A rare and sporadically occurring disease without a recognized hereditary component. It consists of multiple vascular lesions (angiomas) involving the skin of the face, mucus membranes, and covering of the brain (meninges).

Alternative Names

Encephalotrigeminal angiomatosis; Sturge-Weber-Dimitri syndrome

Causes, incidence, and risk factors

The cause of Sturge-Weber is unknown. Because of its spotty appearance in the population, specific risk factors are not yet recognized.

Signs and tests

A skull X-ray shows damage to the inner layer of the skull caused by the vascular lesion and intracranial calcifications . Increased pressure in the eyeball or glaucoma is found in up to 60% of patients. MRI or CT scans of the skull may show benign tumors of the lining of the brain (dural angiomas) or of the choroid plexus.

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.

Support groups

Sturge-Weber Foundation

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.

Prevention

There are no known preventive measures.