Lande A, Bedford A, Schechter L S
Angiology. 1976 Apr;27(4):223-40. doi: 10.1177/000331977602700404.
In Osler-Weber-Rendu syndrome, visceral organs are frequently involved in addition to cutaneous and mucous membranous lesions. Detailed arteriographic investigation of 5 patients with this abnormality disclosed characteristic angiodysplastic lesions involving the lungs, liver, spleen, pancreas, gastrointestinal tract and mesentery. Arterial and aortic aneurysms appear to be an integral part of this abnormality. Common to angiodysplastic lesions is a variable degree of arteriovenous shunting. The arteriographic characteristics and the hemodynamic significance of major visceral arteriovenous shunts in hereditary hemorrhagic telangiectasia are discussed.
在遗传性出血性毛细血管扩张症(Osler-Weber-Rendu综合征)中,除皮肤和黏膜病变外,内脏器官也常受累。对5例患有这种异常情况的患者进行的详细动脉造影检查发现,特征性的血管发育异常病变累及肺、肝、脾、胰腺、胃肠道和肠系膜。动脉和主动脉瘤似乎是这种异常情况的一个组成部分。血管发育异常病变的共同特点是存在不同程度的动静脉分流。本文讨论了遗传性出血性毛细血管扩张症中主要内脏动静脉分流的动脉造影特征及其血流动力学意义。