Venous Malformation: update on etiopathogenesis, diagnosis & management
Identifieur interne : 002A24 ( Pmc/Corpus ); précédent : 002A23; suivant : 002A25Venous Malformation: update on etiopathogenesis, diagnosis & management
Auteurs : Anne Dompmartin ; Miikka Vikkula ; Laurence M. BoonSource :
- Phlebology / Venous Forum of the Royal Society of Medicine [ 0268-3555 ] ; 2010.
Abstract
The aim of this review was to discuss the current knowledge on etiopathogenesis, diagnosis and therapeutic management of venous malformations. Venous malformations (VMs) are slow-flow vascular anomalies. They are simple, sporadic or familial (cutaneo-mucosal venous malformation or glomuvenous malformations), combined (e.g. capillaro-venous, capillaro-lymphaticovenous malformations) or syndromic (Klippel-Trenaunay, Blue Rubber Bleb Naevus and Maffucci). Genetic studies have identified causes of familial forms and of 40% of sporadic VMs. Another diagnostic advancement is the identification of elevated D-dimer level as the first biomarker of venous malformations within vascular anomalies. Those associated with pain are often responsive to Low Molecular Weight Heparin which should also be used to avoid disseminated intravascular coagulopathy secondary to intervention, especially if fibrinogen level is low. Finally, development of a modified sclerosing agent, ethylcellulose–ethanol, has improved therapy. It is efficient and safe, and widens indications for sclerotherapy to sensitive and dangerous areas such as hands, feet and periocular area.
Url:
DOI: 10.1258/phleb.2009.009041
PubMed: 20870869
PubMed Central: 3132084
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PMC:3132084Le document en format XML
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<author><name sortKey="Dompmartin, Anne" sort="Dompmartin, Anne" uniqKey="Dompmartin A" first="Anne" last="Dompmartin">Anne Dompmartin</name>
<affiliation><nlm:aff id="A1">Université de Caen Basse Normandie, CHU Caen, Department of Dermatology, Caen (France)</nlm:aff>
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<author><name sortKey="Vikkula, Miikka" sort="Vikkula, Miikka" uniqKey="Vikkula M" first="Miikka" last="Vikkula">Miikka Vikkula</name>
<affiliation><nlm:aff id="A2">Université catholique de Louvain, de Duve Institute, Laboratory of Human Molecular Genetics, B-1200 Brussels, Belgium</nlm:aff>
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<author><name sortKey="Boon, Laurence M" sort="Boon, Laurence M" uniqKey="Boon L" first="Laurence M" last="Boon">Laurence M. Boon</name>
<affiliation><nlm:aff id="A2">Université catholique de Louvain, de Duve Institute, Laboratory of Human Molecular Genetics, B-1200 Brussels, Belgium</nlm:aff>
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<affiliation><nlm:aff id="A3">Université catholique de Louvain, Cliniques universitaires St Luc, Center for Vascular Anomalies, Division of Plastic Surgery<sup><xref ref-type="bibr" rid="R1">1</xref>
</sup>
, B-1200 Brussels, Belgium</nlm:aff>
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<author><name sortKey="Boon, Laurence M" sort="Boon, Laurence M" uniqKey="Boon L" first="Laurence M" last="Boon">Laurence M. Boon</name>
<affiliation><nlm:aff id="A2">Université catholique de Louvain, de Duve Institute, Laboratory of Human Molecular Genetics, B-1200 Brussels, Belgium</nlm:aff>
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<affiliation><nlm:aff id="A3">Université catholique de Louvain, Cliniques universitaires St Luc, Center for Vascular Anomalies, Division of Plastic Surgery<sup><xref ref-type="bibr" rid="R1">1</xref>
</sup>
, B-1200 Brussels, Belgium</nlm:aff>
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<front><div type="abstract" xml:lang="en"><p id="P3">The aim of this review was to discuss the current knowledge on etiopathogenesis, diagnosis and therapeutic management of venous malformations. Venous malformations (VMs) are slow-flow vascular anomalies. They are simple, sporadic or familial (cutaneo-mucosal venous malformation or glomuvenous malformations), combined (e.g. capillaro-venous, capillaro-lymphaticovenous malformations) or syndromic (Klippel-Trenaunay, Blue Rubber Bleb Naevus and Maffucci). Genetic studies have identified causes of familial forms and of 40% of sporadic VMs. Another diagnostic advancement is the identification of elevated D-dimer level as the first biomarker of venous malformations within vascular anomalies. Those associated with pain are often responsive to Low Molecular Weight Heparin which should also be used to avoid disseminated intravascular coagulopathy secondary to intervention, especially if fibrinogen level is low. Finally, development of a modified sclerosing agent, ethylcellulose–ethanol, has improved therapy. It is efficient and safe, and widens indications for sclerotherapy to sensitive and dangerous areas such as hands, feet and periocular area.</p>
</div>
</front>
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<pmc-dir>properties manuscript</pmc-dir>
<front><journal-meta><journal-id journal-id-type="nlm-journal-id">9012921</journal-id>
<journal-id journal-id-type="pubmed-jr-id">29683</journal-id>
<journal-id journal-id-type="nlm-ta">Phlebology</journal-id>
<journal-title-group><journal-title>Phlebology / Venous Forum of the Royal Society of Medicine</journal-title>
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<issn pub-type="ppub">0268-3555</issn>
<issn pub-type="epub">1758-1125</issn>
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<article-meta><article-id pub-id-type="pmid">20870869</article-id>
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<article-id pub-id-type="doi">10.1258/phleb.2009.009041</article-id>
<article-id pub-id-type="manuscript">NIHMS305372</article-id>
<article-categories><subj-group subj-group-type="heading"><subject>Article</subject>
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<title-group><article-title>Venous Malformation: update on etiopathogenesis, diagnosis & management</article-title>
</title-group>
<contrib-group><contrib contrib-type="author"><name><surname>Dompmartin</surname>
<given-names>Anne</given-names>
</name>
<degrees>MD</degrees>
<xref ref-type="aff" rid="A1">*</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Vikkula</surname>
<given-names>Miikka</given-names>
</name>
<degrees>MD, PhD</degrees>
<xref ref-type="aff" rid="A2">**</xref>
</contrib>
<contrib contrib-type="author"><name><surname>Boon</surname>
<given-names>Laurence M</given-names>
</name>
<degrees>MD, PhD</degrees>
<xref ref-type="aff" rid="A2">**</xref>
<xref ref-type="aff" rid="A3">***</xref>
</contrib>
</contrib-group>
<aff id="A1"><label>*</label>
Université de Caen Basse Normandie, CHU Caen, Department of Dermatology, Caen (France)</aff>
<aff id="A2"><label>**</label>
Université catholique de Louvain, de Duve Institute, Laboratory of Human Molecular Genetics, B-1200 Brussels, Belgium</aff>
<aff id="A3"><label>***</label>
Université catholique de Louvain, Cliniques universitaires St Luc, Center for Vascular Anomalies, Division of Plastic Surgery<sup><xref ref-type="bibr" rid="R1">1</xref>
</sup>
, B-1200 Brussels, Belgium</aff>
<author-notes><corresp id="CR1">Corresponding author: Laurence M Boon MD, PhD, Division of Plastic Surgery, Center for Vascular Anomalies, Cliniques universitaires St Luc, Av Hippocrate 10, B-1200 Brussels, Belgium, Phone: +32 2 764 1425, Fax: +32 2 762 6284, <email>laurence.boon@uclouvain.be</email>
, <ext-link ext-link-type="uri" xlink:href="http://http:/www.md.ucl.ac.be/vasc_anom">http:/www.md.ucl.ac.be/vasc_anom</ext-link>
, <ext-link ext-link-type="uri" xlink:href="http://http:/www.deDuveInstitute.be/vikkula">http:/www.deDuveInstitute.be/vikkula</ext-link>
</corresp>
<fn id="FN1"><p id="P1"><bold>Author Contributions</bold>
:</p>
<p id="P2">Critical revision of the manuscript for important intellectual content: Dompmartin A, Vikkula M, Boon LM.</p>
</fn>
</author-notes>
<pub-date pub-type="nihms-submitted"><day>29</day>
<month>6</month>
<year>2011</year>
</pub-date>
<pub-date pub-type="ppub"><year>2010</year>
</pub-date>
<pub-date pub-type="pmc-release"><day>8</day>
<month>7</month>
<year>2011</year>
</pub-date>
<volume>25</volume>
<issue>5</issue>
<fpage>224</fpage>
<lpage>235</lpage>
<abstract><p id="P3">The aim of this review was to discuss the current knowledge on etiopathogenesis, diagnosis and therapeutic management of venous malformations. Venous malformations (VMs) are slow-flow vascular anomalies. They are simple, sporadic or familial (cutaneo-mucosal venous malformation or glomuvenous malformations), combined (e.g. capillaro-venous, capillaro-lymphaticovenous malformations) or syndromic (Klippel-Trenaunay, Blue Rubber Bleb Naevus and Maffucci). Genetic studies have identified causes of familial forms and of 40% of sporadic VMs. Another diagnostic advancement is the identification of elevated D-dimer level as the first biomarker of venous malformations within vascular anomalies. Those associated with pain are often responsive to Low Molecular Weight Heparin which should also be used to avoid disseminated intravascular coagulopathy secondary to intervention, especially if fibrinogen level is low. Finally, development of a modified sclerosing agent, ethylcellulose–ethanol, has improved therapy. It is efficient and safe, and widens indications for sclerotherapy to sensitive and dangerous areas such as hands, feet and periocular area.</p>
</abstract>
<kwd-group><kwd>Klippel-Trenaunay syndrome</kwd>
<kwd>Ethylcellulose-Ethanol</kwd>
<kwd>Sclerotherapy</kwd>
<kwd>D-dimer</kwd>
<kwd>Maffuci syndrome</kwd>
</kwd-group>
</article-meta>
</front>
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</record>
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