Compression Therapy and Endovascular Treatment of Klippel-Trenaunay-Weber Syndrome

Abstract

Introduction - Klippel-Trenaunay-Weber syndrome (KTWS) is a rare congenital vascular disorder characterized by the classic triad of cutaneous nevi, venous varicosities, and osseous and soft tissue hypertrophy of the affected limb Methods - From August 2015 to April 2017 our hospital made a diagnosis and gave treatment and follow-up of 12 cases of KTW. All patients were treated with compression therapy. Digital subtraction angiography (DSA) in 12 patients were visible enlargement of limb artery, arterial branch in disorder. With micro catheter super choice into target vessels with polyvinyl alcohol particles (PVA particles) and spring coil embolization, treated 3 to 5 times on average. Arteriovenous fistula was treated with covered stent by transcatheter endovascular interventional techniques. Results - All patients were followed up for 1 ∼ 3 years, an average of 1.5 years. 12 cases of conservative treatment leg symptoms including pain, cramping, limb swelling, and varicose veins decreased with no serious complications. Varicose vein disappeared, pain relieved, without limb necrosis. One case of amputation. Conclusion - Compression therapy and surgical treatment significantly improve patients' quality of life. Compression therapy is suitable for all of the patients. Implementation of reasonable surgical treatment after diagnosis can greatly improve patients'quality of life.