Journal Information
Vol. 98. Issue 3.
Pages 141-158 (April 2007)
Vol. 98. Issue 3.
Pages 141-158 (April 2007)
Review article
DOI: 10.1016/S1578-2190(07)70418-3
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Vascular Malformations (I). Concept, Classification, Pathogenesis and Clinical Features
Malformaciones Vasculares (I). Concepto, Clasificación, Fisiopatogenia y Manifestaciones Clínicas
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...
P. Redondo??
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predondo@unav.es

Correspondence: Departamento de Dermatología. Clínica Universitaria de Navarra. 31080 Pamplona. Spain.
Departamento de Dermatología, Clínica Universitaria de Navarra, Pamplona, Spain
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Abstract

Vascular malformations are anomalies always present at birth that, contrary to hemangiomas, never regress and may grow during lifetime. Clinical presentation of vascular malformations is extremely variable and ranges from asymptomatic spots of mere aesthetic concern to lesions with high blood flow or located in critical sites that may be life-threatening. Given the low incidence of these disorders it is difficult to establish therapeutic guidelines. In addition to a correct classification of vascular anomalies, it is necessary a multidisciplinary approach for the follow-up and management of these patients. The first part of this review focuses on the different classifications of vascular anomalies, maintaining as reference the one proposed by the International Society for the Study of Vascular Anomalies (ISSVA). Additionally, clinical features of the different subtypes of vascular anomalies as well as their association in certain syndromes are reviewed.

Key words:
vascular malformation
Klippel-Trenaunay
venous
lymphatic
arteriovenous
Resumen

Las malformaciones vasculares son anomalías presentes siempre en el nacimiento que, al contrario que los hemangiomas, nunca desaparecen y pueden crecer durante toda la vida. La presentación clínica de las malformaciones vasculares es extremadamente variable y va desde manchas asintomáticas con repercusión meramente estética, hasta lesiones de alto flujo o localizaciones peculiares que pueden incluso poner en peligro la vida del enfermo. También al tratarse de enfermedades relativamente raras es difícil alcanzar la suficiente experiencia en su manejo para establecer pautas contrastadas de tratamiento. Además de una correcta clasificación de las anomalías vasculares, es necesario un enfoque multidisciplinar respecto del seguimiento y las posibilidades terapéuticas de estos pacientes. En la primera parte de esta revisión se abordan las diferentes clasificaciones de las malformaciones vaculares, manteniendo como referencia la sugerida por la ISSVA (International Society for the Study of Vascular Anomalies). Además se incide en las características clínicas de los diferentes subtipos de malformaciones vasculares así como sus asociaciones en determinados complejos sindrómicos.

Palabras clave:
malformación vascular
Klippel-Trenaunay
venosa
linfática
arterio-venosa
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Supplemental materials
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¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

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