Hashimoto Manabu, Tate Etuko, Nishii Toshiaki, Watarai Jiro, Shioya Takanobu, White Robert I
Department of Radiology, Akita University School of Medicine, 1-1-1 Hondo, Akita City, Akita 010-8543, Japan.
Cardiovasc Intervent Radiol. 2003 Mar-Apr;26(2):177-80. doi: 10.1007/s00270-002-1507-y. Epub 2003 Mar 6.
To describe the angiographic features of hepatic involvement in hereditary hemorrhagic telangiectasia (HHT), particularly the presence of portovenous shunts.
We reviewed the angiographic findings of seven patients with HHT. The patients comprised three women and four men with a mean age of 51 years.
Intrahepatic telangiectasias were found in all seven patients and shunts between three vascular channels were found in six of seven patients. In the four patients who had portovenous shunts combined with arterioportal shunts, the portovenous shunts were large. Three patients had no portovenous shunts. Two of these patients had arteriovenous shunts, and one had no shunt. The mean age (69 years) of the patients with portovenous shunts was older than those without portovenous shunts (26 years).
Hepatic vascular lesions in HHT are varied, ranging from telangiectasias to large shunts between three vascular channels. In an advanced stage of involvement, large portovenous shunts are present.
描述遗传性出血性毛细血管扩张症（HHT）肝脏受累的血管造影特征，尤其是门静脉分流的存在情况。
我们回顾了7例HHT患者的血管造影结果。患者包括3名女性和4名男性，平均年龄51岁。
所有7例患者均发现肝内毛细血管扩张，7例中有6例发现三个血管通道之间存在分流。在4例门静脉分流合并动门脉分流的患者中，门静脉分流较大。3例患者无门静脉分流。其中2例有动静脉分流，1例无分流。有门静脉分流的患者平均年龄（69岁）高于无门静脉分流的患者（26岁）。
HHT的肝脏血管病变多种多样，从毛细血管扩张到三个血管通道之间的大分流。在疾病进展期，会出现大的门静脉分流。
