Abstract
The presence of a vascular anomaly suggests that capillaries, veins, arteries, and/or lymphatic vessels have demonstrated abnormal development and growth. Often dilated and misshaped, these vessels augment normal flow of blood and lymphatic fluids that increases the overall risk to develop intralesional thrombosis. Abnormal endothelial and lymphoendothelial cells activate hemostasis and hyperfibrinolytic pathways through poorly understood mechanisms, which contribute to the development of localized intravascular coagulopathy. Vascular malformations, tumors, and complex combined syndromes demonstrate varying degrees of prothrombotic activity and consumptive coagulopathy depending on the vessels involved and the pattern and extent of abnormal growth. The clinical impact of venous thromboembolism in pediatric vascular anomalies varies from painful syndromes that disrupt quality of life to life-threatening embolic disease. There remains little literature on the study, evaluation, and treatment of thrombosis in pediatric vascular anomalies. However, there have been great advances in our ability to image complex lesions, to surgically and interventionally augment disease, and to provide enhanced supportive care including patient education, compression therapy, and strategic use of anticoagulation.
Highlights
Pediatric vascular anomalies affect roughly 5% of the population and consist of abnormally developed and misshaped capillaries, veins, arteries, and/or lymphatic vessels
The clinical impact of thrombosis in pediatric vascular anomalies ranges from mild episodic pain associated with small superficial thrombi to life-threatening sequela of embolic thrombi formed via connections to deeper ectatic vessels [1]
A multidisciplinary approach is recommended to treat all vascular anomalies, and the morbidity and mortality associated with thrombotic complications dictates the need to include a physician familiar with pediatric hematology as part of this multidisciplinary team
Summary
Pediatric vascular anomalies affect roughly 5% of the population and consist of abnormally developed and misshaped capillaries, veins, arteries, and/or lymphatic vessels. They demonstrate irregular flow of blood and lymphatic fluids, consist of malformed and overly activated endothelial walls, and can carry an increased risk for intralesional and/or systemic thrombosis. The chronic formation and lysis of thrombi can progressively worsen pain and increase lesion size as the vessel walls lose elasticity These complications can substantially limit daily activities and the negative impact on a child’s quality of life directly contributes to a family’s decision to pursue aggressive therapeutic interventions. The aim of the current article is to summarize the published clinical experience with and treatment of VTE in pediatric vascular anomalies, highlighting those associated with VMs
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