Definition
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, and neurologic problems.
Alternative Names
Encephalotrigeminal angiomatosisCauses, 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:
- Anticonvulsant medicine for seizures
- Physical therapy for paralysis or weakness
- Eye drops or surgery for glaucoma
- Laser therapy for port-wine stains
Support Groups
For information and support, see .
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 addressed.
Complications
- Glaucoma (increased intraocular pressure) with the possibility of blindness in the affected eye
- Seizures
- 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
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.
| • | Glaucoma |
| • | Tonometry |
| • | Blindness |
| • | Seizures |
| • | Muscle function loss |
Reviewed By: Christos Ballas, M.D., Attending Psychiatrist, Hospital of the University of Pennsylvania, Philadelphia, PA. Review provided by VeriMed Healthcare Network.
