Klippel-Trenaunay Syndrome (KTS) is an uncommon congenital disorder of uncertain etiology that comprises the clinical triad of varicose veins, port wine stain, and bony or soft-tissue hypertrophy. The literature suggests that the deep venous system is often under-developed. We propose that duplex venous ultrasound can effectively demonstrate patent deep venous systems in KTS patients with mild to moderate disease, and that endovascular radiofrequency ablation can be utilized in a safe and appropriate therapeutic manner.Methods:
A single center retrospective review of three patients with KTS treated with endovascular radiofrequency ablation of the KT veins and/or great saphenous veins was conducted. Preoperatively, patients underwent both venography and were studied with color flow duplex ultrasound system iU22 with a 7-5 MHz linear array probe (Philips Medical Systems, NA, Bothell, Wash). The anomalous KT veins, great saphenous and saphenous tributaries, and associated incompetent perforators were ablated with radiofrequency catheters (VNUS Medical Technologies, Inc, San Jose, Calif). All the radiofrequency ablations were complimented by ultrasound guided sclerotherapy of the varicose tributaries and when evident, incompetent perforator veins.Results:
The diagnostic series of duplex ultrasounds performed on our KTS patients has demonstrated contiguous deep venous systems in the effected extremity and effectively recognized the associated anomalous superficial venous systems.Results:
Our treatment resulted in successful occlusion of the incompetent veins in all three patients.Discussion:
The three patients, females aged 39, 19, and 16, presented with port wine stains and many years of leg-swelling and varicose veins that were recalcitrant to conservative treatment measures, including compression stockings and pulsed-dye laser therapy. Venography initially revealed poorly developed deep venous systems. However, venous ultrasound demonstrated patent and competent deep venous systems in all of the affected limbs. Radiofrequency ablations were performed to manage the sequella of venous insufficiency. At short-term follow-up, all patients demonstrated markedly decreased leg pain, edema, and varicose vein bulging.Conclusions:
Three KTS patients were successfully treated with radiofrequency ablation of the incompetent great saphenous and/or anomalous superficial veins. Although the deep veins were poorly visualized on venography, they were clearly demonstrated with duplex ultrasound and functioned adequately once the incompetent superficial veins were ablated.