An Institution-wide Algorithm for Direct-stick Embolization of Congenital Peripheral Venous Malformations
Naiem Nassiri, MD RPVI1, Lauren A. Huntress, MS2, Mitchell Simon, MD2, Susan Murphy, MD2.
1Yale University, New Haven, CT, USA, 2Rutgers-RWJMS, New Brunswick, NJ, USA.
Objective: No standardized therapeutic algorithm or embolic agent of choice has yet been identified for management of congenital peripheral venous malformations (CPVMs). Treatment options and reported outcomes, therefore, vary widely. Herein, we present an institution-wide algorithm for management of symptomatic CPVMs using a single embolotherapeutic modality.
Methods: Over 36 months, patients with symptomatic CPVMs underwent contrast-enhanced magnetic resonance imaging (MRI). Hematologic monitoring for localized intravascular coagulopathy (LIC) was performed in all. Peri-operative anticoagulation was administered accordingly. When applicable, venous duplex ultrasound (DUS) was performed to assess for presence and patency of a deep venous system and superficial venous reflux. If the latter was identified, ablation was performed prior to or at the time of initial embolization. Direct-stick embolizations (DSE) were performed by a single operator using two concentrations (1% and 3%) of sodium tetradecyl sulfate (STS) without foam preparation. Patients were followed up clinically for resolution of symptoms, coagulopathic monitoring, and development of complications. All data was prospectively maintained and retrospectively reviewed.
Results: 71 direct-stick embolizations (DSE) were performed in 40 patients (1.8 procedures per patient, range 1-8; 12 male; mean age 22 [range 2-53 years]. Mean follow-up was 17.1 months (range 0.8 – 31.6). Presenting symptoms included pain (n=40, 100%), swelling (n=36, 90%), cosmetic disfigurement (n=32, 80%). Anatomic distribution was upper extremity (n=16, 23%), lower extremity (n=37, 52%), head/neck (n=7, 10%), trunk (n=10, 14%) and visceral (n=1, 1%). There were 33 sporadic cases, 4 Klippel-Trenaunay Syndrome (KTS) (10%), 2 Blue-Rubber Bleb Nevus Syndrome (BRBNS) (5%), and 1 CLOVES (2.5%). 4 patients presented with LIC, two of which required peri-operative enoxaparin. 26 patients (65%) required single DSE session with complete symptom relief. 14 patients (35%) required repeat DSE. 2 patients (5%) required adjunctive surgical excision. There was one post-operative mortality (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 1% STS (range 3-14 mL), and 15mL 3% STS (range 3-42.5 mL).
Conclusion: In absence of allergic reactions, the vast majority of CPVMs can be safely embolized with liquid STS, thereby avoiding well-documented toxicity of ethanol. Venous thromboembolism remains a major source of morbidity and mortality in this patient population despite close hematologic scrutiny. Prospective randomized trials are needed for embolotherapeutic standardization.
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