Jornal Vascular Brasileiro
https://jvascbras.org/article/doi/10.1590/S1677-54492011000100015
Jornal Vascular Brasileiro
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Síndrome de Klippel-Trenaunay-Weber: associação do tratamento operatório à escleroterapia por espuma

Klippel-Trenaunay-Weber Syndrome: association of operative treatment with foam sclerotherapy

Ronald Luiz Gomes Flumignan; Daniel Guimarães Cacione; Silvia Iglesias Lopes; Caroline Nicacio Bessa Clezar; Carolina Dutra Queiroz; Aécio Rubens Dias Pereira-Filho; Newton de Barros Jr; Fausto Miranda Jr

http://dx.doi.org/10.1590/S1677-54492011000100015 J Vasc Bras, vol.10, n1, p.77-80, 2011
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Resumo

A Síndrome de Klippel-Trenaunay-Weber representa a associação de hemangiomas planos, ectasias venosas e hipertrofia do segmento corpóreo afetado. Apresenta-se o caso de um paciente de 39 anos, sexo masculino, seguido desde 1993 no serviço da Disciplina de Cirurgia Vascular da Escola Paulista de Medicina (Unifesp), por quadro inicial da síndrome em membro inferior esquerdo. O paciente foi tratado apenas por elastocompressão e medidas gerais. Neste período, ele evoluiu com piora da estase venosa, da hipertrofia óssea e da hiperpigmentação de pele. Na ultrassonografia Doppler colorida venosa dos membros inferiores havia perviedade do sistema venoso profundo, hiperfluxo venoso, insuficiência segmentar de veia safena magna com sinais de tromboflebite e tributárias insuficientes. Indicou-se, para a melhora da estase venosa, ligadura da junção safeno-femoral esquerda e escleroterapia (polidocanol 3%) retrógrada da mesma, com exérese das ectasias venosas. O paciente recebeu alta hospitalar no primeiro dia pós-operatório e atualmente está em acompanhamento ambulatorial com melhora significativa dos sintomas.

Palavras-chave

Varizes, escleroterapia, Síndrome de Klippel-Trenaunay-Weber, soluções esclerosantes

Abstract

The Klippel-Trenaunay-Weber Syndrome is the association of hemangioma, venous ectasia, and hypertrophy of the affected body segment. We report the case of a 39-year-old male followed-up since 1993 due to onset of symptoms in the left lower limb. He was treated only with the use of elastic stockings and general measures. Over the years, he had worsening of venous stasis, of bone hypertrophy, and of skin hyperpigmentation. Color-coded Doppler ultrasonography of the lower limbs showed patency of the deep venous system, venous overflow, segmentar insufficiency of the greater saphenous vein with signs of thrombophlebitis, and insufficient tributary veins. In order to improve venous stasis, ligation of the left sapheno-femoral junction and retrograde foam sclerotherapy (polidocanol 3%) with resection of tributary veins were performed. The patient was discharged in the first postoperative day and has been followed as an outpatient, presenting significant improvement of the symptoms.

Keywords

Varicose veins, sclerotherapy, Klippel-Trenaunay-Weber Syndrome, sclerosing solutions

Referências

Klippel M, Trenaunay P. Du noevus variquex oste-hypertrophic. Arch Gen Med. 1900;185:641-72.

Latessa V, Frasier K. Case study: A minimally invasive approach to the treatment of Klippel-Trenaunay syndrome. J Vasc Nurs. 2007;25:-84.

Campos HGA, Curado JH. Angiodisplasias. Doenças Vasculares Periféricas. 2008:1949-76.

Weber PF. Angioma formation in connection with hypertrophy of limbs and hemihypertrophy. Br J Dermatol. 1907;19:231-5.

Mullins JF, Naylor D, Redetzki J. The Klippel-Trenaunay-Weber syndrome. Arch Dermatol. 1962;86:202-6.

Villela ALC, Guedes LGS, Paschoa VVA. Perfil epidemiológico de 58 portadores de síndrome de Klippel-Trenaunay-Weber acompanhados no Ambulatório da Santa Casa de São Paulo. J Vasc Bras. 2009;8(3):219-24.

Lindenauer SM. The Klippel-Trenaunay Syndrome: Varicosity, Hypertrophy and Hemangioma With No Arteriovenous Fistula. Ann Surg. 1965;162(2):303-4.

Gloviczki P, Driscoll DJ. Klippel-Trenaunay syndrome: current management. Phlebology. 2007;22:291-8.

Frasier K, Giangola G, Rosen R. Endovascular radiofrequency ablation: A novel treatment of venous insufficiency in Klippel-Trenaunay patients. J Vasc Surg. 2008;47:1339-45.

Delis KT, Gloviczki P, Wennberg PW. Hemodynamic impairment, venous segmental disease, and clinical severity scoring in limbs with Klippel-Trenaunay syndrome. J Vasc Surg. 2007;45:561-7.

Beebe HG, Bergan JJ, Bergqvist D. Classification and grading of chronic venous disease in the lower limbs: A consensus statement. Eur J Vasc Endovasc Surg. 1996;12(4):487-91.

Beebe HG, Bergan JJ, Bergqvist D. Classification and grading of chronic venous disease in the lower limbs: A consensus statement. Int Angiol. 1995;14(2):197-201.

Beebe HG, Bergan JJ, Bergqvist D. Classification and grading of chronic venous disease in the lower limbs--a consensus statement: Organized by Straub Foundation with the cooperation of the American Venous Forum at the 6th annual meeting, February 22-25, 1994, Maui, Hawaii. Vasa. 1995;24(4):313-8.

Eklöf B, Rutherford RB, Bergan JJ. Revision of the CEAP classification for chronic venous disorders: Consensus statement. J Vasc Surg. 2004;40:1248-52.

Vasquez MA, Munschauer CE. Venous Clinical Severity Score and quality-of-life assessment tools: application to vein practice. Phlebology. 2008;23:259-75.

Aelvoet G, Jorens P, Roelen L. Genetic aspects of the Klippel-Trenaunay syndrome. Br J Derm. 1992;126:603-7.

Jorgenson R, Darby B, Patterson R, Trimmer KJ. Prenatal diagnosis of the Klippel-Trenaunay-Weber syndrome. Pren Diag. 1994;14:989-92.

Figueiredo M, Araújo S, Barros-Jr N, Miranda-Jr F. Results of Surgical Treatment Compared with Ultrasound-Guided Foam Sclerotherapy in Patients with Varicose Veins: A Prospective Randomised Study. Eur J Vasc Endovasc Surg. 2009;38:758-63.

Tessari L, Cavezzi A, Frullini A. Preliminary experience with a new sclerosing foam in the treatment of varicose veins. Dermatol Surg. 2001;27:58-60.

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