Venous malformation: update on aetiopathogenesis, diagnosis and management.
The aim of this review was to discuss the current knowledge on aetiopathogenesis, diagnosis and therapeutic management of venous malformations (VMs). VMs are slow-flow vascular anomalies. They are simple, sporadic or familial (cutaneomucosal VMs or glomuvenous malformations), combined (e.g. capillaro-venous and 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 VMs 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.
Dompmartin
A
A
Department of Dermatology, Université de Caen Basse Normandie, CHU Caen, Caen, France.
Vikkula
M
M
Boon
L M
LM
D019468:Disease Management; D006391:Hemangioma; D006801:Humans; D009506:Nevus; D054079:Vascular Malformations / Q000175:diagnosis / Q000209:etiology / Q000628:therapy