Summary

Background: Klippel-Trenaunay syndrome, a congenital disorder, is characterized by capillary malformation, varicosities and bony or soft tissue hypertrophy. Since there is no cure for this syndrome, treatment is directed towards secondary prevention of venous hypertension and preservation of functional integrity of the legs. Elastic stockings are the mainstay of treatment and are indicated in all cases. Surgery is reserved only for a few selected symptomatic patients, however the outcome is unsatisfactory in most cases, with recurrent pain, edema, poor cosmetic result and limb deformity. Ultrasound-guided foam sclerotherapy is a recently introduced minimally invasive ambulatory procedure for the treatment of chronic venous insufficiency. It was recently introduced to treat this disorder.

Objectives: To evaluate the efficacy of USFS[1] in the treatment of patients with Klippel-Trenaunay syndrome.

Methods: Seven patients diagnosed with Klippel-Trenaunay, with massive lower extremity involvement, were treated with USFS between October 2003 and October 2005. Sclerovein® (polidocanol, Resinag, Switzerland) 2–4% was used as the sclerosant. The signs, symptoms and overall patient satisfaction were assessed before, during and after the treatment.

Results: Patients' mean age was 26 years (range 15–54). The CEAP[2] clinical classification, with ascending severity ranging from 0 (no signs) to 6 (active venous ulcer), was C4 in 5 patients (71.5%) and C5 and C6 in one patient each. The average number of sessions was 14.5 (range 9–21). No major complications were encountered. All seven patients reported improvement in signs and symptoms. Five of the 7 patients (71%) were very satisfied with the cosmetic result.

Conclusion: USFS is an effective minimally invasive ambulatory technique, essentially pain-free and with excellent short-term results in patients with Klippel-Trenaunay syndrome (when the deep system is functional). Long-term results and larger study groups are warranted.