College of Medicine, SUNY Upstate, Syracuse, New York 13210, USA.
Neurosurgery. 2010 May;66(5):E1027-8; discussion E1028. doi: 10.1227/01.NEU.0000368392.69904.BE.
We present a comprehensive review of intracranial aneurysms in Klippel-Trenaunay and Klippel-Trenaunay-Weber syndromes (KTS/KTWS), and examine factors influencing the risks of surgery vs conservative management.
A 58-year-old physician with KTS affecting the right extremities presented with left hemispheric cerebellar stroke and was discovered to harbor four intracranial aneurysms of the posterior circulation: fusiform mid and distal BA (2.6 x 2 x 2 cm), fusiform right proximal P1 (2 x 1.3 x 1.3 cm), fusiform right distal P1 (2.8 x 2.7 x 2 cm), and saccular left distal posterior inferior cerebellar artery (2.5 x 2.5 x 2.5 cm). Ten years later he had an infarct in the paramedian distribution of the basilar artery and a right internal capsule stroke. Two months later, he developed hydrocephalus, ultimately presenting in status epilepticus 4 months later secondary to ongoing aneurysm expansion and mass effect.
Systemic anticoagulation for acute thrombosis with possible distal arterioarterial embolization from giant P1 aneurysms. Ventriculoperitoneal shunting for hydrocephalus. The patient died within 9 days after admission and 10 years after the initial discovery of aneurysms.
Strict control of modifiable risk factors compromising vascular integrity and periodic neuroimaging are warranted in KTS/KTWS patients. KTS/KTWS patients are hypercoagulable, and may be predisposed to aneurysm thrombosis with increased risk for distal arterial microembolization. Stroke-related morbidity secondary to distal arterioarterial aneurysm thrombus embolization and acute aneurysm thrombosis may be decreased with systemic anticoagulation in this patient population. KTS/KTWS patients have significantly higher rates of DVT and PE than the general population, and should be classified in the high-risk category for venous thromboembolism prophylaxis. Both endovascular and open cerebrovascular techniques have been used successfully in KTS/KTWS patients with intracranial aneurysms.
我们对 Klippel-Trenaunay 和 Klippel-Trenaunay-Weber 综合征（KTS/KTWS）中的颅内动脉瘤进行了全面回顾，并研究了影响手术与保守治疗风险的因素。
一位 58 岁的医生患有影响右侧肢体的 KTS，表现为左侧小脑半球卒中，并发现存在四个后循环颅内动脉瘤：梭形中远端 BA（2.6 x 2 x 2 cm）、梭形右侧近端 P1（2 x 1.3 x 1.3 cm）、梭形右侧远端 P1（2.8 x 2.7 x 2 cm）和囊状左侧远端小脑后下动脉（2.5 x 2.5 x 2.5 cm）。十年后，他出现基底动脉旁正中分布梗死和右侧内囊卒中。两个月后，他出现脑积水，最终由于持续的动脉瘤扩张和占位效应，四个月后出现癫痫持续状态。
急性血栓形成给予全身抗凝治疗，可能存在来自巨大 P1 动脉瘤的远端动脉-动脉栓塞。脑积水行脑室-腹腔分流术。患者入院后 9 天内死亡，距首次发现动脉瘤 10 年后死亡。
KTS/KTWS 患者需要严格控制影响血管完整性的可改变危险因素，并定期进行神经影像学检查。KTS/KTWS 患者存在高凝状态，可能由于动脉瘤血栓形成而增加远端动脉微栓塞的风险。对于此类患者人群，全身性抗凝治疗可能会降低与远端动脉-动脉瘤血栓栓塞和急性动脉瘤血栓形成相关的卒中发病率。KTS/KTWS 患者的深静脉血栓形成和肺栓塞发生率明显高于普通人群，应将其归类为静脉血栓栓塞症预防的高危人群。KTS/KTWS 患者的颅内动脉瘤可成功采用血管内和开放性脑血管技术治疗。
