- Vascular Malformations are very different from hemangiomas
- They are developmental abnormalities of the vessels
- Always present at birth, even though they may not be apparent until later
- Vascular malformations always grow over the lifetime of the individual and do not regress
- Classified by the vessels involved:
- Venous malformations
- Lymphatic malformations
- Arterial malformations
- Combinations (ie. arterio-venous malformations)
Port Wine Stains (PWS) are a common type of malformation. Technically a venular (small veins) malformation, but the term port wine stain is so ingrained in our language, and the birthmark is so unique, that it has become acceptable to use this term.
- They are quite common, occurring in about 0.3% of the population
- Equal distribution among males and females
- Present at birth and get worse, to some degree, over time.
- They can be pale pink to dark purple in color and their cause is thought to be related to a problem in the nerve regulation of the vessels – though this is not proven.
Capillary Malformations are flat, pink-red birthmarks that are most common on the face and neck. Most commonly these are the lesions associated with Sturge-Weber syndrome. These malformations should not be confused with the very common staining seen in newborns on the forehead, nape of neck and eyelid which very predictably fade in the first year while capillary malformations do not.
Venous malformations can be deep, superficial, diffuse or localized. They commonly appear as bluish, spongy masses that can be compressed easily. When the child is lying down or crying, the lesion fills up with blood and the mass becomes fuller. Occasionally, hard lumps can be felt in the malformation, these are calcified nodules.
- The most common areas affected are the cheek, floor of mouth and extremities.
- Pain and swelling are common complaints
- Klippel-Trenaunay Syndrome (KTS) is a venous-lymphatic malformation of the extremities, most commonly of the legs.
- Venous malformations can get worse during puberty and pregnancy or as a result of infection and trauma.
- Learn more about Venous malformation in this quick video
Lymphatic malformations are most commonly found in the neck, cheek or mouth or in association with venous malformations in KTS. In the neck, they are often called ‘cystic hygroma’ but this term should be discontinued. These lesions are made up of expanded lymphatic channels which can be quite large and typically get bigger due to infection, such as a cold, and then go back to their regular size once the infection goes away. There are two major types of lymphatic malformations – macrocystic and microcystic. Macrocystic lesions have large (macro) pockets and microcystic have very small lesions that invade the tissues. It is important to tell these apart for treatment.
Arterio-venous malformations are typically found in the brain; the second most common site is the head/neck most commonly in the mid-face. They are often pulsatile and firm and, like other malformations, always present at birth.