Classification and medical therapy of congenital vascular malformations

Congenital vascular malformations are the result of an abnormal development during embryogenesis. It is important to distinguish these anomalies from vascular tumors, e.g. infantile hemangioma, as they are two completely different types of lesion with different clinical courses and treatment options. With the modified Hamburg classification vascular malformations are categorized based on the predominant vessel involved (e.g. arterial, arteriovenous, venous, capillary, lymphatic or combined). Additionally, each lesion can be defined as extratruncular or truncular based on the embryological origin of the defect. This is an important distinction as it provides an indication of prognosis and therapeutic options. The hemodynamic characteristics of vascular malformations are defined as low-flow or high-flow according to the International Society for the Study of Vascular Anomalies (ISSVA) classification. In contrast to infantile vascular tumors (hemangiomas) there are no medicinal options to treat vascular malformations. The focus of conservative management is relief of symptoms and prevention of complications. In most cases compression therapy is the single most important measure. © 2014 Springer-Verlag.