Sturge-Weber Syndrome Symptoms and Treatment

Sturge-Weber syndrome is a disorder of the skin and nervous system. Its most noticeable symptom is a light pink to deep purple birthmark on the face called a port-wine stain. However, not all people who have a port-wine stain have Sturge-Weber syndrome. Sturge-Weber syndrome is present at birth, but it is not known what causes it or exactly how often it occurs. It affects both males and females of all ethnic backgrounds.

There are three types of Sturge-Weber syndrome:

  • Type I: Most common type, includes port-wine stain and brain angiomas, and may involve glaucoma
  • Type II: Port-wine stain and possibly glaucoma, but no brain angioma
  • Type III: Brain angioma but no port-wine stain and no glaucoma
Port wine stain on cheek
Khosrork / Getty Images


Symptoms of Sturge-Weber syndrome include:

  • Port-wine stain: This birthmark on the face can vary in size, but it usually covers at least one upper eyelid and the forehead. It can range from light pink to deep purple and is caused by an excessive growth of tiny blood vessels (capillaries) just beneath the surface of the skin. The port-wine stain may be harder to see in dark-skinned individuals.
  • Seizures: About 75% to 90% of individuals with Sturge-Weber syndrome develop seizures, often starting by one year of age. The seizures result from an excessive growth of blood vessels (angioma) on the surface of the brain, usually on the back of the brain on the same side as the port-wine stain.
  • Weakness: About 25% to 56% of individuals develop weakness or loss of use of one side of the body (hemiparesis), often on the side opposite the port-wine stain.
  • Developmental delays: Developmental delays and mental retardation affect about 50% to 60% of individuals.
  • Increased pressure in the eye (glaucoma): About 70% of individuals develop glaucoma in the eye near the port-wine stain.
  • Headaches


A diagnosis of Sturge-Weber syndrome may be suggested by the presence of a port-wine stain over one eye and the forehead. Computed tomography (CT) scan or magnetic resonance imaging (MRI) can examine the brain for the presence of one or more angiomas, which would confirm the diagnosis. The child with Sturge-Weber syndrome will have a neurological examination to check for complications of the brain angioma, such as seizures or weakness.


The treatment of Sturge-Weber syndrome focuses on the symptoms. If seizures occur, antiseizure medications such as carbamazepine (Tegretol), phenytoin (Dilantin), or valproic acid (Depakote, Depakene) are given. Medications are used to reduce and control glaucoma and headaches.

Laser treatment can lighten or remove the port-wine stain on the face. Multiple treatments may be needed. All children with Sturge-Weber syndrome should be treated with aspirin beginning in infancy. These children are prone to the development of blood clots, which can reduce brain blood flow and cause neurological damage. Aspirin can protect against that.

Was this page helpful?
Article Sources
Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read our editorial process to learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.
  • "What is Sturge-Weber Syndrome?" About SWS/KT/PWS. The Sturge-Weber Foundation. 10 May 2007