Ziyeh S, Spreer J, Rössler J, Strecker R, Hochmuth A, Schumacher M, Klisch J
Section of Neuroradiology, Neurocenter, University of Freiburg, Breisacher Strasse 64, 79106 Freiburg, Germany.
Eur Radiol. 2004 Nov;14(11):2025-9. doi: 10.1007/s00330-004-2274-8. Epub 2004 Mar 6.
Klippel-Trenaunay and Parkes Weber (Klippel-Trenaunay-Weber) syndromes consist of vascular malformations of the capillary, venous and lymphatic systems combined with soft tissue and bone hypertrophy of the affected extremity. Klippel-Trenaunay syndrome is a pure low-flow condition, while Parkes Weber syndrome is characterized by significant arteriovenous fistulas. The distinction of both entities is relevant, since the prognosis and therapeutic strategies differ significantly. Our purpose is to demonstrate that thick-slice dynamic magnetic resonance projection angiography (MRPA) is a non-invasive tool to detect arteriovenous shunting in Parkes Weber syndrome. Four patients underwent MR imaging and MRPA. MRPA demonstrated arteriovenous shunting in three patients. Arteriovenous shunting was characterized by early appearing draining veins. The time of arrival between normal arteries and pathological veins varied between less than 0.5 and 1.0 s. Therefore, the diagnosis in these cases could be specified as Parkes Weber syndrome. In all these cases, arteriovenous shunting was confirmed by intraarterial digital subtraction angiography. One patient showed normal results in MRPA and could be diagnosed as having Klippel-Trenaunay syndrome.
克-特综合征和帕克斯-韦伯综合征(克-特-韦伯综合征)由毛细血管、静脉和淋巴系统的血管畸形合并患肢软组织和骨肥大组成。克-特综合征是一种单纯的低流量疾病,而帕克斯-韦伯综合征的特征是存在显著的动静脉瘘。区分这两种疾病很重要,因为它们的预后和治疗策略有显著差异。我们的目的是证明厚层动态磁共振投影血管造影(MRPA)是一种检测帕克斯-韦伯综合征动静脉分流的非侵入性工具。4例患者接受了磁共振成像和MRPA检查。MRPA在3例患者中显示出动静脉分流。动静脉分流的特征是引流静脉早期出现。正常动脉与病变静脉之间的到达时间在不到0.5秒至1.0秒之间变化。因此,这些病例可诊断为帕克斯-韦伯综合征。在所有这些病例中,动脉内数字减影血管造影证实了动静脉分流。1例患者MRPA结果正常,可诊断为克-特综合征。