VS3. The Management of Klippel-Trénaunay Syndrome in the Modern Era

Abstract

Klippel-Trénaunay syndrome (KTS) is a capillary-lymphaticovenous malformation. It typically affects the lower extremity and consists of a port wine stain, varicose veins/venous malformations and limb hypertrophy/orthopedic issues. This is a retrospective review of our approach to patients with KTS who had undergone treatment at our tertiary center. Patients were managed as part of a multidisciplinary team. Preoperative studies, endovascular interventions, and operative imaging are summarized in video format. The KTS management algorithm is shown in the Fig. Patients are referred to our center, and a history and physical are performed. Ultrasonography and magnetic resonance angiography are performed to identify the area of vascular malformation. In the absence of significant symptoms, patients are seen for periodic follow-up. If intervention is required, a venogram is initially performed to further evaluate the deep venous system. The vena marginalis lateralis (‘KT vein’) is embolized if present, incompetent, and the deep system is intact. If there is a coexistent cavernous venous malformation, direct puncture embolization of the malformation is then performed, typically with sodium tetradecyl sulfate. Patients are seen routinely, and if symptoms persist, repeat embolizations or sclerotherapy are performed. Alternatively, patients are referred for assessment by other members of our multidisciplinary team. A multidisciplinary, stepwise approach is critical to the successful management of the patient with KTS. Multiple treatments are often required.