December 22, 2008

Clinical Outcome and Quality of Life after surgical treatment for Klippel-Trénaunay Syndrome: A case-control study

Rafael D. Malgor, MD¹, Peter Gloviczki, MD¹, Manju Kalra, MBBS¹, Gustavo S. Oderich, MD¹, Joseph J. Ricotta, MD¹, Stephen S. Cha, MS², David Driscoll, MD³, Terri Vrtiska, MD⁴, Audra A. Duncan, MD¹.
¹Division of Vascular and Endovascular Surgery, Mayo Clinic, Rochester, MN, Rochester, MN, USA, ²Division of Biostatistics, Mayo Clinic, Rochester, MN, Rochester, MN, USA, ³Division of Vascular Malformation Clinic, Gonda Vascular, Mayo Clinic, Rochester, MN, Rochester, MN, USA, ⁴Department of Radiology, Mayo Clinic, Rochester, MN, Rochester, MN, USA.

OBJECTIVES:Klippel-Trenaunay (KT) syndrome is a malformation characterized by varicose veins, port-wine stains and limb hypertrophy. The purpose of this study was to compare clinical outcome and impact on quality of life (QoL) after varicose vein surgery in patients with and without KT syndrome.
METHODS: Clinical data of 28 patients with KT syndrome (Group 1) were compared to those of 28 patients with primary varicosity (Group 2). The groups were paired on gender and severity of disease ( CEAP classification). Group 1 included 57% of 49 KT patients, operated on between 1987 and 2007 for KT syndrome, Group 2 included 2.5% of the 1118 patients, operated on for primary varicosity. QoL was assessed using the Aberdeen questionnaire
RESULTS: Mean age was lower in Group 1 than in 2 (35.2 vs. 45.1 year, p=.008), mean CEAP scores were similar (3.41±1.07 vs. 3.39±0.96). Initial Venous Comorbidity Severity Score (VCSS) was higher in Group 1 than in 2. (9.30 ± 3.66, vs. 6.35± 4.57, p=.012), pain was present in all Group 1 patients and in 75% of Group 2 (p=0.005). All patients underwent stripping of the great and/or small saphenous vein or other superficial veins and phlebectomy. Two KT patient had vena cava filter placed preoperatively. One KT patient had deep vein thrombosis and 4 developed thrombophlebitis. Follow-up was 104±76 months in Group 1, 107±63 months in Group 2. Reoperation for varicose veins was performed in 25% in Group 1 vs 21% in Group 2 (p= NS). VCSS at last follow-up was better than before surgery in both groups; improvement was similar (p=.004) but those without KT had less pain (p=.0006). QoL scores improved in both groups, more so in Group 2 (8.74±2.64 versus 6.88± 2.49, p=.0009).
CONCLUSIONS:Patients with KT syndrome have more symptoms both at presentation and after surgery than those with primary varicosity. Superficial venous surgery is beneficial in both groups but quality of life improves more after surgery for primary varicosity than for KT syndrome.

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