David Jeffery Seveland
Attacking all obstacles with a strong mind and heart.
Port Wine Stain
I was born on 8-25-06 with a Port Wine Stain that covers 95% of my body from the shoulders down and a very dark red patch at the base of my spine. At birth it presented itself as a "dark purple" birthmark that faded to a red color and then to a pink, which it stays most of the time. The Doctors thought I might have had an oxygen definciency. They took me to the nursery to verify that everything was OK and after 5 1/2 hours my mom finally got to hold me.
After about 5 different doctors looked me over, my parents were told that I had a "Port Wine Stain." The Doctor's also made sure to tell them that everything would be OK and that there were no major issues associated with the birthmark alone. This allowed everybody a big sigh of relief.We went to a Dermatologist for the first time on 9-6-06 to find out more about Port Wine Stains. She was great. My parents were told that I looked very good. It was a very extensive birthmark unlike one she had seen in a while. She also told them that the birthmark would not fade on it's own. The only way to remove it was Laser surgery. This needs to be done before the age of 4 for best results and an 85% chance of complete removal. That's only if it works. We were told to watch for the possibility of my limbs to stop growing. A port wine stain could cause a more fibrous, denser tissue to develop around the ends of the bones hence, stunting bone development. My mom watched me closely for this.
At about 2 1/2 months she noticed that my left leg was longer than my right one and that my left foot was bigger. After measuring me on her own she also found that my left arm and hand were bigger than the right. Since she was worried about the bones growth be altered, she called immediately. She was told to take me to the Pediatrician and get me measured. On November 7, 2006 I was diagnosed with Hemihypertrophy.
Th is is how David's hands and feet looked at birth. They were a very dark purple and looked like they belonged to someone else. The discoloration is due to the Port Wine Stain that is enhanced by acrocyanosis (over exaggeration to hot and cold).
The pictures below show David's birthmark on his chest and back. There are a few patches of white skin around his nipples, by his belly button, on his right shoulder and lower right side. The only other white patches are small patches found on the bottom of his feet and a small patch on the inside of his right thigh.
Description
Port Wine Stain
A port-wine stain or naevus flammeus is a vascular birthmark - a type of hemangioma - in which swollen blood vessels cause a reddish to purplish discoloration of the skin. They are so called for their color, resembling that of Port Wine. It is part of the family of disorders known as vascular malformations.
The tiny blood vessels (capillaries) in port wine stains remain wide (dilated). It is like a permanent localized blush. The reason why this occurs is thought to be due to a damaged or faulty nerve supply to the affected tiny blood vessels. The nerve impulses that make the blood vessels wider or narrower are lost. The tiny blood vessels then stay wide all the time. Apart from their appearance, no other symptoms or problems occur in most cases.
Background
Port-wine stains are present at birth and persist throughout life. They are irregularly shaped vascular malformations with prominent deep layer dermal blood vessels. Generally, the malformation is confined to the skin. Essentially, the deep vessels and capillaries are enlarged and dilated. There is no spontaneous significant regression of the vascular malformation. The area of skin affected grows in proportion to general growth. Where present, they generally appear on one side of the body with a sharp mid-line cut-off. The incidence of occurance is roughly 3 out of 1,000 people.
Port-wine stains occur most often on the face but can appear anywhere on the body. Early stains are usually flat and pink in appearance. As the child matures, the color may deepen to a dark red or purplish color. The skin generally has a more leathery or thickened surface. In adulthood, severe thickening of the lesion or the development of small lumps may occur causing a more unsightly appearance.
More than one-third of infants are born with a pink patch at the nape of the neck or on the eyelids (called an "angel's kiss," "salmon patch," or "stork bite"). These are minor blood vessel dilations and are not true port-wine stains. Those located on the eyelids and forehead fade slowly and eventually disappear. Those at the base of the neck are often permanent. A port wine stain is a localized blood vessel problem. Tiny blood vessels can normally narrow (constrict) or widen (dilate) depending on circumstances. This allows different amounts of blood to the skin surface. The more blood, the redder the skin. For example, normal skin goes pale when we are cold (blood vessels constrict) and goes bright red when we blush (blood vessels dilate). With PWS the body does not regulate how much blood is going into the skin surface therefore leaving a darker, permanent mark.
The presence of PWS can cause emotional and social problems for the affected person because of their cosmetic appearance. It may have a severe impact on the child's social, psychological and economic development.
About 1 in 10 babies born with a port wine stain on the face have problems of the eye or brain. (The port wine stain is then a 'marker' for a more serious problem.) Eye problems may develop if the port wine stain is next to the eye. Blood vessel abnormalities may also develop in the eye and cause glaucoma or other eye problems. If a child has a port wine stain next to an eye then an eye specialist will normally check the child regularly until they are adult. Brain abnormalities are an uncommon association with port wine stains of the face. This is due to extensive blood vessel abnormalities in the brain. If these brain abnormalities are found in association with Sturge-Weber syndrome then epilepsy and other problems may develop.
PWS may be one of a group of symptoms and signs, in which case it is considered to be part of a syndrome such as Sturge-Weber Syndrome, Klippel-Trenaunay-Weber Syndrome, Beckwith-Weidemann Syndrome, Bonnett-Bechaume-Blanc Syndrome, Cobb Syndrome, and Proteus Syndrome. Hypertrophy (increased tissue mass) of the stains may occasionally produce deformity and increasing disfigurement. Nevertheless, all birthmarks should be evaluated by the health care provider during a routine examination.
Diagnosis
A physician can usually diagnose a port wine stain based entirely upon the history and appearance. In unusual cases, a skin biopsy may be needed to confirm the diagnosis. Depending on the location of the birthmark and other associated symptoms, a physician may choose to order a measurement of intraocular pressure or X-ray of the skull.
Treatment
Treatment is for cosmetic, social, and psychological benefit. Depending on the birthmark and the setting, this may be very important, or not important at all. Many treatments have been tried for port-wine stains including freezing, surgery, radiation, and tattooing; port-wine stains can also be covered with cosmetics. Lasers have made the biggest impact on treatment, because they are the sole method of destroying the tiny blood vessels without significant damage to the overlying skin.
Treatment for port wine stains first requires performing a laser response test, which determines the efficacy and response of an individual’s tissue to the laser. Several energy levels are tested and evaluated approximately six weeks later.
The Candela flashlamp pulse dye laser, a yellow light laser, has been the most successful at destroying stains in infants and young children. The neodymium YAG laser is used to treat the nodules that may develop in some adult port-wine stains. Candela pulse dye laser treatment of port wine stains typically requires several sessions, usually anywhere from one and ten treatments. The average tends to run in the range of three to eight. Essentially, very superficial lesions will quickly clear and are more successfully treated. Deeper lesions clear less easily and require more treatments.
Prognosis
Treatment of infants with the flashlamp pumped dye laser generally produces marked improvement in appearance though complete disappearance is rare. Unfortunately, in a small percentage of cases (approximately 20%) there may be no improvement at all. Stains on the face respond better than those on the trunk, limbs, or middle of the body. Older stains may be more difficult to treat.
Possible Complications
Some Stains may cause deformity and increasing disfigurement. Persons with port-wine stains may have emotional and social problems related to their appearance. Port-wine Stains that involve the upper and lower eyelids may be associated with the development of glaucoma.
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