KTS and Marginal Vein Insufficiency: Treatment Options Good afternoon. I must first apologize for running in at the last minute, and also for having to leave immediately following this presentation. I am chairing a symposium in another room, so please forgive me. I would like to describe treatment of a young male patient with a congenital vascular malformation often referred to as Klippel-Trenaunay Syndrome (CLICK) 3:40-3:50 Nick Morrison, MD President, International Union of Phlebology Slide Rock, Sedona, AZ
Disclosures Medtronic Research Grant Scientific Advisory Board Speakers Bureau MediUSA Educational Grant Merz Consultant Morrison Vein/Training Institute Medical Director This is my obligatory disclosure slide Northern Arizona, USA
Kleppel-Trenaunay Syndrome Description Vascular malformations: Including venous, lymphatic, capillary (Not arterial – Parkes-Weber Syndrome) Boney/Muscular Hypertrophy Atypical Lateral (Marginal) Varicosity Point out the bilateral capillary vascular malformations, the longer right leg with boney hypertrophy, and the varicosities
Patient Presentation Twenty-year old white male Diffuse port-wine staining primarily of the right leg, foot, hemithorax, abdomen Slight lengthening of the right leg with osseous hypertrophy and macrodactyly Extensive varicosities of the superficial venous system in the right thigh, infragenicular area and the foot Symptoms of pain, heaviness, and fatigue of increasing severity Our patient is a 20 year-old Caucasian male who presented to our center in 2005 with the following findings: (CLICK) diffuse bilateral capillary malformation or port-wine staining showing more involvement of the right side; (CLICK) a longer right leg with osseous hypertrophy and macrodactyly; (CLICK) large varicosities of the right leg; (CLICK) and symptoms including worsening primarily right leg pain, heaviness, and fatigue
Kleppel-Trenaunay Syndrome Point out the bilateral capillary vascular malformations, the longer right leg with boney hypertrophy, and the varicosities
These are still shots taken on presentation in 2005 of the patient’s symptomatic right leg and foot
And some closer views, both of the right leg
Diagnostic Studies Duplex-ultrasonography Mild femoral vein hypoplasia Moderate reflux in the deep system from the CFV to the PTVs with moderate ectasia of the proximal popliteal and distal femoral vein and a dilated, dominant profunda vein Gross reflux of the GSV from the SFJ distally (including an incompetent anterior accessory GSV and SSV A thorough diagnostic workup of this patient was undertaken, especially to examine the full extent of his vascular malformation (CLICK) with first a duplex scan showing gross reflux along the lengths of the right great and small saphenous veins, (CLICK) with moderate reflux in the deep system involving the common femoral, femoral, popliteal, and deep calf veins. Also noted was moderate ectasia of the distal femoral and proximal right popliteal veins, with a dilated and dominant profunda vein. No abnormalities of the left leg deep or superficial venous system were identified.
Confirmed duplex valve presence This slides demonstrates a valve in the right popliteal vein, ruling out avalvulosis
Confirmed duplex valve presence And a valve, albeit incompetent, in the right common femoral vein
CFV Reflux This slide demonstrates the spectral analysis of right common femoral vein reflux
Diagnostic Studies Arterial doppler study normal with a normal ABI MRA and MRV of the right leg and pelvis normal with no evidence of A-V fistula or AVM Ascending and Descending Venogram no evidence of AVM Lymphoscintigraphy normal lymphatic system Thrombophilia profile Hyperhomocysteinemia – 3x normal The arterial doppler study demonstrated a normal ankle-brachial index bilaterally
Treatment Endovenous laser ablation of proximal GSV and SSV Ambulatory phlebectomy of varicosity of the medial aspect and dorsum of the foot Foam sclerotherapy of accessory saphenous, distal GSV, SSV, and infragenicular varicosities *(always “covered” with LMWH because of thrombophilia) The patient was then treated for his superficial venous component, with prophylactic doses of LMWH, utilizing endovenous thermal ablation for the proximal portions of the right great and small saphenous veins, (CLICK) along with ambulatory phlebectomy of the varicosities of the medial aspect of the thigh and lower leg, and the medial and dorsal aspects of the foot. (CLICK) Foam sclerotherapy was then used in subsequent visits to ablate the distal great and small saphenous veins, the anterior accessory saphenous vein, and the smaller varicosities of the lower leg.
9-year follow up 2007 Endovenous laser ablation of the right great saphenous veins and ambulatory phlebectomy below the right knee. 2009 Endovenous laser right marginal vein, left GSV 2012 Right BK AP re-do 2014 Right GSV re-do laser ablation Right SSV, lateral vein re-do laser ablation (11 months later) 2007-Present Multiple UGS sessions with Foam
9-year follow up 2016 “He is doing well but is tired of all of the procedures and the expense is a strong consideration for him. On PE he has recurrent varices over the lateral patella and proximal calf, as well as the feet. On duplex exam, the right deep system is patent along its length, mildly hypoplastic, and incompetent at the CFV and PV. The right SSV is closed, and the right GSV is closed proximally but wide open from the distal thigh to the ankle, with the exception of a 5cm sclerotic segment in the proximal calf. The left GSV is also partially patent along its length and the incompetent tributaries are noted from the knee inferiorly.”
9-year follow up April, 2016 Right GSV Adhesive ablation (donated by Medtronic) right ankle to proximal calf – above too sclerotic to cannulate Right proximal GSV, large tributaries of foot, ankle, and peri- genicular UGS foam sclerotherapy
Kleppel-Trenaunay Syndrome Other treatment options RF ablation – likely same outcome as laser ablation Surgical excision of large lateral vein – BLEEDING!! Compression therapy alone – inadequate symptom relief Point out the bilateral capillary vascular malformations, the longer right leg with boney hypertrophy, and the varicosities
Thank you for your kind attention nickmorrison2002@yahoo.com