Capillary Malformations

Recommend Email

Capillary Malformations

A capillary malformation (commonly referred to as a port-wine stain), is a flat, sharply defined vascular stain of the skin. It may cover a large surface area or it may be scattered and appear as smaller spots of color. It is usually located on one side of the body without crossing the midline. It can be found anywhere on the body (illustration 1), most commonly the head / neck region (illustration 2).

Recently, a genetic change (mutation) was found in the affected skin from capillary malformations but was not present in nearby normal skin. This pattern tells us that capillary malformations occur in early development when tiny blood vessels of the skin form abnormally as a result of faulty instructions. The occurrence of capillary malformations is unrelated to drugs or medications that may have been taken during pregnancy or to environmental exposures that may have occurred at that time.

Another capillary vascular lesion, called nevus simplex, is mostly seen in newborns and is often confused with capillary malformations. When these birthmarks appear on the forehead, eyelids, nose or upper lip, they are commonly called “angel kisses.” When seen on the back of the neck, they are commonly called “stork bites.” These lesions usually fade by 1 year of age and do not require treatment.

What Do Capillary Malformations Look Like?Show

Capillary malformations appear pink, red or purple due to an increased number and size (diameter) of affected blood vessels in comparison to normal capillaries of the skin. As your child grows, the affected blood vessels will continue to enlarge and thicken, causing the color of the lesion to darken. Over time, clusters of tiny, dilated venules (small vessels that collect blood from the capillary junctions and join to form veins) may give a lumpy appearance to the skin. The degree and length of time to develop skin thickening varies greatly among individuals, and may be delayed into early or mid-adulthood.

Diagnosis of Capillary MalformationShow

Most capillary malformations are diagnosed by history and physical exam alone. A full physical exam that looks for thickening, asymmetry or warmth is also helpful in eliminating similar or related diagnoses.

Complications of Capillary MalformationShow

Capillary malformations can be associated with overgrowth of underlying soft tissues or bones. The color of a facial lesion can darken, and the skin can thicken with age and may be prone to bleeding.

When the capillary malformation involves the forehead and/or upper eyelid, abnormalities of the eye and/or brain may be present (Sturge-Weber syndrome). All lesions around the eye should have routine eye exams to evaluate for glaucoma. Magnetic resonance imaging (MRI) may be used to determine if there is brain involvement.

Capillary malformations in the skin directly overlying the spine can be associated with spinal abnormalities such as tethering of the spinal cord and should be investigated with an ultrasound or MRI.

Treatment for Capillary Malformation Show

Several treatment approaches are used to treat capillary malformations.

Laser therapy with the pulsed-dye laser is the standard treatment for capillary malformations on the face. The goals of treatment are both to prevent lesions from thickening and to allow children to maintain an appearance that is cosmetically acceptable. Many lesions lighten significantly after multiple treatments, but very few resolve completely. Laser therapy may also prevent the skin from thickening over time. The outcome with laser is better in the facial area than on the trunk or limbs. Since the laser does not penetrate deeply, deeper vessels do not respond as well as vessels nearer the skin surface. The number of treatments required varies significantly (depending on the size and depth of the lesion). Treatments are usually spaced at two- to three-month intervals. Because the procedure is typically painful, either a general or a topical anesthetic is used. General anesthesia is preferred in children, but the frequency of procedures is limited due to sedation risks.

Surgical procedures may be considered when there is overgrowth of soft tissue or bone enlargement. In cases where the skin is very thick and laser therapy is unsuccessful, surgery and skin grafting may be performed.

Possible Complications and Risks from SurgeryShow

While each of these treatment approaches can be effective, they both involve certain risks.

The risks of treatment with pulsed-dye laser are low if treatment is performed by an experienced clinician. Rarely, blistering may occur, resulting in scarring. Pigment changes, either whitening or temporary darkening of the skin, may also occur with variable risk depending on skin color. Even with laser treatment, a capillary malformation may reappear after several years.

The risks of surgery depend upon the type of surgery performed and the complexity of the problems in each individual patient. However, general risks include infection, minor bleeding and scarring. A risk of skin grafting is the loss of the graft.

Reappearance of Capillary MalformationShow

Even with laser treatment, a capillary malformation may reappear after several years. The goals of treatment are thus to both prevent lesions from thickening and to allow children to maintain an appearance that is cosmetically acceptable.

Last Updated 11/2016

The Hemangioma and Vascular Malformation Center at Cincinnati Children’s cares for patients of all ages and offers treatment options not widely available at other centers.

Conditions & Treatments