A port-wine stain is a congenital vascular condition consisting of dilated blood vessels in the skin. The affected vessels expand over the course of life. As a result, a port-wine stain can eventually develop a bumpy appearance. This vascular condition occurs in approximately 0.3-0.6% of newborns. Boys and girls have port-wine stains just as often. Such a birthmark can sometimes have significant consequences for the (psycho)social development of children and adults. Sometimes a port-wine stain can bleed spontaneously, often as a result of a so-called granuloma pyogenicum (a wart-like growth that bleeds when touched). Early treatment of a port-wine stain—if successful—could potentially reduce the psychosocial consequences.
Definition:
"Port-wine stain" is often referred to in English literature as "port-wine stain." Other names are also used: nevus flammeus or hemangioma. It is actually a disorder of the capillaries, present from birth and whose surface area grows with the child. A hemangioma is a vascular disorder that is rarely present at birth, grows rapidly, and disappears after the first year of life. Stork bites are disorders of the skin's capillaries, located in specific areas: the center of the forehead or the back of the neck, or on the eyelids, which become apparent during the first year of life.
The area affected by a "port-wine stain" can be thicker/raised than the healthy surrounding area from an early age: this is called hypertrophy.
A port-wine stain on the face can be accompanied by abnormal vessels in the eye on the same side. If abnormal vessels are also found on the surface of the brain on the affected side, this combination is called Sturge-Weber syndrome, after the first physicians who wrote about it.
Congenital
vascular disorders arise from an abnormality in the "formation" of the blood vessels. This can include capillaries, arteries, veins, and lymphatic vessels. Sometimes, combinations of affected vessels are also seen (Klippel-Trenaunay syndrome). When examining the vessels of a capillary malformation (port-wine stain) under a microscope, you see dilated blood vessels with a normal inner cell layer (endothelium) in the part of the skin called the dermis. When examining these abnormal vessels, no normal nerve endings are found in the vessel wall, meaning these vessels do not receive the stimulus to contract in response to cold, for example.
Port-wine stains occur more frequently in some families than in the general population. This suggests a hereditary factor. More and more evidence is being found for this, although this doesn't explain every port-wine stain. In a study of 280 consecutive patients, we found that 55 families had multiple affected individuals.
Psychology:
It's generally said that someone who looks attractive has an easier time in life than someone who looks less attractive. Renowned psychologists have found that people tend to quickly assume that someone who looks attractive has a good figure. Psychological research on adults with a port-wine stain has revealed nothing abnormal. Nevertheless, having a port-wine stain is often perceived as a burden. In this age where everything is readily available, a visible abnormality is considered unacceptable.
Therapy:
Many methods have been developed to treat a port-wine stain. In the past, X-rays were used. This could lead to skin cancer many years after treatment and, moreover, had no effect on the visibility of the stain. Surgical treatment often resulted in unacceptable scarring. Sometimes, skin tattooing can be a solution, especially for small stains.
Initially, the argon laser seemed to offer a good treatment for port-wine stains. However, this laser led to scarring and therefore could not be used, especially in children. In 1985, the flash-lamp-pumped color laser was developed. Because of its specific effect on blood vessels, it was more suitable for treating blood vessels in the skin. The effects are explained as follows: because the hemoglobin (red) in the blood absorbs the colored laser light, heat is generated in the "diseased" blood vessel of the port-wine stain. This blood vessel then closes/breaks. Healing then occurs through ingrowth of normal blood vessels. A subsequent treatment can be treated a layer deeper than the previous one, and so on. Six to eight weeks should elapse between treatments of the same area, as the redness caused by the inflammatory reaction after the laser treatment only then has subsided. The epidermis remains intact throughout this entire process.
A laser pulse on the skin is not painless. It's similar to the pain caused by a rubber band snapping against the skin. The pain is intense but brief. Sometimes hundreds of pulses need to be administered during a laser treatment. Initially, we try to prevent the pain by applying an anesthetic ointment to the area to be treated: EMLA cream. This cream should be applied under plastic, about an hour before the treatment. Sometimes the area to be treated is also numbed by an injection around the nerve that supplies sensation to that area. It's clear that in children, treatment can sometimes only be performed under general anesthesia, especially since the treatment needs to be repeated several times. During the laser treatment, the stain is cooled with gauze soaked in ice water.
In practice, you'll see a blue-gray discoloration of the stain immediately after the laser treatment. This discoloration lasts for a week to ten days, after which redness appears. Only after two months will you see a lightening (if the port-wine stain responds well). The treated area is no longer painful after the treatment; sometimes people say it feels like a sunburn.
For years, there was debate about whether laser treatment of a port-wine stain at a young age would yield better results than at an older age. After all, the stain is still relatively small in a child, the skin is thinner than in an adult, and the blood vessels are more easily accessible. Extensive research within the plastic surgery department of the AMC has shown that, unfortunately, treating port-wine stains at a young age does not yield better results than treatment in adulthood. This doesn't mean you can't treat at a young age. We often treat at a young age. It's simply not the case that you've lost the best chance of successfully lightening the stain by starting treatment at an older age.
In the research, we also investigated whether it was possible to predict which port-wine stains would respond well to treatment and which would not. Unfortunately, the success rate cannot be predicted in advance. The greatest lightening is achieved during the first few treatments. After that, the rate of lightening gradually decreases. Up to 25 treatments, each with a minimum six-week break between treatments, can achieve lightening. It's not always worthwhile to continue for this long. Unfortunately, some port-wine stains don't respond to dye laser therapy at all. These may be caused by deeper blood vessels. The penetration depth of the dye laser light is only 1.2 millimeters. We know that dilated capillaries sometimes lie much deeper than 1.2 mm. |
