphangiectasis, even though a rare condition, should be considered in the differential diagnosis of otherwise unclear acute abdomen.
一名32岁男性10年前因小肠反复肠梗阻接受过多次剖腹手术。术中发现严重的肠道水肿，组织学检查显示淋巴系统增生，但病因不明。在无症状的10年间隔期后，他出现了明显的低蛋白血症性水肿和渗出性肠病，其病因将通过剖腹探查切除淋巴结和一小段小肠来明确。
组织学检查显示肠道淋巴管扩张，伴有部分增生的淋巴组织。淋巴管造影显示主动脉分叉和肾血管区域有几个淋巴结，中央有充盈缺损。据认为，该区域淋巴流动受阻导致肠壁水肿，进而引起反复肠梗阻发作。
回顾性推测原发性肠道淋巴管扩张是此次急腹症初始表现的原因。为治疗低蛋白血症，植入了人血白蛋白输注系统。病程中出现了反复的炎症和端口导管血栓形成。由于免疫缺陷，原发性肠道淋巴管扩张患者患癌风险很高。
原发性肠道淋巴管扩张虽然是一种罕见疾病，但在鉴别诊断不明原因的急腹症时应予以考虑。
