Besson A, Saegesser F
Schweiz Med Wochenschr. 1977 Jul 30;107(30):1057-64.
Massive gastrointestinal bleeding is known to occur in patients presenting the hemorrhagic hereditary telangiectasis condition known as Rendu-Osler-Weber (ROW) disease. Bleeding is most frequent in the fourth decade. Endoscopic coagulation of upper gastrointestinal telangiectatic spots is nowadays possible even in the presence of active bleeding. In the lower digestive tract the naevi are basicallly located on the terminal ileum and the right colon; emergency coloscopic examination is difficult when active bleeding occurs. 10-15% of ROW patients present sudden life-threatening episodes of bleeding. The danger is aggravated through delayed diagnosis as well as difficulty in localizing the hemorrhagic area. Selective angiogram of the three digestive arteries is a safe and efficient method of diagnosing the nature and source of the bleeding. It should be obtained routinely, on an emergency basis, before surgery. Operation includes removal of the involved part of the digestive tract. A specimen angiogram should be obtained during the procedure, to make sure that the bleeding malformation has been removed. Laparotomy without preliminary angiogram is likely to be unsuccessful. An illustrative case is presented.
已知患有遗传性出血性毛细血管扩张症（即Rendu - Osler - Weber病，简称ROW病）的患者会发生大量胃肠道出血。出血在四十多岁时最为常见。如今，即使存在活动性出血，对上消化道毛细血管扩张斑点进行内镜下凝血也是可行的。在下消化道，痣基本上位于回肠末端和右结肠；当发生活动性出血时，急诊结肠镜检查很困难。10% - 15%的ROW病患者会出现危及生命的突发性出血事件。由于诊断延迟以及难以确定出血区域，危险会加剧。对三条消化动脉进行选择性血管造影是诊断出血性质和来源的一种安全有效的方法。应在紧急情况下常规在手术前进行。手术包括切除消化道受累部分。在手术过程中应获取标本血管造影，以确保已切除出血性畸形。没有初步血管造影就进行剖腹手术很可能不成功。本文给出了一个说明性病例。
