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Vascular Birthmarks

Overview

The term 'vascular birthmark' includes hemangiomas and malformations. It is estimated that approximately 10% of births have a vascular birthmark – that’s about 400,000 children per year in the United States alone. Of these, approximately 30% - nearly 120,000 children born each year – will have a vascular birthmark that should be evaluated by a specialist. While hemangiomas and malformations are related, and often confused, they are very different in their natural history and in the options for treatment.

"Leave it alone, it will go away" is commonly the advice given to parents of a child with a vascular birthmark. This approach is no longer universally acceptable because it does not allow for the major diagnostic differences and therapeutic options for the different types of lesions. We are at an exciting time in medical history when our knowledge, technological advances and clinical experience have come together to effect changes in the management of these birthmarks.

The emotional and physical consequences suffered by families and patients, some of whom have waited decades for their lesions to "go away", are no longer necessary.

The Hemangioma Treatment Foundation is here to give those afflicted with these birthmarks hope for a better life.

Hemangiomas

These are the most common benign tumors of infancy. They affect girls five times more often than boys and occur in light skinned people more often than those of darker complexion. They occur on the face or head and neck area in over 80% of cases. Most are not present at birth and become apparent within the first month of life. The current thought is that hemangiomas develop from cells that cross from the placenta into the baby and become lodged and grow.

Hemangiomas that are flat and involve the upper levels of the skin are called superficial. Those that are deep in the skin and appear as a lump are called deep. When a hemangioma has both a deep and a superficial component it is called compound. The terms cavernous hemangioma, strawberry hemangioma, stork-bite, angel kiss, and other historical terms should all be dropped. They don’t describe the birthmark in a way that is meaningful.

Once they appear they proliferate, or grow, for up to 18 months. Most don't grow past 6-8 months. Then they begin a slow process of regression or involution. This period of involution is quite variable, lasting over ten years in some patients. The degree to which the birthmark involutes is also variable. Most doctors give the advice that the birthmark will "go away" if you "leave it alone" for a long enough period of time. This is what has been taught for decades and is no longer held to be universally accurate. Yes, all hemangiomas regress to some degree but the majority, however, do not involute to a cosmetically acceptable level. In general, more than half of patients with a facial vascular lesion who wait more than 5 years will end up having some surgical or laser intervention because the result is not acceptable.

Our philosophy is, "If we can get a result now that is at least as good as if we waited 5 years then why not do it now?" The time can be used for touch up procedures, scar maturation, etc. A very important element to take into account is the child’s developmental stage. Children develop a sense of ‘self’ around 2 to 3 years of age. Around that time they develop the ability to compare their bodies to others’. They start to notice that mommy does not have the red thing on her nose or that little brother’s cheek looks different. Of course, other 3 year olds without birthmarks are learning the same. By age 5 most children are getting close to starting school where the social pressures of appearance and body differences become more acute. So, in keeping with our early intervention philosophy, we try to get as much done as is reasonable by age 3 and certainly by age 5 to try to avoid the psycho-social effects.

In some cases, hemangiomas can be life threatening or functionally impairing by interfering with vision, breathing, hearing or eating. Very large hemangiomas can cause problems by trapping cells that aid in blood clotting (Kassabach-Merritt phenomenon). Internal hemangiomas involving the abdominal organs can be very problematic as well. When a child has multiple external lesions and/or very large ones a search for internal hemangiomas should be done. There is no exact number but, in general, if there are more than 6-7 external ones consideration should be given to looking for internal ones. Airway hemangiomas most typically are found just below the vocal cords. They can be life-threatening and present with noisy breathing. Aggressive treatment with steroids and/or various lasers is indicated.

Vascular Malformations

Vascular malformations include a wide variety of lesions which are very different from hemangiomas. They are true developmental abnormalities of the vessels involved so they are always present at birth, even though they may not be apparent until later. They always grow over the lifetime of the individual and never regress. Some grow very slowly, others rapidly but they all get worse over time to some degree. The malformations are classified by the vessels involved. So, there are venous malformations, lymphatic malformations, arterial malformations or combinations (such as arterio-venous malformations).

Port Wine Stains (PWS) are a common type of malformation. They are technically a venular (small veins) malformation but the term port wine stain is so ingrained in our language, and the birthmark is not easily confused, that it is acceptable to keep this term. They are quite common, occurring in about 0.3% of the population with an equal distribution among males and females. Like all malformations, they are 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 eyelids. These 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. 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.
 

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