Frasier Krista, Giangola Gary, Rosen Robert, Ginat Daniel T
Lenox Hill Hospital, Department of Vascular Surgery, New York City, NY 10021, USA.
J Vasc Surg. 2008 Jun;47(6):1339-45. doi: 10.1016/j.jvs.2008.01.040.
Klippel-Trenaunay Syndrome (KTS) is an uncommon congenital disorder of uncertain etiology that comprises the clinical triad of varicose veins, port wine stain, and bony or soft-tissue hypertrophy. The literature suggests that the deep venous system is often under-developed. We propose that duplex venous ultrasound can effectively demonstrate patent deep venous systems in KTS patients with mild to moderate disease, and that endovascular radiofrequency ablation can be utilized in a safe and appropriate therapeutic manner.
A single center retrospective review of three patients with KTS treated with endovascular radiofrequency ablation of the KT veins and/or great saphenous veins was conducted. Preoperatively, patients underwent both venography and were studied with color flow duplex ultrasound system iU22 with a 7-5 MHz linear array probe (Philips Medical Systems, NA, Bothell, Wash). The anomalous KT veins, great saphenous and saphenous tributaries, and associated incompetent perforators were ablated with radiofrequency catheters (VNUS Medical Technologies, Inc, San Jose, Calif). All the radiofrequency ablations were complimented by ultrasound guided sclerotherapy of the varicose tributaries and when evident, incompetent perforator veins.
The diagnostic series of duplex ultrasounds performed on our KTS patients has demonstrated contiguous deep venous systems in the effected extremity and effectively recognized the associated anomalous superficial venous systems. Our treatment resulted in successful occlusion of the incompetent veins in all three patients.
The three patients, females aged 39, 19, and 16, presented with port wine stains and many years of leg-swelling and varicose veins that were recalcitrant to conservative treatment measures, including compression stockings and pulsed-dye laser therapy. Venography initially revealed poorly developed deep venous systems. However, venous ultrasound demonstrated patent and competent deep venous systems in all of the affected limbs. Radiofrequency ablations were performed to manage the sequella of venous insufficiency. At short-term follow-up, all patients demonstrated markedly decreased leg pain, edema, and varicose vein bulging.
Three KTS patients were successfully treated with radiofrequency ablation of the incompetent great saphenous and/or anomalous superficial veins. Although the deep veins were poorly visualized on venography, they were clearly demonstrated with duplex ultrasound and functioned adequately once the incompetent superficial veins were ablated.
克-特综合征（KTS）是一种病因不明的罕见先天性疾病，其临床三联征包括静脉曲张、葡萄酒色斑和骨或软组织肥大。文献表明，深静脉系统常发育不全。我们提出，双功能静脉超声能够有效地显示病情轻至中度的KTS患者的深静脉系统通畅情况，并且血管内射频消融可安全、适当地用于治疗。
对3例接受KT静脉和/或大隐静脉血管内射频消融治疗的KTS患者进行单中心回顾性研究。术前，患者接受了静脉造影，并使用配备7-5MHz线性阵列探头的iU22彩色血流双功能超声系统（飞利浦医疗系统公司，美国华盛顿州博塞尔）进行检查。使用射频导管（VNUS医疗技术公司，美国加利福尼亚州圣何塞）对异常的KT静脉、大隐静脉及其属支以及相关的功能不全穿通支进行消融。所有射频消融均辅以超声引导下对曲张属支以及明显存在的功能不全穿通静脉进行硬化治疗。
对我们的KTS患者进行的一系列双功能超声诊断显示，受累肢体的深静脉系统连续，并有效识别了相关的异常浅静脉系统。我们的治疗使所有3例患者的功能不全静脉成功闭塞。
这3例患者为女性，年龄分别为39岁、19岁和16岁，表现为葡萄酒色斑以及多年的腿部肿胀和静脉曲张，对包括弹力袜和脉冲染料激光治疗在内的保守治疗措施无效。静脉造影最初显示深静脉系统发育不良。然而，静脉超声显示所有受累肢体的深静脉系统通畅且功能正常。进行射频消融以处理静脉功能不全的后遗症。短期随访时，所有患者的腿痛、水肿和静脉曲张均明显减轻。
3例KTS患者通过对功能不全的大隐静脉和/或异常浅静脉进行射频消融获得成功治疗。尽管静脉造影时深静脉显示不清，但双功能超声清晰显示了深静脉，并且在功能不全的浅静脉消融后深静脉功能良好。
