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Sturge-Weber syndrome
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Sturge-Weber syndrome

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Encephalotrigeminal angiomatosis

Sturge-Weber syndrome is a rare disorder that is present at birth. A child with this condition will have a port-wine stain birthmark (usually on the face) and may have nervous system problems.

I Would Like to Learn About:

  • Causes

    The cause of Sturge-Weber is unknown. It is not thought to be passed down (inherited) through families.

  • Symptoms

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

    Glaucoma may be one sign of the condition.

    Tests may include:

    • CT scan
    • MRI scan
    • X-rays
  • 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
  • Outlook (Prognosis)

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

    Patients will need to visit an ophthalmologist at least once a year to treat glaucoma. They also will need to see a neurologist to treat seizures and other nervous system symptoms.

  • Possible 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
  • When to Contact a Medical Professional

    The health care provider should check all birthmarks, including a port-wine stain. Seizures, vision problems, paralysis, and changes in alertness or mental state may mean the coverings of the brain are involved. These symptoms should be evaluated right away.

  • Prevention

    There is no known prevention.

Related Information

  GlaucomaTonometryBlindness and visi...SeizuresMuscle function lo...     Glaucoma

References

Sahin M. Neurocutaneous syndromes. In: Kliegman RM, Stanton BF, St. Geme III JW, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 589.

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Review Date: 10/29/2013  

Reviewed By: Chad Haldeman-Englert, MD, FACMG, Wake Forest School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team.

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