Sturge-Weber syndrome

• Illustrations
• Alternative names
• Definition
• Causes, incidence, and risk factors
• Symptoms
• Signs and tests

• Treatment
• Support Groups
• Expectations (prognosis)
• Complications
• Calling your health care provider
• Prevention

Alternative names    Return to top

Definition    Return to top

Sturge-Weber syndrome is a rare disorder present at birth. It is characterized by a birthmark, usually on the face, known as a port wine stain (from too many blood vessels just beneath the skin) and neurologic problems.

Causes, incidence, and risk factors    Return to top

The cause of Sturge-Weber is unknown. There is no known hereditary component.

Symptoms    Return to top

• Port-wine stain (more common on the face than the body)
• Convulsions
• Paralysis or weakness on one side
• Glaucoma
• Learning disabilities

Signs and tests    Return to top

X-rays, MRI, or CT scans are useful to look for associated problems.

Treatment    Return to top

Treatment is based on the patient's signs and symptoms:

• Anticonvulsant medicine for convulsions.
• Physical therapy for paralysis or weakness
• Eye drops or surgery for glaucoma
• Laser therapy for port wine stains

Support Groups    Return to top

For information and support, see www.sturge-weber.com.

Expectations (prognosis)    Return to top

Most cases of Sturge-Weber are not life threatening. The quality of life depends on how well the symptoms, such as convulsions, can be addressed.

Complications    Return to top

• Glaucoma (increased intraocular pressure) with the possibility of blindness in the affected eye
• Convulsions
• Continued growth of the port-wine stain
• Paralysis on one side
• Emotional and behavioral problems
• Developmental delays
• Abnormal blood vessel growth in the skull

Calling your health care provider    Return to top

All birthmarks, including a port-wine stain, should be evaluated by the health care provider. Convulsions, visual problems, paralysis, and change in alertness or mental state may mean the coverings of the brain are involved. These symptoms should be evaluated promptly.

Prevention    Return to top

There is no known prevention.