Jornal Vascular Brasileiro
https://www.jvascbras.org/article/doi/10.1590/1677-5449.005417
Jornal Vascular Brasileiro
Case Report

Klippel-Trenaunay and Parkes-Weber syndromes: two case reports

Síndromes de Klippel-Trenaunay e Parkes-Weber: dois relatos de caso

Carlos Alberto Araujo Chagas, Lucas Alves Sarmento Pires, Marcio Antonio Babinski, Tulio Fabiano de Oliveira Leite

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Abstract

Parkes-Weber syndrome is a congenital vascular disease that comprises capillary, venous, lymphatic, and arteriovenous malformations. Although Parkes-Weber syndrome is a clinically distinct entity with serious complications, it is still frequently misdiagnosed as Klippel-Trenaunay syndrome, which consists of a triad of malformations involving the capillary, venous, and lymphatic vessels, without arteriovenous fistulas. Both syndromes are generally diagnosed with Doppler ultrasound and confirmed by magnetic resonance angiography. The aim of this study is to describe one case of Klippel-Trenaunay syndrome, in a 36-year-old patient, and one case of Parkes-Weber syndrome, in a 21-year-old patient. We review the literature in order to discuss the possible causes and consequences of these diseases related to venous hypertension and angiodysplasia, taking a clearer approach to their differences, and discussing their treatment.

Keywords

Klippel-Trenaunay syndrome; Parkes-Weber syndrome; angiodysplasia; nevus; arteriovenous malformations

Resumo

A síndrome de Parkes-Weber é uma doença vascular congênita que consiste em malformações capilares, venosas, linfáticas e arteriovenosas. Embora seja uma entidade clinicamente distinta com complicações graves, essa síndrome ainda é frequentemente diagnosticada erroneamente como síndrome de Klippel-Trenaunay, que consiste em uma tríade de má formação nos vasos capilares, venosos e linfáticos, sem fístula arteriovenosa. Ambas as síndromes são geralmente diagnosticadas através de ultrassom Doppler e confirmadas pela angiografia por ressonância magnética. O objetivo deste estudo é descrever um caso de síndrome de Klippel-Trenaunay em um paciente de 36 anos de idade e um caso de síndrome de Parkes-Weber em uma paciente de 21 anos. A literatura foi revisada com o objetivo de discutir as possíveis causas e consequências dessa doença e sua associação à hipertensão venosa e angiodisplasia. O presente trabalho também levanta discussão a respeito das diferenças sintomatológicas de ambas as síndromes e seus respectivos tratamentos.

Palavras-chave

síndrome de Klippel-Trenaunay; síndrome de Parkes-Weber; angiodisplasias; nevus; malformações arteriovenosas.

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