Accurate epidemiological analysis of vascular anomalies is extremely difficult to afford for several reasons: – First, majority of vascular malformations are indolent, unnoticeable, of minimal size, and harboring no progression or disappearing spontaneously. This group of patients will never report their « problems» to any physician and therefore the anomaly will not be registered. Typical examples are nuchal or frontal capillary malformations in neonates or small lymphatic malformations in the abdominal cavity. – Second, diagnosis of vascular anomalies remains a challenge for too many specialists. Tremendous confusion exists with regard to the classification and treatment of vascular lesions. For a long time, vascular anomalies have been categorized using inconsistent terminology, such as “hemangioma”, and a significant number of patients receive ineffective and potentially harmful treatments based on this misclassification. A first version of the International Society for the Study of Vascular Anomalies (ISSVA) classification was adopted in 1996 and allowed to classify vascular anomalies into two groups (tumors and malformations). Many subsequent revisions led to the 2014 ISSVA classification system, based on clinical, histological, histochemical, and radiological findings. With the improvement of molecular and genetic understanding, the updated classification ISSVA 2018 [1] incorporated the causative genes involved in many of these lesions, opening the era of precision medicine. – Third, there is an urgent need to establish vascular anomalies registry (VAR), an organization for the systematic collection, storage,analysis, interpretation, and reporting of data on subjects with vascular tumors and malformations. These information may help to increase the knowledge on epidemiology and natural history of vascular anomalies, to design new clinical trials, and to more easily select cohorts for targeted therapies. Efforts should be done by the National Health Care provider system to promote these registries. Some examples of fruitful national and international registries are those developed for liver hemangioma, PHACES syndrome, multifocal lymphangioendotheliomatosis with thrombocytopenia, or generalized lymphatic anomalies and Gorham-Stout disease. In order to accurately evaluate epidemiology of vascular anomalies, we will follow the current ISSVA classification and nomenclature.
Universidad Autonoma of MadridDepartment of Pediatrics
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Seront, E., Lopez Gutierrez, J. C., & Vikkula, M. (2026). Epidemiology of Vascular Anomalies. In Mariella (Maria) Catalano, Gerit-Holger Schernthaner, Katalin Farkas, Grigoris Gerotziafas, Agata Stanek, et.al. (eds) (ed.), VAS European Book on Angiology/Vascular Medicine (p. p. 1091-1097). Springer. https://doi.org/10.1007/978-3-031-82232-2_96