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      Termoablación venosa de extremidad inferior en un paciente con síndrome de Klippel-Trénaunay Translated title: Lower extremity venous thermoablation in a patient with Klippel-Trénaunay syndrome

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          Abstract

          Resumen Introducción: el síndrome de Klippel-Trénaunay (SKT) es una entidad clínica rara y esporádica. La tríada clásica define malformación capilar, hipertrofia ósea y de tejidos blandos y varicosidades atípicas. Caso clínico: se describe el caso de una paciente de 14 años de edad que consulta por mancha en vino de Oporto, dolor en miembro inferior izquierdo, edema y discrepancia en la longitud de las extremidades inferiores. Se catalogó como síndrome de Klippel-Trénaunay por la tríada clásica presentada. Discusión: la paciente se benefició de termoablación venosa asociada a flebectomías y de oclusión venosa con escleroespuma.

          Translated abstract

          Abstract Introduction: Klippel-Trénaunay syndrome (SKT) is a rare and sporadic disease, characterized by the clinical triad of capillary malformations, bone and soft tissue hypertrophy and varicose veins. Case report: we report the case of a 14-year-old female patient who presented a port-wine stain, pain in the left lower limb, edema, and discrepancy in the length of this limb. It was classified as Klippel-Trénaunay syndrome due to the classical triad presented. Discussion: the patient underwent venous thermoablation associated with phlebectomies and sclerofoam venous occlusion.

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          Klippel-Trénaunay syndrome: spectrum and management.

          To describe a series of 252 patients with Klippel-Trénaunay syndrome (KTS), a rare congenital malformation characterized by the triad of capillary malformations, atypical varicosities or venous malformations, and bony or soft tissue hypertrophy usually affecting one extremity. We reviewed the clinical characteristics and findings in 136 female and 116 male patients with KTS who underwent assessment at Mayo Clinic Rochester between January 1956 and January 1995. In addition, management options are discussed. Capillary malformations (port-wine stains) were found in 246 patients (98%), varicosities or venous malformations in 182 (72%), and limb hypertrophy in 170 (67%). All three features of KTS were present in 159 patients (63%), and 93 (37%) had two of the three features. Atypical veins, including lateral veins and persistent sciatic vein, occurred in 182 patients (72%). Operations performed in 145 patients with KTS included epiphysiodesis, stripping of varicose veins or venous malformations, excision of vascular malformations, amputations, and debulking procedures. Most patients with KTS should be managed conservatively. The clearest indication for operation is a leg length discrepancy projected to exceed 2.0 cm at skeletal maturity, which can be treated with epiphysiodesis in the growing child. If a functioning deep vein system is present, removal of symptomatic varicosities or localized superficial venous malformations in selected patients can yield good results.
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            Klippel–Trénaunay Syndrome – A Very Rare and Interesting Syndrome

            Klippel–Trénaunay syndrome (KTS or KT) is an infrequently seen dermatological syndrome, which is often viewed as a triad of vascular malformation (capillary malformations or port-wine brands), venous varicosity, and soft tissue and/or bony hypertrophy. We report a case of a 12-year-old male who presented to us with the symptoms of varicose plaques over both lower limbs and was diagnosed as a case of KTS. Management is normally conservative and includes stockings for compression of the branches to reduce edema because of chronic venous insufficiency; modern devices that cause on and off pneumatic compression; and rarely, surgical correction of varicose veins with lifelong follow-up. The orthopedic abnormalities are treated with epiphysiodesis in order to prevent (stop) overgrowing of limb and correction of bone deformity.
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              Radiological Aspect of Klippel-Trénaunay Syndrome: A Case Series With Review of Literature.

              Klippel-Trénaunay syndrome (KTS) is a rare angio-osteo-hypertrophic syndrome characterized by vascular malformations, soft tissue and/or bone hypertrophy, and varicose veins. For the purpose of describing the imaging findings and elucidating the role of medical imaging in the diagnosis and assessment of patient with KTS, we have reviewed the imaging data of 14 KTS patients. The imaging features on different imaging modalities were analyzed. Unilateral lower limb involvement was evident in 71% of cases (n=10) and bilateral but asymmetric lower limb involvement in the remaining 29% of cases (n=4). The most commonly depicted imaging features were varicosities in 93% (n=13), muscle hypertrophy in 79% (n=11) and venous anomalies in 64% (n=9). Other less common imaging findings included lymphedema in 29% (n=4), arterial malformations 29% (n=4), soft tissue hemangiomas 21% (n=3), pelvic and thigh phleboliths 21% (n=3), venous aneurysms 21% (n=3), bone abnormalities 14% (n=2) and lymphadenopathy 14% (n=2). A severe unilateral lower limb deformity resulting in contractures and muscle atrophy of the whole limb was depicted in 1 case. The pathognomonic marginal vein of Servelle was identified in 2 cases. AV shunt was highly suspected in 4 cases and was confirmed by DSA in 1 case, making Klippel-Trénaunay-Weber syndrome a more apt diagnosis. Associated ipsilateral duplicated renal artery was found in 1 case. We have concluded that medical imaging is the cornerstone in the diagnosis and assessment of severity and complications, follow-up and differentiation of KTS from other similar conditions. Different imaging modalities play complementary roles in the evaluation of KTS patients.
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                Author and article information

                Journal
                angiologia
                Angiología
                Angiología
                Arán Ediciones S.L. (Madrid, Madrid, Spain )
                0003-3170
                1695-2987
                December 2023
                : 75
                : 6
                : 399-402
                Affiliations
                [1] Quito orgnameHospital General Docente de Calderón Ecuador
                Article
                S0003-31702023000600008 S0003-3170(23)07500600008
                10.20960/angiologia.00548
                bb2c67a3-605f-4fce-a3a1-b749d92853bb

                This work is licensed under a Creative Commons Attribution-NonCommercial-ShareAlike 4.0 International License.

                History
                : 05 July 2023
                : 11 September 2023
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 10, Pages: 4
                Product

                SciELO Spain

                Categories
                Casos Clínicos

                Klippel-Trénaunay,Syndrome,Angiodisplasia,Síndrome,Angiodysplasia

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