.

A Port Wine Stain is always present at birth but the appearance evolves over the first few weeks of life as the pigmentation increases. Approximately .3-. 5% of all infants will be born with a PWS. There is equality among male and female children.

The development of a PWS is said to be a result of a deficiency or absence of the nerve supply to the affected areas blood supply The nerves control the size and diameter of the blood vessels. An abnormality in the nerves results in a dilation of the blood vessels. The number of blood vessels is usually normal in the affected area. Since only the size is affected, more blood can flow through the dilated vessel. Since these vessels are close to the surface of the skin the skin appears pink or purple. The faster the flow of blood through the vessels the darker the stain.

PWS follow a progression. From birth they are flat, and most often light pink. As the vessels continue to enlarge and thicken the lesion will darken. In the teens it can be red and by age 30 many are purple. The skin can become lumpy. The growth of these lesions is varied from person to person. Some do not darken until 50-60 years of age. However it is important to note that all PWS will eventually darken, thicken and form irregularities to the skin texture.

The development of a PWS is said to be a result of a deficiency or absence of the nerve supply to the affected areas blood supply The nerves control the size and diameter of the blood vessels. An abnormality in the nerves results in a dilation of the blood vessels. The number of blood vessels is usually normal in the affected area. Since only the size is affected, more blood can flow through the dilated vessel. Since these vessels are close to the surface of the skin the skin appears pink or purple. The faster the flow of blood through the vessels the darker the stain.

PWS follow a progression. From birth they are flat, and most often light pink. As the vessels continue to enlarge and thicken the lesion will darken. In the teens it can be red and by age 30 many are purple. The skin can become lumpy. The growth of these lesions is varied from person to person. Some do not darken until 50-60 years of age. However it is important to note that all PWS will eventually darken, thicken and form irregularities to the skin texture.

PWS may cause complications when near the eye. They can create pressure to the blood vessels of the eye. If the PWS is near the eye evaluation by an ophthalmologist is essential. Early diagnosis and treatment can prevent blindness.

Since Port Wine Stains are considered superficial (not deep in the skin) lesions the best treatment course is laser. Rarely should surgical removal be considered and only in older patients. Port wine stains need multiple laser treatments. The Flash-Lamp Pulsed Dye laser is the recommended laser. Statistics show that 15-20% will respond and completely resolve after completion of laser. 10-125 will only lighten slightly. The deeper the lesion the less likely for a good response since the laser wont penetrate the deep components of the PWS. It is best to avoid continuous wave lasers like the Argon laser since the thermal reaction in the skin is high and it is difficult to prevent scarring. There are a few surgeons with extensive experience in the use of Argon lasers that have documented successful results with the Argon laser.

Once the lesion presents with an irregular skin texture the copper vapor or Nd: Yag laser is recommended.

The interval between laser treatments is still controversial. In some patients the PWS will continue to fade for 6 months - 1 year with a single treatment. But yearly treatments are time consuming and many patients want quicker results. Many physicians use 2-6 month intervals. The lesion has time to fade but the total treatment course is shortened.

Even with treatment many PWS reoccur after 5-10 years. This occurs because the remaining vessels continue to dilate since the nerve supply is still deficient. The laser does not treat the nerve supply. Touch up treatments can be performed through out life.

Some organizations question treatment since they can reoccur. This of course is a personal decision based on the cosmetic effects and psychological effects of facial deformity. Early laser prevents the irregularities to the skin texture minimizing the long-term deformity. It must be a personal decision when to begin and start treatment. Many physicians are treating as young as 6 months.

It must be noted that Port Wine Stains are associated with several complicated diseases, Sturge Webber, and Klippel Trenaunay Syndrome are just two. NOVA encourages all patients to have an evaluation by an experienced physician. A complete list of treating facilities and other support services is posted on our web site resource list.

References

Waner, M., Suen, J., Hemangiomas and Vascular Malformations of the Head and Neck, 1999 Wiley-Liss New York .

This article was originally written in 2003.

The article was updated in June 2006.

NOVA PROVIDES THIS INFORMATION TO FAMILIES AS A RESOURCE. IT IS NOT INTENDED TO ENGAGE IN THE PRACTICE OF MEDICINE OR TO REPLACE THE PHYSICIAN. NOVA DOES NOT CLAIM TO HAVE MEDICAL KNOWLEDGE. NOVA DOES NOT ENDORSE ANY PARTICULAR PHYSICIAN, TREATING FACILITY OR TREATMENT PROTOCAL. IN ALL CASES NOVA AND ITS BOARD OF DIRECTORS RECOMMENDS THAT YOU SEEK THE OPINION OF A PHYSICIAN EXPERIENCED IN THE MANAGEMENT OF HEMANGIOMAS AND VASCULAR MALFORMATIONS. 

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