Surgical treatment of venous malformations in Klippel-Trenaunay syndrome

Purpose: Klippel-Trenaunay syndrome (KTS) is a complex congenital anomaly c haracterized by varicosities and venous malformations (VMs) of one or more limbs, port-wine stains, and soft tissue and bone hypertrophy. Venous drain age is frequently abnormal because of embryonic veins, a,agenesis, hypoplas ia, valvular incompetence, or aneurysms of deep veins. We previously report ed on the surgical management of KTS. In this article, we update our experi ence. Methods: Twenty patients with KTS underwent surgical treatment for VMs betw een July 1, 1987, and January 1, 2000. This group represented 6.9% of 290 p atients with I(TS who were seen at our institution during this 12.5-year st udy period. Surgical indications, venous anatomy (determined with duplex sc an, contrast phlebography, magnetic resonance imaging or magnetic resonance phlebography), operative procedures, and complications were reviewed, and outcomes mere recorded. Results: Twelve male and eight female patients (mean age, 23.4 years; range , 7.7-40.6 years) underwent 30 vascular surgical procedures in 21 lower lim bs. All 20 patients (100%) had varicose veins or VMs, 13 (65%) had port-wil e stains, and 18 (90%) had limb hypertrophy. Pain was the most common compl aint, which was present in 16 patients (80%), followed by swelling in 15 (7 5%), bleeding in 8 (40%), and superficial thrombophlebitis and cellulitis i n 3 (15%). Imaging confirmed patent deep veins in 18 patients, hypoplastic femoral vein in 1, and entrapped popliteal veins bilaterally in 1. Four pat ients (20%) had large persistent sciatic veins (PSVs). The CEAP clinical cl assification was C-3 for 17 patients (85%), C-4 for 1 patient (5%), and C-6 for 2 patients (10%). Stripping of large lateral veins, avulsion, and exci sion of varicosities or VMs were performed on all limbs. Three patients req uired staged resections. The release of entrapped popliteal veins was perfo rmed in both limbs of one patient; another underwent a popliteal-saphenous bypass graft. One patient underwent excision of a PSV. Open and endoscopic perforator vein ligation was performed in one patient each. Two patients (1 2%) had hematomas that required evacuation. No patients had caval filter pl acement; none had postoperative deep venous thrombosis or pulmonary embolus . The mean follow-up was 63.6 months (range, 0-138 months). All patients re ported initial improvement, but some varicosities recurred in 10 patients ( 50%), an ulcer did not heal in one, and a new ulcer developed in one, 8 yea rs after surgery. Three patients underwent reoperation for recurrent varico sities. Follow-up CEAP scores were C-2 in 10 patients (50%), C-3 in 6 patie nts (30%), C-4 and C-5 in 1 patient each (5%), and C-6 in 2 patients (10%). Clinical scores improved from 4.3 +/- 2.2 to 3.1 +/- 2.3. (P = .03). Conclusions: The management of patients with KTS continues to be primarily nonoperative, but those patients with patent deep veins can be considered f or excision of symptomatic varicose veins and VMs. Although the recurrence rate is high, clinical improvement is significant, and reoperations can be performed if needed. Occasionally, deer vein reconstruction, excision of PS Vs, or subfascial endoscopic perforator surgery is indicated. Because I(TS is rare, patients should receive multidisciplinary care in qualified vascul ar centers.