Surgical treatment of venous malformations in Klippel-Trénaunay syndrome

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1 Surgical treatment of venous malformations in Klippel-Trénaunay syndrome Audra A. Noel, MD, a Peter Gloviczki, MD, a Kenneth J. Cherry, Jr, MD, a Thom W. Rooke, MD, b Anthony W. Stanson, MD, c and David J. Driscoll, MD, d Rochester, Minn Purpose: Klippel-Trénaunay syndrome (KTS) is a complex congenital anomaly, characterized by varicosities and venous malformations (VMs) of one or more limbs, port-wine stains, and soft tissue and bone hypertrophy. Venous drainage is frequently abnormal because of embryonic veins, agenesis, hypoplasia, valvular incompetence, or aneurysms of deep veins. We previously reported on the surgical management of KTS. In this article, we update our experience. Methods: Twenty patients with KTS underwent surgical treatment for VMs between July 1, 1987, and January 1, This group represented 6.9% of 290 patients with KTS who were seen at our institution during this 12.5-year study period. Surgical indications, venous anatomy (determined with duplex scan, contrast phlebography, magnetic resonance imaging or magnetic resonance phlebography), operative procedures, and complications were reviewed, and outcomes were recorded. Results: Twelve male and eight female patients (mean age, 23.4 years; range, years) underwent 30 vascular surgical procedures in 21 lower limbs. All 20 patients (100%) had varicose veins or VMs, 13 (65%) had port-wine stains, and 18 (90%) had limb hypertrophy. Pain was the most common complaint, which was present in 16 patients (80%), followed by swelling in 15 (75%), bleeding in 8 (40%), and superficial thrombophlebitis and cellulitis in 3 (15%). Imaging confirmed patent deep veins in 18 patients, hypoplastic femoral vein in 1, and entrapped popliteal veins bilaterally in 1. Four patients (20%) had large persistent sciatic veins (PSVs). The CEAP clinical classification 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 excision of varicosities or VMs were performed on all limbs. Three patients required staged resections. The release of entrapped popliteal veins was performed 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 (12%) had hematomas that required evacuation. No patients had caval filter placement; none had postoperative deep venous thrombosis or pulmonary embolus. The mean follow-up was 63.6 months (range, months). All patients reported 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 years after surgery. Three patients underwent reoperation for recurrent varicosities. Follow-up CEAP scores were C-2 in 10 patients (50%), C-3 in 6 patients (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 for 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, deep vein reconstruction, excision of PSVs, or subfascial endoscopic perforator surgery is indicated. Because KTS is rare, patients should receive multidisciplinary care in qualified vascular centers. (J Vasc Surg 2000;32:840-7.) From the Division of Vascular Surgery a and the Departments of Vascular Medicine, b Vascular and Interventional Radiology, c and Pediatric and Adolescent Medicine, d Mayo Clinic and Mayo Foundation. Competition of interest: nil. Reprint requests: Peter Gloviczki, MD, Mayo Clinic, 200 First Street SW, Rochester MN ( gloviczki.peter@mayo.edu). Copyright 2000 by The Society for Vascular Surgery and The American Association for Vascular Surgery, a Chapter of the International Society for Cardiovascular Surgery /2000/$ /6/ doi: /mva

2 Volume 32, Number 5 Noel et al 841 Klippel-Trénaunay syndrome (KTS) is a complex congenital malformation that may affect the lower or upper extremities or, less commonly, involve the trunk, head, or neck. The three main components of KTS are varicosities and venous malformations (VMs), capillary malformations (port-wine stains), and hypertrophy of the soft tissue and bone. Frequently, venous drainage is abnormal because of persistent embryonic veins, agenesis, hypoplasia, valvular incompetence, or aneurysms of deep veins. The management of chronic venous insufficiency in patients with KTS requires precise preoperative documentation of the venous anatomy and function. In 1991, we reported on our initial experience with surgical treatment for chronic venous insufficiency in patients with KTS. 1 In this article, we update our experience. The data of six previously reported patients have been included in this report. METHODS Between July 1, 1987, and January 1, 2000, 290 patients with KTS were evaluated at the Mayo Clinic. Of this group, 20 patients (6.9%) underwent surgical treatment for symptomatic varicosities and vascular malformations or for swelling and pain associated with chronic venous insufficiency. Each patient s clinical history and results of a physical examination, including the presence of soft tissue and bony hypertrophy, capillary malformations (port-wine stains), and VMs, were recorded. Venous anatomy was documented with contrast phlebography, magnetic resonance imaging (MRI), magnetic resonance phlebography (MRP), and duplex scans. Twenty phlebograms were available; all were rereviewed by two authors (A. W. S. and A. N.). Surgical procedures and complications were recorded. Follow-up evaluation was obtained through a clinic visit or a patient questionnaire. The CEAP and clinical severity scale, based on pain, edema, venous claudication, skin changes, and ulceration, as recommended by the Consensus document of the Joint Vascular Societies, 2 was recorded. A paired 2-tailed t test was used to compare preoperative and postoperative clinical scores, with significance defined as a P value less than.05. RESULTS Clinical characteristics. Twenty patients, 12 male and eight female (mean age, 23.4 years; range, years) underwent 30 vascular surgical procedures in 21 limbs. All 20 patients (100%) had varicose veins, 11 (55%) had large lateral embryonic veins (Fig 1, A), 13 (65%) had port-wine stains (Fig A B Fig 1. A, 19-year-old man with a large incompetent lateral embryonic vein extending from the ankle to saphenofemoral junction. B, Same patient is allowed to stand for 5 minutes, and the veins are marked with ink pen before vein stripping and avulsion of varicosities. 2), and 18 (90%) had limb hypertrophy. One woman had two pigmented lesions over her affected knee without the typical capillary malformation characteristics. Eleven patients (55%) had all three sequelae of KTS. Clinical findings were noted at birth in 16 patients (80%), with a mean age 6.8 months (range, birth to 6 years). No patients had a family history of KTS. Three patients had concomi-

3 842 Noel et al November 2000 Table I. Clinical presentation of VMs in 20 patients with KTS Symptom No. of patients (%) Pain 16 (80) Edema 15 (75) Bleeding 8 (40) Superficial thrombophlebitis 3 (15) Cellulitis 3 (15) Table II. Preoperative contrast phlebography findings in 20 patients with KTS Anatomic feature No. of patients (%) Fig 2. Port-wine stain (capillary malformation) on affected extremity of patient with KTS. Note large lateral embryonic vein of the thigh (arrow). Large lateral embryonic vein 20 (100) Medial varicosities 13 (65) Band-like narrowing of popliteal vein 9 (45) Incompetent perforating veins 6 (30) Hypoplastic SFV 5 (25) Popliteal vein aneurysm 4 (20) SFV ectasia 4 (20) PSV 4 (20) Entrapped popliteal veins 1 (5) Fig 3. Ascending phlebogram in 36-year-old woman demonstrating a PSV (arrow) and a markedly enlarged greater saphenous vein (double arrows). tant malformations including mitral valve prolapse, spinal stenosis, and pectus carinatum. Pain was the most common complaint at the time of presentation to our institution, occurring in 16 patients (80%). One lower extremity was affected in 16 patients (80%), both lower extremities in 2 (10%), both lower extremities and one upper extremity in 1 (5%), and a crossed upper and lower extremity in 1 (5%). No patients were asymptomatic. Other presenting symptoms are summarized in Table I. CEAP classification scores were C-3 in 17 patients (85%), C-4 in 1 (5%), and C-6 in 2 (10%). The mean clinical severity scale before surgical treatment was 4.3 ± 2.2. Diagnostic imaging. All patients underwent contrast phlebography, 19 (95%) underwent MRI examinations, and 13 (65%) underwent venous duplex scan. Table II summarizes the contrast phlebography findings (Figs 3 and 4). All patients had evidence of lateral embryonic veins. An abnormal contour of the popliteal or superficial femoral vein (SFV) was noted in 19 patients (95%), with the most common deep venous abnormality of band-like narrowing of the popliteal vein demonstrated in nine patients (45%). Four patients (20%) had persistent sciatic veins (PSVs), based on contrast phlebogram and MRI. MRI and MRP confirmed extension of VMs into the subfascial plane or muscle involvement in 12 (63%) of 19 patients (Fig 5). Four patients (21%) had pelvic VMs and one (5%) had labial varicosities. Venous duplex scan documented deep venous incompetence in nine (70%) of 13 patients. Although, avalvulia accounts for venous incompe-

4 Volume 32, Number 5 Noel et al 843 tence in a subgroup of patients with KTS, we could not determine the type of valvular abnormalities in the nine patients with venous incompetence. Surgical treatment and morbidity. Preoperative vein marking with an ink pen was performed in all patients (Fig 1, B). Twenty-nine procedures (97%) were performed with patients under general anesthesia, whereas one procedure (3%) was performed with the patient under epidural anesthesia. Avulsion and excision of varicosities or VMs were performed on all limbs, after confirmation of a patent deep system, with concomitant stripping of the incompetent saphenous vein in three patients (15%) or the lateral embryonic vein in eight patients (40%). The lesser saphenous vein was stripped in three patients (15%). Tourniquet was used to limit blood loss in 13 (43%) of 30 procedures. The greater saphenous vein was stripped from the saphenofemoral junction to the ankle with a flexible stripper. Varicosities and VMs were excised or avulsed through stab incisions or small incisions, with care taken during avulsion of lateral varicosities to stay in a superficial plane therefore avoiding the peroneal nerve. If possible, a stripper was used to remove large lateral embryonic veins. The lateral embryonic vein was stripped while the tourniquet was inflated in six patients (30%). Because of the large size of this vein, multiple skin incisions were made along the vein, and the vein was tied to the stripper with 2-0 silk. The release of entrapped bilateral popliteal veins was performed in one patient, before resection of large superficial veins in both limbs. One patient, reported previously, had excision of a large PSV and excision of symptomatic labial varicosities. One patient, also included in our previous report, underwent deep vein reconstruction with the saphenous vein. 1 In this patient, the saphenous vein from the contralateral unaffected limb was interposed from the popliteal vein to the proximal segment of the greater saphenous vein. The patient with the poplitealto-saphenous vein reconstruction had a patent graft with ultrasound scan examination 102 months postoperatively, although small venous ulcers recurred, probably because of deep and incompetent perforating veins. Incompetent perforators were ligated in one patient with an open technique early in the series and in one patient with subfascial endoscopic perforator surgery (SEPS). Blood was transfused to one patient (2 units) postoperatively after popliteal vein release, despite tourniquet control. A second patient received blood (1 unit) during resection of a VM without an occluding tourniquet. Two patients (10%) received perioperative subcutaneous heparin injections for deep venous thrombosis (DVT) prophylaxis, Fig 4. Ascending phlebogram in 19-year-old man confirms large lateral embryonic vein with multiple varicosities (arrow) and a phlebectasia (double arrows) of the popliteal vein with band-like narrowing. and one patient (5%) who had a deep vein reconstruction was given heparin intravenously and converted to permanent warfarin therapy. All patients had postoperative compression of the affected extremity with an elastic wrap and were given 30 to 40 mm Hg stockings to wear starting 10 to 14 days after surgery. The intervention was complicated by a hematoma that required drainage in two patients (12%). No patients had DVT, pulmonary embolism, wound infection, or peroneal nerve injury. No patients reported postoperative neuralgia. Caval interruption was not performed preoperatively in any of the patients. The mean follow-up was 63.6 months (median, 56 months; range, months). All patients reported initial improvement. At the time of the last follow-up, 18 patients (90%) were free of pain and free of swelling with the use of compressive garments, with good compliance reported in all 18 patients. The remaining two patients (10%) relayed the same symptoms as before surgery. Varicosities recurred in 10 patients (50%). Three patients (15%) underwent repeat excision for recurrence. Three patients (15%) required multiple resections of the same VM, with delays of 8 months, 8 years, and 9 years. The ulcer did not heal in one patient, another patient had new ulcers 8 years after surgery, and one had chronic cellulitis associated with chylous drainage from lymphatic vesicles of the lower extremity. Follow-up CEAP

5 844 Noel et al November 2000 Fig 5. MRI of the leg of 7-year-old boy with extensive VMs of left leg involving the soleus and gastrocnemius muscles. Also note large lateral vein (arrow). Table III. Comparison of preoperative and postoperative CEAP scores in 20 patients with KTS Preoperative Postoperative CEAP score no. of patients (%) no. of patients (%) C1 0 (0) 0 (0) C2 0 (0) 10 (50) C3 17 (85) 6 (30) C4 1 (5) 1 (5) C5 0 (0) 1 (5) C6 2 (10) 2 (10) scores were C-2 in 10 patients (50%), C-3 in 6 (30%), C-4 and C-5 in 1 each (5%), and C-6 in 2 (10%) (Table III). Clinical severity scores improved from 4.3 ± 2.2 to 3.1 ± 2.3. (P =.03). DISCUSSION In 1900, Klippel and Trénaunay 3 described a syndrome encompassing extremity soft tissue and bony hypertrophy, varicosity and hemangioma. Since that time, the definition of KTS has been refined. The hemangiomas in patients with KTS are vascular malformations. Frequent anomalies of the deep venous system, such as agenesis, hypoplasia, atresia, valvular incompetence, or external compression of the veins by fibrous bands accompany the classic clinical presentation of KTS. 1,4,5 True hemangiomas, which are neoplasms with proliferating endothelium, rarely occur in KTS, and the VMs or capillary malformations associated with KTS contain static endothelium. 6 An important distinction of KTS is the absence of significant arteriovenous shunting. High-flow high-shunt arteriovenous fistulae are characteristics of Parkes-Weber syndrome, a malformation that has significantly more hemodynamic complications. 7,8 The etiology of KTS is still under investigation. According to the definition by Szilagyi et al, 6 KTS is a mixed vascular malformation without macrofistulous arteriovenous communication. Suggested causes include a developmental mesodermal abnormality, which results in an increase in the size and number of veins and subsequently causes increased bone and soft tissue growth. 9 This hypothesis is supported by the measurement of increased blood flow in affected KTS limbs, especially those extremities with capillary malformations, despite the absence of detectable arteriovenous fistulae. 9 The hypothesized mesodermal abnormality may be regulated by angiogenesis and vasculogenesis factors such as vascular endothelial growth factor (VEGF). 10 Disruption of the delicate balance of VEGF-mediated vascular remodeling may result in abnormal vascular development and subsequent limb hypertrophy. However, the effect of hemodynamic factors on limb hypertrophy is not confirmed and may be less significant than previously thought. Our opinion remains that soft tissue and bony hypertrophy are not direct consequences of venous stasis in these patients. Most cases are sporadic, and familial trends have not been confirmed, although some cases within the same family have been reported. In a recent series of 114 patients with KTS, two families with familial KTS were documented, as well as the occurrence of familiar hemihypertrophy and naevi inflammei in first-degree relatives in other pedigrees. 11 In a report of 14 patients from Spain, paternal and maternal age and the number of pregnancies were related to the prevalence of KTS, which suggested a variably expressed autosomal dominant inheritance. 12 However, because of the small sample size, larger series must be reviewed to document genetic associations. In most cases, the patient s family notes the clin-

6 Volume 32, Number 5 Noel et al 845 ical presentation of KTS at birth or in the first few years of life. 13 In a large series of 252 patients with KTS reported from our institution, 98% had capillary malformations, 72% had varicosities or VMs, and 67% had limb hypertrophy. Of these 252 patients, 63% had all three features of KTS, which is comparable to the 55% of patients with all three sequelae in our small surgical group. Although they are not noted in our current series, syndactyly, macrodactyly, polydactyly, and hip dysplasia have been reported in up to 29% of patients with KTS. 1,13 Clinical symptoms are related to the extent and location of the hypertrophy and VMs, which may vary from mild varicosities to massively enlarged limbs or truncal involvement. 14,15 In our series, all patients considered for surgical treatment for VMs and varicosities were symptomatic, with pain and edema the most frequent complaints. Asymptomatic patients were not offered vascular surgical intervention and were managed expectantly because of the high frequency of recurrence of varicosities (50%). KTS may also be associated with lymphedema or lymphatic malformations, as evidenced in one of our patients with chylous reflux. 4,16 Before surgical intervention, contrast phlebography and MRI or MRP should document venous anatomy. The primary goal is to confirm patency of the deep venous system before excision of superficial veins. In addition, descending phlebography, with duplex scanning, is used to assess deep venous incompetence in the deep, superficial, and perforator system in the extremity and the pelvis. 1 All patients considered for surgical treatment should have contrast phlebography for operative planning. Despite narrowing or hypoplasia that is evident on phlebography, satisfactory collateralization is often seen on ascending phlebography. Therefore, judgment and experience are necessary to determine the need for venous reconstruction or excision of constricting bands. Duplex scan examination is important, particularly in mapping incompetent perforating veins if SEPS is planned on the basis of patient symptoms. 17,18 MRI and MRP provide threedimensional imaging of VMs and are useful in defining the hypertrophy of the bony and soft tissue and the extent and depth of VMs within the subcutaneous or subfascial space. MRI may provide the only documentation of PSV, which is confirmed with phlebography in only 60% to 70% of patients with PSV. 19 Evidence of intramuscular VMs on MRI will guide surgical therapy, because such lesions are difficult to treat surgically and nearly always recur. Scanograms (long bone films) are usually performed serially in all patients with KTS to document leg length discrepancy and bony hypertrophy. Surgical intervention is guided by symptoms. Although bleeding may be an indication to operate, most patients present with symptoms of persistent venous hypertension. Pain, aching, swelling, and ulceration can develop as a result of severe valvular incompetence in persistent superficial embryonic veins, in perforating veins, or in abnormal deep veins. Although rare, deep venous reconstruction or release of restricting fibrous bands is indicated in a small group of patients, including two (10%) of our 20 patients. 20,21 One of our patients had symptoms of venous hypertension after stripping of the greater saphenous vein elsewhere. Hypoplastic deep veins were subsequently diagnosed, and the patient underwent venous bypass grafting. The results for these patients are good, but difficult to assess because of the small volume of cases. Deep vein reconstruction is indicated in symptomatic patients with unilateral hypoplastic or absent veins with venous outflow obstruction due to inadequate or surgically removed collateral drainage. SFV transposition has been described by Taheri et al 21 in a 17- year-old patient with KTS with absent SFV valves and symptomatic Grade IV reflux. The release of extrinsic compressive bands surrounding deep veins in KTS has been best documented by Servelle. In his series of 768 cases, he found popliteal compression in 71%, with a perivenous sheath in 15%, which was based primarily on operative observations rather than preoperative phlebography. 4 On the basis of this large series, Servelle recommended resection of the compressive sheaths along the length of the vein to restore the vein to nearly normal caliber. The most common venous surgical treatments are stripping of veins, including lateral embryonic veins, and avulsion or excision of varicosities and VMs. Before surgery, venous anatomy must be confirmed with contrast phlebography, MRI, and MRP. The use of intraoperative tourniquet, inflated to 300 mm Hg to reduce blood loss in patients with KTS with high venous pressure, is helpful. Although 50% of patients with KTS will have recurrent varicosities and it is nearly impossible to remove all varicosities because of the extent of disease, the overall clinical improvement after surgical treatment is good. This improvement is documented by a significant reduction in the clinical severity score in our patients. In addition, most of our patients reported subjective improvement in symptoms, which is consistent with other series of surgically treated patients with KTS. 22 Mildly symptomatic patients, however,

7 846 Noel et al November 2000 should still be managed with compressive garments unless symptoms progress. Postoperative sclerotherapy with 0.75% to 1% polidocanol or sodium tetradecyl sulfate (Sotradecol) has been reported to be a useful adjunct to surgery in patients with intractable symptoms. 23 To date, we have not used sclerotherapy in our practice. In our series, perioperative complications were fortunately rare. Despite the reported proclivity of patients with KTS for DVT, 24 DVT or pulmonary embolism did not occur in patients in our series, perhaps because of the use of compressive garments and early mobilization. In a subset of patients with incompetent perforating veins, the adjunctive use of SEPS can be considered after careful documentation of the venous anatomy. Early in our series, open ligation of incompetent perforating veins through a limited incision was performed at the time of VM excision in one patient. Open perforator vein ligation procedures have been replaced at our institution with SEPS, which is completed under tourniquet control to provide a bloodless endoscopic field. Again, preoperative imaging, especially duplex scan documentation and marking of incompetent perforators, is an important adjunct to successful SEPS. 25 Although most patients with KTS are managed nonoperatively, the venous disease associated with KTS can be treated successfully with surgery when patients are selected carefully and venous anatomy is well defined with preoperative contrast phlebography and MRI. Patent deep veins must be present to excise symptomatic varicose veins and VMs. Although excision of varicosities is often incomplete and VMs may recur in 50% of patients, overall clinical improvement is noted in most patients, and reexcision may be performed as indicated. Occasionally, deep vein reconstruction, excision of a PSV, or SEPS is indicated. Because KTS is rare and the patients problems often complex, patients should receive multidisciplinary care in qualified vascular centers. REFERENCES 1. Gloviczki P, Stanson AW, Stickler GB, Johnson CM, Toomey BJ, Meland NB, et al. Klippel-Trenaunay syndrome: the risks and benefits of vascular interventions. Surgery 1991;110: Executive Committee of the American Venous Forum. Classification and grading of chronic venous disease in the lower limbs: a consensus statement. In: Gloviczki P, Yao JST, editors. Handbook of venous disorders. 1st ed. New York: Chapman & Hall Medical; p Klippel M, Trénaunay P. Du naevus variquex osteohypertrophique. Archives of General Medicine (Paris) 1900;3: Servelle M. Klippel and Trenaunay s syndrome: 768 operated cases. Ann Surg 1985;201: Baskerville PA, Ackroyd JS, Lea TM, Browse NL. The Klippel- Trenaunay syndrome: clinical, radiological and haemodynamic features and management. Br J Surg 1985;72: Szilagyi DE, Smith RF, Elliott JP, Hageman JH. Congenital arteriovenous anomalies of the limbs. Arch Surg 1976;111: Lindenauer SM. The Klippel-Trenaunay syndrome: varicosity, hypertrophy and hemangioma with no arteriovenous fistula. Ann Surg 1965;162: Villavicencio JL. Congenital vascular malformations of venous predominance: Klippel-Trenaunay syndrome. In: Raju S, Villavicencio JL, editors. Surgical management of venous disease. 1st ed. Baltimore: Williams & Wilkins; p Baskerville PA, Ackroyd JS, Browse NL. The etiology of the Klippel-Trenaunay syndrome. Ann Surg 1985;202: Berry SA, Peterson C, Mize W, Bloom K, Zachary C, Blasco P, et al. Klippel-Trenaunay syndrome. Am J Med Genet 1998;79: Aelvoet GE, Jorens PG, Roelen LM. Genetic aspects of the Klippel-Trenaunay syndrome. Br J Dermatol 1992;126: Lorda-Sanchez I, Prieto L, Rodriguez-Pinilla E, Martinez- Frias ML. Increased parental age and number of pregnancies in Klippel-Trenaunay-Weber syndrome. Ann Hum Genet 1998;62: Jacob AG, Driscoll DJ, Shaughnessy WJ, Stanson AW, Clay RP, Gloviczki P. Klippel-Trenaunay syndrome: spectrum and management. Mayo Clin Proc 1998;73: Telander RL, Kaufman BH, Gloviczki P, Stickler GB, Hollier LH. Prognosis and management of lesions of the trunk in children with Klippel-Trenaunay syndrome. J Pediatr Surg 1984;19: Samuel M, Spitz L. Klippel-Trenaunay syndrome: clinical features, complications and management in children. Br J Surg 1995;82: Collins PS, Villavicencio JL, Abreu SH, Gomez ER, Coffey JA, Connaway C, et al. Abnormalities of lymphatic drainage in lower extremities: a lymphoscintigraphic study. J Vasc Surg 1989;9: Gloviczki P, Bergan JJ, Menawat SS, Hobson RW, Kistner RL, Lawrence PF, et al. Safety, feasibility, and early efficacy of subfascial endoscopic perforator surgery: a preliminary report from the North American registry. J Vasc Surg 1997;25: Gloviczki P, Bergan JJ, Rhodes JM, Canton LG, Harmsen S, Ilstrup DM. Mid-term results of endoscopic perforator vein interruption for chronic venous insufficiency: lessons learned from the North American subfascial endoscopic perforator surgery registry. The North American Study Group. J Vasc Surg 1999;29: Cherry KJ, Gloviczki P, Stanson AW. Persistent sciatic vein: diagnosis and treatment of a rare condition. J Vasc Surg 1996;23: Gloviczki P, Hollier LH, Telander RL, Kaufman B, Bianco AJ, Stickler GB. Surgical implications of Klippel-Trenaunay syndrome. Ann Surg 1983;197: Taheri SA, Williams J, Boman L, Pisano S. Superficial femoral vein transposition in Klippel-Trenaunay syndrome. J Pediatr Surg 1989;24: Villavicencio JL. Treatment of varicose veins associated with congenital vascular malformations. In: Bergan JJ, Goldman MP, editors. Complex problems involving varicose veins. Part 4.St Louis: Quality Medical Publishing Company; p Raju S, Fredericks R. Venous obstruction: an analysis of one hundred thirty-seven cases with hemodynamic, venographic, and clinical correlations. J Vasc Surg 1991;14:

8 Volume 32, Number 5 Noel et al Stone DH, Adelman MA, Rosen RJ, Riles TS, Lamparello PJ, Jacobowitz GR, et al. A unique approach in the management of vena caval thrombosis in a patient with Klippel- Trenaunay syndrome. J Vasc Surg 1997;26: Gloviczki P, Bergan JJ, Rhodes JM, Canton LG, Harmsen S, Ilstrup DM, et al. Mid-term results of endoscopic perforator vein interruption for chronic venous insufficiency: lessons learned from the North American Subfascial Endoscopic Perforator Surgery (NASEPS) Registry. J Vasc Surg 1999;29: Submitted Feb 17, 2000; accepted Jun 5, LIFELINE FOUNDATION E. J. Wylie Traveling Fellowship Guidelines: The primary purpose of the E. J. Wylie Traveling Fellowship is to provide the recipient with the opportunity to visit a number of excellent vascular surgery centers in the United States and abroad. Though brief, these visits stimulate academic inspiration, promote international exchange, and foster development of fraternal fellowship in vascular surgery. The achievement of these objectives will enhance the development of the fellow s career in vascular surgery. This award is not intended to support specific research interests but rather to assist the fellow in a unique opportunity for travel and professional exchange within established vascular centers in this country and abroad. Eligibility for Selection: 1. Be under age 40 at the time of the award 2. Have completed a postgraduate vascular training program or have considerable experience in vascular surgery supplemental to surgical training 3. Be committed to an academic career in vascular surgery and have obtained an academic appointment in a medical school or freestanding clinic devoted to excellence in medical education 4. Have a demonstrated record of success in pursuing clinical or basic science research sufficient to ensure academic excellence in his or her pursuit of a career in vascular surgery Selection will be made without regard to the candidate s geographic location. Requirements for Consideration: A candidate submitting documentation for consideration for selection must furnish an up-to-date curriculum vitae; a list of publications, research projects, and current research support; and a list of the centers that he or she wants to visit. Three letters of recommendation are required, including one from the division head and another from the chairman of the department of surgery of the institution in which the candidate holds a faculty appointment. A 500-word essay describing the objectives of the candidate s travel plans and linking these to his or her career goals must be appended. Report to Committee: A report covering your experience should be prepared and forwarded to the Chairman of the Research & Education Committee within 3 months of completion of your fellowship travel. This report should be five to eight double-spaced typewritten pages and should summarize your activities during the fellowship. Although factual statements of activities should be included, you are encouraged to place these within an overall context of their impact on your education and maturation. The format of the report and its content should be suitable for consideration by the Committee for publication in the Journal of Vascular Surgery. Financial Support: The generosity of W. L. Gore & Associates, Inc, has allowed the establishment of this fellowship. Their graciousness ensures the noncommercial nature of the award and its continuation in years to come. The E. J. Wylie Traveling Fellowship of the Lifeline Foundation will pay up to $12,000 for expenses of travel, research, and clerical help. The fellowship monies may not be used for other purposes. Application: No application forms are required. A letter demonstrating interest in applying for the E. J. Wylie Traveling Fellowship or nominating a candidate may be sent to the Chairman of the Research and Education Committee. Details of the application should include the materials requested above. The deadline for receiving applications is January 15. Decisions regarding the award will be mailed to the applicants by mid April. A letter of nomination or intent should be directed to: Chairman Research and Education Committee Lifeline Foundation 13 Elm Street Manchester, MA 01944

Klippel - Trenaunay Syndrome (KTS) When and What to do? Dr. Ayhan ŞENOL SBU.Gazi Yasargil ETH. Diyarbakır /TURKEY

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