MedlinePlus Medical Encyclopedia: Sturge-Weber syndrome

Skip navigation


Medical Encyclopedia
Other encyclopedia topics:	A-Ag Ah-Ap Aq-Az B-Bk Bl-Bz C-Cg Ch-Co Cp-Cz D-Di Dj-Dz E-Ep Eq-Ez F G H-Hf Hg-Hz I-In Io-Iz J K L-Ln Lo-Lz M-Mf Mg-Mz N O P-Pl Pm-Pz Q R S-Sh Si-Sp Sq-Sz T-Tn To-Tz U V W X Y Z 0-9

Sturge-Weber syndrome

Contents of this page:
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

Illustrations

Sturge-Weber syndrome - legs

Sturge-Weber syndrome - soles of feet

Circulatory system

Alternative names Return to top

Encephalotrigeminal angiomatosis

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.

Update Date: 11/4/2003

Updated by: Celeste Krauss, M.D., Board Certified Clinical Genetics, Clinical Cytogenetics and Pediatrics. Founding Fellow, American College of Medical Genetics. Review provided by VeriMed Healthcare Network.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed physician should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. Copyright 2002 A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.