What is a Port Wine Stain?
Port Wine Stain (PWS) vascular capillary malformation birthmarks vary from light pink to deep purple, caused by an overabundance of capillaries just beneath the surface of the skin. . The marks can be minimal to very extensive, covering 50% or more of the body. It is thought that a PWS develops within the first 2-8 weeks of gestation. One million Americans have a PWS with varying degrees of involvement. Left untreated, a PWS can thicken and become nodular, leading to complications including bleeding. However, many treatments are now available, and improvements in understanding are occurring rapidly.
Some of the options for PWS are: laser treatment, cosmetic cover-up; and excision utilizing tissue expansion. Laser treatment technologies include various pulsed dye lasers (including sclerolaser and v-beam) as well as pulsed light sources (photo-derm) and green light lasers, coherent, and versa pulse. Depending on the age of the patient and the location and extent of the treatment area, the laser can be used in an office setting with or without anesthesia.
How does a Port Wine Stain Result in Sturge-Weber Syndrome?
The most apparent indication of Sturge-Weber Syndrome (SWS) is a facial birthmark or Port Wine Stain (PWS). The PWS is due to an overabundance of capillaries just beneath the surface of the involved areas. When the PWS covers the first and second trigeminal nerve distribution (the eye and forehead region of the face), Sturge-Weber Syndrome (SWS) must be suspected. In individuals with dark pigmentation, the PWS may be difficult to recognize. However, a Port Wine Stain is not the only indicator of SWS and this syndrome can exist without the presence of a detectable Port Wine Stain. SWS consists of three basic manifestations involving a PWS on the face, eye, and the brain.
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