Health Encyclopedia

Sturge-Weber syndrome

Definition

Sturge-Weber syndrome is a rare disorder present at birth in which the child has a port-wine stain birthmark (usually on the face) and neurologic problems.

Alternative Names

Encephalotrigeminal angiomatosis

Causes, incidence, and risk factors

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

Symptoms

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

Signs and tests

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

Treatment

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

Anticonvulsant medicines for seizures
Eye drops or surgery to treat glaucoma
Laser therapy for port-wine stains
Physical therapy for paralysis or weakness
Possible brain surgery to prevent seizures

Support Groups

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

Expectations (prognosis)

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

Complications

Abnormal blood vessel growth in the skull
Continued growth of the port-wine stain
Developmental delays
Emotional and behavioral problems
Glaucoma, which may lead to blindness
Paralysis
Seizures

Calling your health care provider

All birthmarks, including a port-wine stain, should be evaluated by the health care provider. Seizures, 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

There is no known prevention.

Review Date:10/23/2007
Reviewed By:Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor of Pediatrics, NYU School of Medicine, New York, NY. Review provided by VeriMed Healthcare Network.

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