ABSTRACT

If treatment of congenital venous malformations (VMs) is indicated by critical location or symptoms limiting the quality of life of the patient, endovascular, surgical, and medical treatment strategies are possible. Treatment of VMs depends on the type and extent of lesion, age of the patient, and feasibility of interventions. Endovascular embolo-sclerotherapy using undiluted ethanol (96%) or other sclerosants such as detergents (e.g., Ethibloc, Aethoxysclerol), microfoams (e.g., polidocanol; sodium tetradecyl sulfate [STS]), and chemotherapeutics such as bleomycin should be the first choice for management of symptomatic VM. Endovascular sclerotherapy is superior in terms of efficacy and safety, but large, randomized trials are missing. Primary surgical treatment should be reserved for small localized lesions, easily accessible locations, and special indications such as glomuvenous malformations, truncular lesions, and marginal vein excision. Surgery after embolo-sclerotherapy to improve functional and cosmetic outcomes should be discussed in an interdisciplinary team. In case of no interventional treatment options, such as embolo-sclerotherapy or surgical resection or failure to improve symptoms, a targeted medical therapy with sirolimus should be discussed, which will depend on the type/extent of the vascular malformation, age of the patient, and feasibility of interventions.