Division of Vascular and Endovascular Surgery, Department of Surgery, Yale University School of Medicine and Yale New Haven Hospital, New Haven, Conn.
Rutgers Robert Wood Johnson Medical School, New Brunswick, NJ.
J Vasc Surg Venous Lymphat Disord. 2018 May;6(3):351-357. doi: 10.1016/j.jvsv.2017.12.011. Epub 2018 Feb 1.
No standardized therapeutic algorithm or embolic agent of choice has yet been identified for management of congenital peripheral venous malformations (VMs). Treatment options and reported outcomes therefore vary widely. Herein, we present an institution-wide algorithm for management of symptomatic congenital peripheral VMs using a single embolotherapeutic modality.
During 36 months, patients with symptomatic congenital peripheral VMs underwent contrast-enhanced magnetic resonance imaging. Hematologic monitoring for localized intravascular coagulopathy was performed in all. Perioperative anticoagulation was administered accordingly. When applicable, venous duplex ultrasound was performed to assess for presence and patency of a deep venous system and superficial venous reflux. If superficial venous reflux was identified, radiofrequency ablation was performed per standard protocol before or at the time of initial embolization. Direct-stick embolizations (DSEs) were performed by a single operator using two concentrations (1% and 3%) of sodium tetradecyl sulfate (STS; Sotradecol; AngioDynamics, Latham, NY) without foam preparation. Patients were followed up clinically for resolution of symptoms, coagulopathic monitoring, and development of complications. All data were prospectively maintained and retrospectively reviewed.
There were 71 DSEs performed in 40 patients (1.8 procedures per patient [range, 1-8]; 12 male patients; mean age, 22 years [range, 2-53 years]). Mean follow-up was 17.1 months (range, 0.8-31.6 months). Presenting symptoms included pain (n = 40 [100%]), swelling (n = 36 [90%]), and cosmetic disfigurement (n = 32 [80%]). Anatomic distribution was upper extremity (n = 16 [23%]), lower extremity (n = 37 [52%]), head and neck (n = 7 [10%]), trunk (n = 10 [14%]), and visceral (n = 1 [1%]). There were 33 sporadic cases, 4 (10%) Klippel-Trénaunay syndrome cases, 2 (5%) blue rubber bleb nevus syndrome cases, and 1 (2.5%) CLOVES (congenital lipomatous overgrowth, vascular malformations, epidermal nevus, and skeletal deformities) syndrome case. Four patients presented with localized intravascular coagulopathy, two of whom required perioperative enoxaparin. Twenty-six patients (65%) required a single DSE session with complete symptom relief. Fourteen patients (35%) required repeated DSE. Two patients (5%) required adjunctive surgical excision. There was one postoperative death (1.4%) secondary to massive pulmonary embolism. Complications were otherwise limited to skin necrosis (n = 2 [3%]). Mean volume of sclerosant per session was 7 mL of 1% STS (range, 3-14 mL), and 15 mL of 3% STS (range, 3-42.5 mL).
In the absence of allergic reactions, most congenital peripheral VMs can be safely embolized with liquid STS, thereby avoiding the well-documented toxicity of ethanol. Venous thromboembolism remains a major source of morbidity and mortality in this population of patients despite close hematologic scrutiny. Prospective randomized trials are needed for embolotherapeutic standardization.
对于先天性周围性静脉畸形（VMs）的治疗，目前尚无标准化的治疗算法或首选的栓塞剂。因此，治疗选择和报告的结果差异很大。在此，我们提出了一种使用单一栓塞治疗方法治疗有症状的先天性周围性 VMs 的机构内算法。
在 36 个月期间，有症状的先天性周围性 VMs 患者接受了对比增强磁共振成像。所有患者均进行局部血管内凝血的血液学监测。相应地给予围手术期抗凝治疗。如果适用，进行静脉双功超声检查以评估深静脉系统和浅静脉反流的存在和通畅性。如果发现浅静脉反流，则按照标准方案在初次栓塞前或同时进行射频消融。由一名操作人员使用两种浓度（1%和 3%）的十四烷基硫酸钠（Sotradecol；AngioDynamics，Latham，NY）进行直接粘贴栓塞（DSE），无需泡沫准备。患者在临床随访中观察症状缓解、凝血监测和并发症发生情况。所有数据均进行前瞻性维护和回顾性审查。
在 40 名患者中进行了 71 次 DSE（每位患者 1.8 次[范围，1-8]；12 名男性患者；平均年龄 22 岁[范围，2-53 岁]）。平均随访时间为 17.1 个月（范围，0.8-31.6 个月）。主要症状包括疼痛（n=40[100%]）、肿胀（n=36[90%]）和美容畸形（n=32[80%]）。解剖分布为上肢（n=16[23%]）、下肢（n=37[52%]）、头颈部（n=7[10%]）、躯干（n=10[14%]）和内脏（n=1[1%]）。有 33 例散发性病例、4 例（10%）Klippel-Trénaunay 综合征病例、2 例（5%）蓝橡皮疱痣综合征病例和 1 例（2.5%）CLOVES（先天性脂肪过度生长、血管畸形、表皮痣和骨骼畸形）综合征病例。有 4 名患者出现局部血管内凝血，其中 2 名需要围手术期依诺肝素治疗。26 名患者（65%）只需单次 DSE 即可完全缓解症状。14 名患者（35%）需要重复 DSE。2 名患者（5%）需要辅助手术切除。有 1 例患者（1.4%）术后因大面积肺栓塞死亡。其他并发症仅限于皮肤坏死（n=2[3%]）。每次治疗的硬化剂平均体积为 1% STS 7mL（范围，3-14mL），3% STS 15mL（范围，3-42.5mL）。
在没有过敏反应的情况下，大多数先天性周围性 VMs 可以安全地用液体 STS 栓塞，从而避免乙醇的已知毒性。尽管进行了密切的血液学检查，但静脉血栓栓塞仍然是此类患者发病率和死亡率的主要原因。需要前瞻性随机试验来确定栓塞治疗的标准化。
