Support Groups, Resources, Facts, Family Connection
Entries provide a detailed account of Aiden's experince with Sturge-Weber
Lots of pictures of my handsome little man
Get the facts
Help raise awareness
Newspaper and television
What's New
About Sturge-Weber Syndrome
Sturge-Weber Syndrome is a congenital, non-familial disorder of unknown incidence and cause. It is characterized by a congenital facial birthmark and neurological abnormalities. Other symptoms associated with Sturge-Weber can include eye and internal organ irregularities. Each case of Sturge-Weber Syndrome is unique and exhibits the characterizing findings to varying degrees.
Facial Birthmark
The most apparent indication of Sturge-Weber Syndrome is a facial birthmark or "Port Wine Stain" present at birth and typically involving at least one upper eyelid and the forehead. Much variation in the size of the stain has been reported and may be limited to one side of the face or may involve both sides. The stain, varying from light pink to deep purple, is due to an overabundance of capillaries just beneath the surface of the involved skin. In persons with dark pigmentation, the stain may be difficult to recognize. In rare instances, there is an absence of a Port Wine Stain.
Neurological Abnormalities
Neurological concerns relate to the development of excessive blood vessel growth on the surface of the brain (angiomas). These are located typically on the back (occipital) region of the brain on the same side as the port wine stain. These angiomas create abnormal conditions for brain function in the region. Seizure activity is the most common early problem, often starting by one year of age. The convulsions usually appear on the opposite side of the body from the Port Wine Stain and vary in severity. Vigorous attempts are made to control the seizures with medication. A weakening or loss of the use of one side of the body (hemiparesis), may develop opposite to the port wine stain. Developmental delay of motor and cognitive skills may also occur to varying degrees.
Other Manifestations
Increased pressure within the eye (glaucoma) is another condition which can be present at birth or develop later. The incidence of glaucoma in patients with Sturge-Weber is approximately 70% and 40% for choroidal lesions. The glaucoma is usually restricted to the eye which has the stain involvement. Enlarging of the eye (buphthalmos) can also occur in the eye which has been affected by the stain. Multiple other body organs are rarely affected in Sturge-Weber syndrome. Infants affected with Sturge-Weber Syndrome are often monitored by a pediatrician, neurologist, ophthalmologist and dermatologist.
Treatment
Laser treatment is available to lighten and or remove port wine stains in children as young as one month of age. Anti-convulsants are used to control the seizures. VNS implants and brain surgery can be also be utilized for seizure management. Eye drops or oral medications are used to control the glaucoma. Should topical or oral medications not prove effective, the next step would be surgery.
To learn more visit www.sturge-weber.org
For a more in-depth look at how Sturge-Weber affects the brain, click here.
Web Design by Rachel Waters
Send an email or sign our guestbook
Your tax-deductible donation will go to the Sturge-Weber Foundation
