Clinical Features and Evaluation of Superficial & Deep Capillary Malformation (CM)



A distinct classification of congenital vascular anomalies is necessary because there is a broad list of lesions with variability in signs, symptoms, and clinical behavior. Vascular malformations may have any combination of capillary, venous, arterial, and lymphatic components, with or without fistulae (Table 20.1). There are truncular and extratruncular forms [1–3]. Often one can find a combination of both. An irregular structure of the vessel wall, position anomaly as a result of an irregular origin and course, or a persistent fetal vessel can be the reason for this. The vascular system results in a differentiation from mesodermal steam cells into Hemangioblasts [4]. They later differentiate to the primitive vascular system – the Vasculogenesis. This has to be discriminate from the Angiogenesis, the formation of vessels from existing vascular system. In which embryological phase the defect happens determines the kind of vascular malformation – the later in the phase after differentiation to the vascular system the more truncular malformations happens. Typical example for this are congenital heart defects. The more in the vasculogenesis phase or before the differentiation to hemangioblasts the more extratruncular malformations or combined other mesenchymal malformations happens. This explains the findings in Phakomatosis e.g. in overgrowth syndromes [5]. Furthermore this explains why the use of syndrome names make sense: They are typical combinations of defects in different organ systems. Capillary Vascular Malformations results from an early defect in the vasculogenesis. This means that Capillary Vascular Malformations mostly are part of a mixed Vascular Malformation. Furthermore this explains why Capillary Vascular Malformation never can heal but have progress. For several forms there is a tumor like growing behavior, the so called Hamartoma. In some cases, the vascular malformation remains preformed and latent and grows as a result of a lesion, trauma, or a hormonal effect. This can happen during adolescence or even during adulthood. The most vascular anomalies are well known with their eponymous conditions [6].


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© Springer-Verlag Berlin Heidelberg 2017

Authors and Affiliations

  1. 1.Wissenschaft und Forschung, Lasermedizin, Elisabeth KlinikBerlinGermany

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