Port Wine Stain: Can This Birthmark Be Treated?

For the most part, birthmarks are harmless. Although they appear on the bodies of many babies, birthmarks often fade away in a few weeks. In rare instances, they disappear after a year. There are occasions, however, when birthmarks are symptoms of something more concerning. With a port wine stain, that’s the scenario parents have to deal with.

What Is a Port Wine Stain?

A port wine stain is a rare congenital disorder involving the abnormal development of blood vessels. It is usually part of a syndrome (a collection of signs and symptoms) known as Klippel-Trenaunay syndrome and involves port wine stains over areas of the body. However, it is called Sturge-Weber syndrome when it appears on a baby’s face. Aside from the visible port wine stains, these syndromes may also involve abnormal development of soft tissues, bones, and the lymphatic system.

It is called port wine stain because it is characterized by a red birthmark that resembles a stain caused by port wine. An overgrowth of tissues and bones, as well as vein malformations with or without lymphatic abnormalities, are also present, especially in the syndromes they come with.

Port Wine Stain Symptoms

A port wine stain is usually pink to reddish-purple and covers part of one of the baby’s legs. It can be on any portion of the skin and may grow darker or lighter as the baby grows. The birthmark is caused by extra tiny blood vessels or capillaries in the top layer of the skin. An increase in vascular profiles and ectasia also characterizes this condition.

Vein malformations in the form of swollen or twisted veins on the surface of the legs also appear. Other places where veins can appear include the arms, legs, abdomen, and pelvis.

An overgrowth of bones and soft tissue can begin in infancy and is usually found in just one leg. This can occur in an arm and, on rare occasions, in the trunk or face. A larger and longer extremity is the result of this overgrowth. A fusion of fingers or toes or having extra fingers or toes may also occur.

The lymphatic system that protects against infection and disease can be abnormal. An extra number of lymphatic vessels can be present that might not work properly. This can lead to swelling or leakage.

Klippel-Trenaunay syndrome can also lead to cataracts, glaucoma, hip dislocation at birth, and blood clotting problems.

Port Wine Stain Treatment

Since this birthmark does not fade away gradually, treatments have been sought to remove port wine stains. One hundred two patients aged one month to 66 years were given flashlamp-pumped dye laser treatment for their port wine stains from 1989 to 1994.

Out of those treated, 15.3% saw more than 90% of their lesions lightening while 65.3% had lightening from 50% to 90%. Poor response from 11% to 49% was registered by 17.8% of the subjects. Only 1.7% had less than 10% or no response.

While this response yielded positive results, the researchers noted that the port wine stain recurred at a rate approaching 50% between three to four years after treatment was completed.

Key Takeaways

Port wine stains are different from other birthmarks as they involve abnormal development of blood vessels.

Known as Klippel-Trenaunay syndrome when it appears everywhere but on the face, symptoms aside from port wine stains include an overgrowth of bones and soft tissue, vein malformations, and a possibly abnormal lymphatic system.

Sturge-Weber syndrome is what it is called when this associated birthmark appears on the face. This syndrome is also characterized by eye abnormalities like glaucoma and caused by somatic mosaic mutations disrupting vascular development.

Flashlamp-pumped dye laser treatment has proven effective in treating port wine stain. Unfortunately, research shows that it can recur between three to four years after treatment.

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