Sturge-Weber disease

Alternative names:

encephalotrigeminal angiomatosis; Sturge-Weber-Dimitri syndrome

Treatment:

Treatment is based on the findings:

• Seizures are treated with antiseizure medications such as phenobarbital, Dilantin, or carbamazepine. Occasionally, surgical excision is done in hopes of preventing seizures that are difficult to control with medications.
• Paralysis or weakness is treated with appropriate physical therapy.
• Learning disabilities require appropriate school intervention.
• Glaucoma requires early intraocular pressure testing and treatment to prevent blindness.
• Port-wine-stain treatment varies. Laser therapy has proved effective at destroying the vascular lesion without damaging the normal skin.

Expectations (prognosis):

Most cases of Sturge-Weber are fairly mild. Intervention is indicated as noted in the treatment section. For mild cases life expectancy is normal. Surgical treatment of the intracranial lesion is sometimes successful in reducing symptoms.

Complications:

• glaucoma (increased intraocular pressure) with the possibility of blindness in affected eye
• convulsions
• hypertrophy of the port-wine-stain (continued growth) with disfigurement and bleeding
• paralysis on one side

Calling your health care provider:

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 indicate involvement of the coverings of the brain. These symptoms should be evaluated promptly.