Sturge-Weber Syndrome

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What is Sturge-Weber syndrome?

Sturge-Weber syndrome is with abnormal groups of blood vessels on the skin leading to a reddish color on one side of the face (port-wine birthmark, See figure 1) as well as groups of abnormal blood vessels in the brain.

Fig. 1: Sturge-Weber Syndrome showing a reddish color of the skin on one side of the face.

  • DOES EVERY CHILD WITH A PORT-WINE BIRTHMARK OF THE SKIN HAVE STURGE-WEBER SYNDROME?

    No. The birthmark on the face may be seen on its own. Sturge-Weber syndrome is diagnosed when the brain has problems as well.

    IN WHAT WAY IS THE BRAIN INVOLVED?

    Abnormal blood vessels in the brain (also called leptomeningeal malformations) may cause a variety of problems like seizures, weakness, developmental delay, and other brain problems. Many children, however, can have normal intelligence and good development.

    WHAT TESTS ARE DONE TO LOOK FOR BRAIN INVOLVEMENT?

    Imaging studies (MRI / CT) of the brain may be needed to look for abnormal blood vessels.

    WHAT CAUSES STURGE-WEBER SYNDROME?

    We are still learning more about the cause of Sturge-Weber syndrome. It is not a genetic disease that runs in families. The problems seen with Sturge-Weber syndrome happen very early in pregnancy - probably between the second and third month of pregnancy.

    WHEN IS STURGE-WEBER DIAGNOSED?

    The port-wine birthmark is usually noted shortly after birth. Further testing may if there are problems with the brain leading to a diagnosis of Sturge-Weber syndrome

    WHY IS THE SKIN OF THE FACE SO RED?

    Redness (port-wine birthmark) of the skin is caused by a large group of blood vessels. The redness is often on the forehead and upper eyelid and less often on the lower eyelid and cheek.

    IS THERE ANY TREATMENT FOR THE SKIN?

    The color of the skin can be improved with laser treatments in some cases.

    WHAT EYE PROBLEMS CAN OCCUR WITH STURGE-WEBER SYNDROME?

    Some children with Sturge-Weber have eyes that are different colors. The white surface of the eye can have enlarged blood vessels which cause the eye to look pink or red. These extra blood vessels can occur inside the eye as well and cause, high eye pressure. If this extra pressure damages the optic nerve, this condition is called “glaucoma.”

    Glaucoma is the most serious eye problem seen with Sturge-Weber syndrome. About 50% of children with Sturge-Weber will get glaucoma.  Glaucoma can occur early in infancy or may not appear until later in childhood. Signs of glaucoma may include a large eye, a cloudy appearing eye, light sensitivity, or a watery eye.

    If the optic nerve or vision areas of the brain are affected by Sturge-Weber, a problem with peripheral vision (side vision) may develop.

    Any child with a port-wine birthmark should be evaluated by an ophthalmologist. Children diagnosed with Sturge-Weber Syndrome should be examined by an ophthalmologist soon after the diagnosis and should be monitored by an ophthalmologist for eye problems.

    HOW IS GLAUCOMA FROM STURGE-WEBER SYNDROME TREATED?

    Eye drops may be used to reduce the pressure inside of the eye. Sometimes, eye surgery is needed to help lower the pressure and protect vision.

    WHERE CAN I LEARN MORE ABOUT STURGE-WEBER SYNDROME?

    ·         Sturge-Weber Foundation

    ·         https://eyewiki.org/Sturge-Weber_Syndrome_and_Secondary_Glaucoma

Updated 04/2023


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