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Multicenter Study
. 2008 Jul;144(7):873-7.
doi: 10.1001/archderm.144.7.873.

Association of localized intravascular coagulopathy with venous malformations

Anne Dompmartin  1 , Aurélie Acher, Pascal Thibon, Sébastien Tourbach, Cédric Hermans, Véronique Deneys, Ben Pocock, Agnès Lequerrec, Daniel Labbé, Marie-Thérèse Barrellier, Romain Vanwijck, Miikka Vikkula, Laurence M Boon
Affiliations
Multicenter Study

Association of localized intravascular coagulopathy with venous malformations

Anne Dompmartin et al. Arch Dermatol. 2008 Jul.

Abstract

Objective: To determine which venous malformations (VMs) are at risk for coagulopathy. Venous malformations are slow-flow vascular malformations present at birth, and localized intravascular coagulopathy (LIC) causes pain and thrombosis within a lesion and severe bleeding during surgical procedures.

Design: Prospective convenience sample accrued from 2 multidisciplinary sites in Brussels, Belgium, and Caen, France.

Participants: The study population comprised 140 patients with clinical data and coagulation parameters. Magnetic resonance imaging was performed for 110 patients.

Main outcome measure: Measurement of D-dimer levels.

Results: Of the 140 participants, 59 (42%) showed high D-dimer levels, 36 (61%) of whom had levels higher than 1.0 microg/mL. Six of the participants had low fibrinogen levels. In univariate analysis, large surface, presence of palpable phleboliths, and truncal localization were associated with high D-dimer levels. In the multivariate analysis, only large surface area and presence of phleboliths remained independently associated with high D-dimer levels. Severe LIC, characterized by concomitant low fibrinogen level, was associated with extensive venous malformations of the extremities.

Conclusions: Localized intravascular coagulopathy is statistically significantly associated with large and/or deep venous malformations that affect any location, which can have a palpable phlebolith. These patients are at risk of local pain due to thrombosis. Lesions with elevated D-dimer levels associated with low fibrinogen levels (severe LIC) commonly affect an extremity and have a high risk of hemorrhage. Low-molecular-weight heparin can be used both to treat the pain caused by LIC and to prevent decompensation of severe LIC to disseminated intravascular coagulopathy.

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Figures

Figure 1
Figure 1
A) Large, unifocal, trunkal, skin-color, flat venous malformation of a 13-year-old male associated with high D-dimers (1000ng/ml) with palpable phleboliths B) MRI shows muscle involvement of this deep VM C) Multifocal VM on right arm of a 28-year-old male with high D-dimer (7818ng/ml). Additional lesions are present on trunk and other limbs.
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