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Year : 2015  |  Volume : 2  |  Issue : 1  |  Page : 16-17

Klippel Trenaunay Syndrome and Variants: How to Proceed

Division of Vascular and Endovascular Surgery, Gonda Vascular Center, Mayo Clinic, Rochester, MN, USA

Date of Web Publication5-Mar-2015

Correspondence Address:
Dr. Manju Kalra
Division of Vascular and Endovascular Surgery, Gonda Vascular Center, Mayo Clinic, Rochester, MN
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0972-0820.152826

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How to cite this article:
Kalra M. Klippel Trenaunay Syndrome and Variants: How to Proceed. Indian J Vasc Endovasc Surg 2015;2:16-7

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Kalra M. Klippel Trenaunay Syndrome and Variants: How to Proceed. Indian J Vasc Endovasc Surg [serial online] 2015 [cited 2022 Dec 3];2:16-7. Available from:

Klippel-Trenaunay syndrome (KTS) is a complex congenital malformation that may affect the lower or upper extremities, or less commonly involve the trunk, head or neck. Described by Klippel and Trenaunay in 1900 as 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 KTS patients are vascular malformations. 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. These lesions are frequently of lateral distribution and rarely cross the midline. Typically, one lower extremity is involved, but bilateral presentation or upper extremity involvement is possible. Frequently, venous drainage is abnormal due to persistent embryonic veins, and agenesis, hypoplasia, atresia, valvular incompetence, external compression of the veins by fibrous bands or aneurysms of deep veins. The hallmark of VMs in KTS is the persistence of embryonic veins. The lateral marginal vein of Servelle has been the most typical finding. Another persistent embryonic vein is the sciatic vein. An important distinction of KTS is the absence of significant arteriovenous shunting. High flow high shunt arteriovenous fistulae are more characteristics of Parkes Weber syndrome, a malformation that has significantly more hemodynamic complications.

The etiology of KTS is still under investigation. Based on the definition by Szilagyi et al., KTS is a mixed vascular malformation without macrofistulous arteriovenous communication. The prominent theory suggests 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. The hypothesized mesodermal abnormality is a disruption of the delicate balance of vascular endothelial growth factor mediated vascular remodeling. It is likely that the soft tissue and bony hypertrophy is not the direct consequence of venous stasis in these patients. Most cases are sporadic and occasional familial trends have been reported but not widely confirmed, suggesting a variably expressed autosomal dominant inheritance.

Diagnostic tests in KTS should focus on the evaluation of the type, extent, and severity of the malformation. The absence of a clinically significant arteriovenous shunt should be confirmed. A thorough physical examination is complemented by color duplex of the venous system to confirm atresia, hypoplasia, and aneurysms of the deep veins as well as evaluate patency and incompetence of deep, superficial, and perforator veins. Plain X-rays of the long bones (scanogram) are helpful to measure the length of bones. Magnetic resonance imaging (MRI) can differentiate between muscle, bone, fat, and vascular tissue. Contrast venography can be performed through multiple injections in the limb. A tourniquet can be used to force a contrast into the deep system to visualize it. Venography is frequently the only test that can help estimate the degree of deep venous occlusion and the presence of sufficient collateral circulation to permit excision or ablation of large incompetent superficial embryonic veins.

The management of KTS is mostly conservative with a small minority of patients undergoing surgical treatment. The mainstay has been compression therapy in the form of elastic garments, nonelastic bandages, intermittent pneumatic compression and physical therapy using massage treatment. The psychological impact caused by a visible deformity of KTS should not be underestimated. Absolute indications for surgical treatment include hemorrhage, infections, acute thromboembolism or refractory venous ulcers. Relative indications include pain, functional impairment, swelling due to chronic venous insufficiency, limb asymmetry or major cosmetic reasons.

Intervention is reserved for selectively symptomatic patients with KTS. A careful evaluation must precede any intervention. The extent of malformations and patency of the deep system must be assessed with MRI/conventional phlebography. Patent deep veins must be present in order to excise symptomatic varicose veins. High ligation of the incompetent marginal vein, invagination stripping of long superficial veins and ambulatory phlebectomy through stab wounds are the most commonly used techniques. The use of subfascial endoscopic perforator surgery in patients with large incompetent perforating veins and venous ulcers has been useful and some patients benefit from deep venous reconstructions. Limb exsanguination with Esmarque bandage and tourniquet use help to reduce intraoperative blood loss during varicose vein avulsion or SEPS. Over the last decade, endovenous thermal ablation was gaining popularity and can be safely performed with the use of adequate tumescent anesthesia. In addition, serial alcohol ablation of VMs and liquid/foam sclerotherapy with Polidocanol or Sodium Tetradecyl sulfate for varicose veins are being used as adjunctive techniques with increasing frequency. Lumbar sympathectomy is helpful for occasional severe hyperhidrosis in these patients. The placement of a temporary IVC filter is indicated in patients with a history of pulmonary thromboembolism. Limb amputation remains the last resort in the setting of severe pain, hemorrhage or dysfunction.

Although excision/ablation of varicosities often is incomplete and VMs may recur in 50% of patients, overall clinical improvement is noted in most patients, and re-excision may be performed as indicated. Since KTS is rare and the patients' problems often complex, patients should receive multidisciplinary care in qualified vascular centers.


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