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Anne Dompmartin, MD; Aurélie Acher, MD; Pascal Thibon, MD; Sébastien Tourbach, MD; Cédric Hermans, MD, PhD; Véronique Deneys, MD; Ben Pocock, MD; Agnès Lequerrec, MPhSc; Daniel Labbé, MD; Marie-Thérèse Barrellier, MD; Romain Vanwijck, MD, PhD, FRCS; Miikka Vikkula, MD, PhD; Laurence M. Boon, MD, PhD

Arch Dermatol. 2008;144(7):873-877.

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 µg/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.

Author Affiliations: Departments of Dermatology (Drs Dompmartin, Acher, and Barrellier), Hygiene (Dr Thibon), Hematology (Ms Lequerrec), and Plastic Surgery (Dr Labbé), Université de Caen Basse Normandie, Centre Hospitalier Universitaire Caen, Caen, France; Division of Plastic Surgery, Center for Vascular Anomalies (Drs Tourbach, Pocock, Vanwijck, and Boon) and Hematosis and Thrombosis Unit, Division of Haematology & Laboratory of Thrombosis and Haemostasis, Department of Biological Chemistry (Drs Hermans and Deneys), Université catholique de Louvain, Cliniques Universitaires St Luc, Brussels, Belgium; and Laboratory of Human Molecular Genetics, Université catholique de Louvain, Institut de Duve, Brussels (Drs Vikkula and Boon).

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