Venous malformation: risk of progression during childhood and adolescence

Venous malformations (VMs) can cause significant morbidity, particularly because they enlarge over time and become symptomatic. The purpose of this study was to determine the natural progression of VMs to facilitate patient counseling, gain insight into pathophysiology, and guide therapy. Our Vascular Anomalies Center database was reviewed for patients with cutaneous and soft-tissue VMs. Predictive variables were age, gender, location, pregnancy, and size. The outcome variable was natural progression of the malformation defined by expansion of the lesion or the onset/worsening of symptoms. The study included 614 patients. Children had a 26.1% risk of progression prior to adolescence, 74.9% before adulthood, and 93.2% over their lifetime. Progression was more likely in adolescence (60.9%) than in childhood (22.5%); the relative risk was 2.6 (95% confidence interval, 2.1-3.2) (P = 0.0001). Diffuse VMs progressed more often than localized lesions (P = 0.002); extremity and trunk VMs worsened more frequently than head/neck lesions (P = 0.03). VMs have a higher risk of progression in adolescents than in children; pubertal hormones may contribute to expansion. Because of their high rate of progression, early treatment of asymptomatic VMs should be considered.