Association of localized intravascular coagulopathy with venous malformations.

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.

• Authors: Anne Dompmartin, 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
• Journal: Archives of dermatology (Arch Dermatol) Vol. 144 Issue 7 Pg. 873-7 (Jul 2008) ISSN: 1538-3652 [Electronic] United States
• PMID: 18645138 (Publication Type: Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't)

Chemical References

• Fibrin Fibrinogen Degradation Products
• fibrin fragment D

Topics

• Adolescent
• Adult
• Aged
• Belgium (epidemiology)
• Blood Coagulation Disorders (blood, complications, epidemiology, pathology)
• Child
• Child, Preschool
• Female
• Fibrin Fibrinogen Degradation Products (metabolism)
• France (epidemiology)
• Humans
• Infant
• Male
• Middle Aged
• Pain Measurement
• Prospective Studies
• Risk Factors
• Severity of Illness Index
• Sex Factors
• Ultrasonography
• Vascular Malformations (blood, complications, diagnostic imaging, epidemiology, pathology)