2015•PubMedRequires access
[Some reflections on the Klippel Trenaunay syndrome: A single entity or an association of vascular malformations].
Albino Ja, Lourenço C E Sousa, Pedro Amorim, João Batista Vieira, Gonçalo Sousa, Karla Danielly da Silva Ribeiro, Teresa Vieira, Gonçalo Sobrinho, Nuno Meireles
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Abstract
The author started in the year 2000, in the St Marta Hospital, a consultation for congenital vascular malformations, that was extrapolated to the Pulido Valente Hospital - CHLN where he continues to see multiple patients with this type of pathology, mainly located to the extremities.The Klippel Trenaunay syndrome, which was described as a set of anomalies constituted by the presence of a cutaneous angiomatous malformation ( port wine stain ), varicose veins and gigantism of the limbs, is undoubtedly the most common malformations that he is asked to treat. So he thought that it would be interesting to undertake an actual review of this pathology.After analyzing the various components in question he concludes that the condition is undoubtedly a set of malformations ranging from a cutaneous component, to the venous and lymphatic malformations and clear the osseous component, that causes the bone dysmorphic appearance, and that is essential a multidisciplinary approach to achieve a final satisfactory therapeutic result.