Klippel—Trenaunay Syndrome: Knowledge Revision and Current Treatment Options

Klippel—Trenaunay syndrome (KTS) is a congenital disorder caused by somatic genetic mutations with formation of capillary, venous, and lymphatic malformations. KTS is characterized by limb enlargement, its deformation, varicose veins, and port-wine stains on the skin. Persistent embryonic veins and incompetent saphenous veins cause venous reflux and subsequent venous hypertension, leading to severe chronic venous insufficiency symptoms in KTS patients. Deep vein anomalies in KTS accompany superficial vein anomalies in only a relatively small number of patients, making it possible to consider various methods of removing the latter to alleviate venous hypertension. In the majority of patients, endovascular techniques for thermal and non-thermal superficial vein ablation and perforator vein embolization enable a sustained reduction in the severity of disease symptoms without a high risk of postoperative complications. This literature review aims to revise the current concepts of KTS and analyze available publications on the surgical management of this pathology in adults.