An institution-wide algorithm for direct-stick embolization of peripheral venous malformations.

Abstract

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 36months, 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, 22years [range, 2-53years]). Mean follow-up was 17.1months (range, 0.8-31.6months). 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 7mL of 1% STS (range, 3-14mL), and 15mL of 3% STS (range, 3-42.5mL). 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.