title: Diagnosis and Treatment of Venous Malformations. Consensus Document of the International Union of Phlebology (IUP): updated 2013.
abstract: Venous malformations (VMs) are the most common vascular developmental anomalies (birth defects) . These defects are caused by developmental arrest of the venous system during various stages of embryogenesis. VMs remain a difficult diagnostic and therapeutic challenge due to the wide range of clinical presentations, unpredictable clinical course, erratic response to the treatment with high recurrence/ persistence rates, high morbidity following non-specific conventional treatment, and confusing terminology. The Consensus Panel reviewed the recent scientific literature up to the year 2013 to update a previous IUP Consensus (2009) on the same subject. ISSVA Classification with special merits for the differentiation between the congenital vascular malformation (CVM) and vascular tumors was reinforced with an additional review on syndrome-based classification. A "modified" Hamburg classification was adopted to emphasize the importance of extratruncular vs. truncular sub-types of VMs. This incorporated the embryological ongm, morphological differences, unique characteristics, prognosis and recurrence rates of VMs based on this embryological classification. The definition and classification of VMs were strengthened with the addition of angiographic data that determines the hemodynamic characteristics, the anatomical pattern of draining veins and hence the risk of complication following sclerotherapy. The hemolymphatic malformations, a combined condition incorporating LMs and other CVMs, were illustrated as a separate topic to differentiate from isolated VMs and to rectify the existing confusion with name-based eponyms such as Klippei-Trenaunay syndrome. Contemporary concepts on VMs were updated with new data including genetic findings linked to the etiology of CVMs and chronic cerebrospinal venous insufficiency. Besides, newly established information on coagulopathy including the role of D-Dimer was thoroughly reviewed to provide guidelines on investigations and anticoagulation therapy in the management of VMs. Congenital vascular bone syndrome resulting in angio-osteo-hyper/hypotrophy and (lateral) marginal vein was separately reviewed. Background data on arterio-venous malformations was included to differentiate this anomaly from syndromebased VMs. For the treatment, a new section on laser therapy and also a practical guideline for follow up assessment were added to strengthen the management principle of the multidisciplinary approach. All other therapeutic modalities were thoroughly updated to accommodate a changing concept through the years.
authors.forename: B B
authors.initials: BB
authors.lastname: Lee
authors.forename: I
authors.initials: I
authors.lastname: Baumgartner
authors.forename: P
authors.initials: P
authors.lastname: Berlien
authors.forename: G
authors.initials: G
authors.lastname: Bianchini
authors.forename: P
authors.initials: P
authors.lastname: Burrows
authors.forename: P
authors.initials: P
authors.lastname: Gloviczki
authors.forename: Y
authors.initials: Y
authors.lastname: Huang
authors.forename: J
authors.initials: J
authors.lastname: Laredo
authors.forename: D A
authors.initials: DA
authors.lastname: Loose
authors.forename: J
authors.initials: J
authors.lastname: Markovic
authors.forename: R
authors.initials: R
authors.lastname: Mattassi
authors.forename: K
authors.initials: K
authors.lastname: Parsi
authors.forename: E
authors.initials: E
authors.lastname: Rabe
authors.forename: M
authors.initials: M
authors.lastname: Rosenblatt
authors.forename: C
authors.initials: C
authors.lastname: Shortell
authors.forename: F
authors.initials: F
authors.lastname: Stillo
authors.forename: M
authors.initials: M
authors.lastname: Vaghi
authors.forename: L
authors.initials: L
authors.lastname: Villavicencio
authors.forename: P
authors.initials: P
authors.lastname: Zamboni
mesh_terms: D001706:Biopsy; D003131:Combined Modality Therapy; D003952:Diagnostic Imaging / Q000379:methods / Q000592:standards*; D057510:Endovascular Procedures / Q000009:adverse effects / Q000592:standards*; D006801:Humans; D010348:Patient Care Team / Q000592:standards; D018579:Patient Selection; D011237:Predictive Value of Tests; D012307:Risk Factors; D015911:Sclerotherapy / Q000009:adverse effects / Q000592:standards*; D009626:Terminology as Topic; D016896:Treatment Outcome; D054079:Vascular Malformations / Q000145:classification / Q000175:diagnosis* / Q000628:therapy*; D014656:Vascular Surgical Procedures / Q000009:adverse effects / Q000592:standards*; D014680:Veins / Q000002:abnormalities