Jornal Vascular Brasileiro
https://jvascbras.org/article/doi/10.1590/1677-5449.200010
Jornal Vascular Brasileiro
Case Report

Conservative management of knee arthropathy in a patient with Klippel Trenaunay syndrome

Manejo conservador da artropatia do joelho em paciente com síndrome de Klippel-Trenaunay

Fanny Rodríguez Santos; Victoria Loson; Agustín Coria; Hugo Martínez

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Abstract

Abstract: Klippel-Trenaunay syndrome (KTS) is a rare vascular malformation characterized by capillary malformation, venous malformations, and soft tissue or bone hypertrophy that affect the extremities in most cases. Knee or hip arthropathy are common associated conditions and cause serious disability. We present the case of a patient with a diagnosis of KTS and severe knee arthropathy. A 34-year-old man with KTS was referred to our hospital with severe knee arthropathy, with the joint fixed in a 90° position. CT Angiography and MRI of the left leg showed important varicose development of the superficial venous system with intraarticular vessels. After discussion of the case by a multidisciplinary committee, the patient was enrolled on a physiotherapy program and had achieved significant improvements in movement and quality of life at 12-month follow-up. Treatment of KTS is primarily conservative and a multidisciplinary approach is necessary.

Keywords

Klippel-Trenaunay syndrome, vascular malformation, knee arthropathy

Resumo

Resumo: A síndrome de Klippel-Trenaunay (SKT) é uma malformação vascular rara caracterizada por malformação capilar, malformações venosas e hipertrofia de tecidos moles ou ósseos que afetam as extremidades na maioria dos casos. A artropatia do joelho ou do quadril é uma condição comumente associada e causa sérias deficiências. Apresentamos o caso de um paciente com diagnóstico de SKT e artropatia grave do joelho. Um homem de 34 anos com SKT foi encaminhado ao nosso hospital com artropatia grave do joelho com articulação fixa na posição de 90 °. A angiotomografia e a ressonância magnética da perna esquerda mostraram importante desenvolvimento varicoso do sistema venoso superficial com vasos intra-articulares. Após o caso ser discutido em um comitê multidisciplinar, o paciente foi incluído em um programa de fisioterapia, obtendo uma melhora significativa nos movimentos e na qualidade de vida após 12 meses de acompanhamento. O tratamento da SKT é principalmente conservador e exige uma abordagem multidisciplinar.
 

Palavras-chave

síndrome de Klippel-Trenaunay, malformação vascular, artropatia do joelho

References

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