Sunday, July 15, 2007

Vascular Birthmarks

Macular stains. Also called salmon patches, angel kisses, or stork bites, these faint red marks are the most common type of vascular birthmark. They're often on the forehead or eyelids, the back of the neck, or on the nose, upper lip, or on the back of the head. They may be more noticeable when the baby cries. Most often they fade on their own by the time a child is 1 to 2 years old, although some last into adulthood.

Hemangiomas. Hemangiomas are classified as superficial when they appear on the surface of the skin ("strawberry marks") and cavernous when found deeper below the skin's surface. They can be slightly raised and bright red and sometimes aren't visible until a few days or weeks after a baby is born. Cavernous, deep hemangiomas may be bluish because they involve blood vessels in deeper layers of the skin. Hemangiomas grow rapidly during the first 6 months or so of life, but usually shrink back and disappear by the time a child is 5 to 9 years old. Some, particularly larger ones, may leave a scar as they regress that can be corrected by minor plastic surgery. Most are on the head or neck, although they can be anywhere on the body, and can cause complications if their location interferes with sight, feeding, breathing, or other body functions.


Approximately thirty percent of all hemangiomas are visible at birth. The remaining seventy percent become visible within one to four weeks after birth. They affect approximately one in 10 to 20 Caucasian children, with a 3:1 predilection for the female sex. The incidence in non-Caucasian infants is lower. Hemangiomas are endothelial neoplasms. To date, there is no universally accepted model for the etiology of hemangiomas. There is general consensus on the importance of endothelial cells, but the source of the endothelial cells is speculative and there is no consensus on the possible mechanisms by which the hemangioma endothelium interacts with surrounding cells. There is a scientific debate on whether they are of mutational or placental origin or fit in a developmental field disruption. In the past hemangiomas were once thought to be due to maternal behavior or thoughts during pregnancy. The term vascular birthmark itself implies a connection between birth and a vascular lesion. The term strawberry nevus indicates that the mother's intake of red fruits (strawberries) was once thought to cause birthmarks. It is important for the family to remember that the cause of hemangiomas has not been determined, and neither parent should bear guilt over the occurrence or appearance of one of these birthmarks. Approximately 83% percent occur on the head and neck area. The remaining 17% appear throughout the rest of the body (both externally and internally).


At birth, a precursor lesion may be present as an erythematous patch or a telangiectasia. Within weeks the lesion expands rapidly. Hemangiomas can grow for up to 18 months and then begin a long slow regression known as involution. This involution can last from 3- 10 years. This dramatic/fast growth of a hemangioma may seem alarming, but usually no treatment is indicated. This is because this rapid growth phase is followed by a gradual involution (regression) in the following years. At the age of 5 years, 50 percent of the hemangiomas have involuted, and at the age of 9 years 90 percent have. The ones that do need treatment are the ones that ulcerate, obstruct the airway, or cause visual deficits or cardiovascular symptoms. Current treatment may include intralesional or systemic corticosteroid treatment, systemic interferon treatment, local bleomycin treatment, and surgery. A hemangioma usually occurs as a solitary, superficial lesion in the head and neck area, but it can be present anywhere in or on the body. Size can vary greatly, from nodular lesions several millimeters in diameter to plaque-like tumors covering the entire face or a quadrant of the body. In selected cases, however, prompt intervention is needed. These cases involve hemangiomas that ulcerate, obstruct the airway, or cause visual deficits or cardiovascular symptoms. Early intervention has been shown to reduce the need for corrective surgery after "involution" has occurred; or to, at least, minimize extensive corrective surgeries in the future. Psycho-social scarring which occurs when a child has been forced to live with a facial deformity until "involution" has been completed can be avoided by early, aggressive intervention.



Vascular Malformations differ from hemangiomas in several crucial ways. Most distinctively, vascular malformations are present at birth, grow commensurately with the body, and never show signs of spontaneous involution. Photo credit.


Port wine stains are Venual Malformations. They are always present at birth and can range from pale pink to dark purple in color. These are discolorations that look like wine was spilled on an area of the body, most often on the face, neck, arms, or legs. Port wine stains can be any size, but grow only as the child grows. They tend to darken over time and can thicken and feel like pebbles in midlife adulthood unless treated. They never go away on their own. Ones near the eye must be assessed for possible complications involving the eye. In the past these lesions were erroneously called "capillary hemangiomas." Port Wine Stains occur in 0.3% of births and occur equally among males and females. The cause has been associated with a deficiency or absence in the nerve supply to the blood vessels of the affected area. These nerves control the diameter of the blood vessels. If the nerves are absent or defective, the vessels will continue to dilate and blood will pool or collect in the affected area. The result will be a visible birth mark. This is important to know because laser therapy which is used to remove a port wine stain will only be temporary. Since the deficiency is in the nervous system, in time the blood will repool in the affected area and the birthmark will once again appear. Once a Port Wine Stain is lasered, it is important at the first sign of reoccurence to have one or two treatments to keep it faded. The individual will have to have maintenance laser treatments for life. Because Port Wine Stains can be progressive, treatment should be done early to prevent cobbling of the skin and thickening and darkening of the stain. These lesions vary from low-grade to high-grade, pale to dark. Low-grade progress at a slower rate than high-grade. Rarely capillary malformations are clues to the presence of other defects such as Sturge-Weber Syndrome or Klippel-Trenaunay Syndrome.

Venous Malformation is an abnormality of the larger, deep veins and is often mistakenly called a "hemangioma." A good history will reveal whether or not the lesion is a hemangioma or vascular malformation. A venous malformation can be deep or superficial, localized or diffused. The closer the vessels are to the surface, the deeper the color to the eye. A very deep lesion will have no color but will show a protruding mass. The jaw, cheek, tongue and lips are common sites for a venous malformation. These lesions are soft to the touch, the color disappears and empties as the lesion is compressed. When the child cries or is lying down, the lesion expand, the vessels fill and the color becomes more intense. The natural history is a slow-steady enlargement. Regardless of how small it is upon detection, it will grow. Certain things can cause them to grow more rapidly such as serious sickness, trauma, infection, hormone changes (puberty, pregnancy, menopause). Partial removal is not recommended since these lesions will just grow back.


Arteriovenous Malformations are always present at birth but are usually not noticed until later in life. Sometimes they do not appear until adulthood. Defective blood flow has been associated with these lesions. As the lesion ages, the vessels enlarge and thicken to compensate for the increased blood supply. There are two grades: low and high. Low grade grow slowly with the child and high grade expand rapidly, growing faster than the child until the lesion may eventually become life threatening. An AV malformation is a firm mass. Common sites are the lips and other head and neck areas. Mixed malformations include a combination of two or more vascular lesions.


Lymphatic Malformations used to be called cystic hygroma, hemangiolymphangioma, or lymphangiomas. Lymphatic malformations are composed of dilated lymphatic channels. The lymphatics serve as a collection and transfer system for tiisue fluids. When something disturbs this system, a lymphatic malformation is formed. The excess fluid accumulates and the affected lymphatic vessels enlarge and you see a mass. If the lymph vessels in the face are affected, the face swells because the normal active transport mechanism has been disturbed. These lesions can occur anywhere but are common in the head and neck area. These lesions may be superficial or deep (superficial ones are seen in the mouth area and look like frogs eggs) . These lesions increase or grow with the individual. They may enlarge following an upper respiratory infection.


References:
Hemangiomas and Vascular Malformations in Infants and Children: A Classification Based on Endothelial Characteristics; The Pathogenesis of Hemangiomas: A Review; Bauland, Constantijn G. M.D.; van Steensel, Maurice A. M. M.D., Ph.D.; Steijlen, Peter M. M.D., Ph.D.; Rieu, Paul N. M. A. M.D., Ph.D.; Spauwen, Paul H. M. M.D., Ph.D.; Plastic and Reconstructive Surgery:Volume 117(2)February 2006
Progress to ward Understanding Vascular Malformations [Special Topic]; Breugem, Corstiaan C. M.B.Ch.B.; van der Horst, Chantal M. A. M. M.D., Ph.D., and; Hennekam, Raoul C. M. M.D., Ph.D.; Plastic and Reconstructive Surgery:Volume 107(6)May 2001
Vascular Birthmarks: Hemangioma and Malformations. by Mulliken, John B. MD; Young, Anthony E. MD; Textbook--W.B. Saunder, Philadelphia, PA, 1988.
Hemangiomas and Vascular Malformations of the Head and Neck edited by Milton Waner, MD and James Suen, MD--Wiley-Liss, NY 1999

4 comments:

Just a Crazy Woman said...

I was recently diagnosed with a venous malformation by Dr. Suen. Until I saw him, I was confused on what it was that I had. I was doing a casual google on malformations and your blog came up. Just thought I would stop by and say hi :)

rlbates said...

Is that our Dr James Suen at UAMS? You are in good hands.

Just a Crazy Woman said...

The one and only :) I'm actually having minor laser surgery next week. He's so nice and I'm SO glad to have him right here in Little Rock.

iBeth said...

In 1960 I was born with a stawberry mark on my left arm that disappeared by the time I was two. Mother worried she caused it because she ate so many strawberries during pregnancy. So when I came across a post in my LEWIS research from Crawford County, Arkansas, I was surprised to read your blog about the subject. Thought you would be interested to know I fit all your criteria. ;-D.