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Port wine stain

Port wine stain or nevus flammeus is a rather darker birthmark - a type of hemangioma - that can be deep red to purple (or even black in African-American babies). It often affects the facial region. It has a more leathery or thickened surface than a stork bite and does not fade, but will require removal if disfiguring. Port wine stains on the face in the area of distribution of the trigeminal nerve (forehead and cheekbone on one side around the eye) are sometimes a sign of more serious blood

vessel malformations within the brain. For this reason children with this type of birthmark will undergo further testing, usually neurological consultation and CT or MRI scanning.

Port wine stain is an irregularly shaped vascular malformation with prominent deep layer dermal blood vessels that are oftentimes present at birth and essentially do not disappear with time. 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. Port-wine stains (nevus flammeus) are malformations of tiny blood vessels that connect the veins to the arteries (capillaries) located in the upper levels of the skin. These irregularly shaped patches range in color from light pink to red to dark red-violet and may darken with age. Port-wine stains, usually located on the neck, face, and scalp, occur in about 0.5% of newborns.

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 localised 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).

The tiny blood vessels (capillaries) in port wine stains remain wide (dilated). It is like a permanent localised blush. The constant supply of extra blood makes the skin look red or purple. 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. However, 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. (The Sturge-Weber syndrome.) Epilepsy and other problems may then develop.

The mainstay of therapy for port wine stains in today’s medical environment is the Candela flash lamp pulse dye laser, which is specifically designed to treat vessels in the epidermis and dermis of children and adults with port wine stains. The fancy nomenclature for laser treatment of port wine stains is “selective photo thermal lysis," which simply means that the laser will specifically treat the dilated vessels and not injure the surrounding epidermis, dermis, and soft tissue structures other than the vessels. 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.

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.

Topical EMLA cream is oftentimes used for anesthesia on the areas to be treated. Based upon the test energy levels, an appropriate energy is selected. Sometimes, infiltration with Lidocaine is performed. Protection is worn over the eyes and the entire lesion is treated with the laser. There is some “overlap” of the laser treatment delivered, so as to avoid a mottled or “egg crate” appearance. Darker purple lesions are somewhat less responsive to laser treatment. Individuals with darker pigmented skin are somewhat less responsive to treatment due to inhibition of the laser penetration by the increased protective melanin. Essentially, individuals with darker colored skin require more treatments to obtain satisfactory clearing. Between two and ten treatments are required to resolve a port wine stain.

Cosmetic or camouflage creams are often very helpful and expert advice is available in special British Red Cross clinics based at selected dermatology centres. Changing Faces and the Disfigurement Guidance Centre also offer a comprehensive service for patients and their families with all types of birthmarks.

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All information is intended for reference only. Please consult your physician for accurate medical advices and treatment. Copyright 2005,, all rights reserved. Last update: July 18, 2005