Sturge-Weber Syndrome

Written by Kristeen Moore | Published on August 7, 2012
Medically Reviewed by Brenda B. Spriggs, MD, MPH, FACP

What Is Sturge-Weber Syndrome?

Sturge-Weber syndrome is a rare disorder characterized by nervous system problems and a permanent birthmark—a port-wine stain—usually on the face. It is a rare type of neurocutaneous (nerve and skin) disorder that is present at birth. Complications may result from lack of treatment, but this condition isn’t usually life threatening.

Causes of Sturge-Weber Syndrome

The cause of Sturge-Weber syndrome is unknown. Though it is present at birth, it is not a genetic disorder.

Symptoms of Sturge-Weber Syndrome

The most noticeable symptom of Sturge-Weber syndrome is a port-wine stain birthmark on the face. Although this type of birthmark generally covers one side of the face, it can also form on the neck, as well as on other parts of the body. It begins as a pink mark at birth, but thickens and darkens with age.

Other symptoms related to the condition aren’t as prominent at birth, but may develop as a child grows older. These include:

  • paralysis
  • learning disabilities
  • seizures

Diagnosing Sturge-Weber Syndrome

A port-wine stain is the most common indication of this condition. If you have one, your doctor will run tests to check your neurological functions.

X-rays, computed tomography (CT) scans, and magnetic resonance imaging (MRI) scans can be used to take detailed images of your brain. This can help measure your risk for seizures and help your doctor determine which treatment plans are best for you. Seizures caused by Sturge-Weber syndrome may develop as early as infancy.

Treatment Options

There is no cure for Sturge-Weber syndrome. The purpose of treatment is to control symptoms and improve your quality of life. Anticonvulsant drugs can help prevent seizures, and brain surgery may be necessary in severe cases. Patients with paralysis may undergo physical therapy to help improve mobility and muscle function.

Laser therapy is the only way to fade port-wine stains. According to KidsHealth.org, infants as young as one month old may start laser therapy to remove birthmarks.

Potential Complications

Glaucoma is one of the most common complications of this condition. According to KidsHealth.org, approximately 30 percent of Sturge-Weber patients develop glaucoma, which is caused by increased pressure behind the eyes (Kids Health). If left untreated, glaucoma can lead to blindness in one or both eyes. It usually occurs on the same side of the face where the port-wine stain is located.

Other potentially serious complications of this syndrome include:

  • growth of the port-wine stain marking
  • paralysis
  • abnormal blood vessel development in the brain
  • severe seizures
  • developmental delays
  • learning disabilities
  • emotional and behavioral disorders

Outlook for Sturge-Weber Syndrome

Sturge-Weber syndrome isn’t life threatening in most cases. However, quality of life can be significantly improved by keeping up with treatment as recommended by your doctor. It’s important that patients with this disorder see a neurologist and ophthalmologist (eye doctor) regularly to ensure proper management of their symptoms and to monitor of their neurological development.

Most patients have better self-image after having laser treatment to reduce port-wine stains.

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