Differential diagnosis of vascular malformations can be problematic even in specialized interdisciplinary centers. Localized Intravascular Coagulopathy, characterized by elevated D-dimer levels, has been observed in about 40% of patients with venous malformations. We evaluated if this is specific for them, and thus useful for differential diagnosis.

Prospective convenience sample accrued from 2 interdisciplinary sites in Brussels, Belgium and Caen, France.

The study population comprised 280 patients with clinical data, Doppler ultrasound (for 251 patients) and coagulation parameters.

Measurement of D-dimer levels.

A venous malformation was diagnosed in 69,6% (n=195/280) of patients, and 83 of them had elevated D-dimer levels; the sensitivity of D-dimer dosage was 42.6% [95%CI: 35.6%–49.5%]. Among the 85 patients without venous malformation, D-dimer levels were elevated only in 3 patients; the specificity of the dosage was 96.5% [95%CI: 92.5%–100%].

Elevated D-dimer level is highly specific for venous malformations (pure, combined or syndromic), and therefore, this easy and cheap biomarker test should become part of the clinical evaluation of vascular anomalies. It can detect hidden venous malformations and help differentiate glomuvenous malformation (normal D-dimer levels) from other multifocal venous lesions. Elevated D-dimer level also differentiates a venous malformation from a lymphatic malformation. Moreover, slow-flow Klippel-Trenaunay syndrome (capillaro-lymphatico-venous malformation with limb hypertrophy) can be distinguished from fast-flow Parkes Weber syndrome (capillary malformation with underlying multiple microfistulas and limb hypertrophy). For these reasons, D-dimer level measurement is a useful complementary tool for diagnosing vascular anomalies in everyday practice.