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Hopkins Health
A Devastating Disease Beneath the Skin
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| Pediatric dermatologist Bernard Cohen with Sturge Weber patient, 4-year-old Madeline Peterson. |
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As pediatric dermatologist Bernard Cohen, M.D., examines the purplish blotch on his three-year old patient’s face, it’s not this so-called port wine stain that concerns him the most.
He knows that the discoloration covering her left eye, forehead and scalp follows the first branch of the trigeminal nerve beneath the skin. That means his patient could have Sturge-Weber syndrome, or SWS, which begins in utero as a spider-like tangle of abnormal blood vessels in the tissues surrounding the brain. This patient is not only afflicted with a facial disorder—she may be facing glaucoma, seizures, strokes and developmental delays, too.
“What appears to be in the skin only can sometimes lead to a major disability, socially and medically,” Cohen says. “A stain with this type of distribution puts the child at risk.”
Using the latest laser therapies, Cohen says he is able to eliminate up to 75 percent of the port wine stains in the majority of his patients.
Patients who wear the marker for SWS he refers to the Johns Hopkins Sturge-Weber Syndrome Center, a new option for patients who have had to shuttle between specialists at several institutions for treatment.
The center’s approach, Cohen stresses, hinges on early diagnosis.
Infants are screened at birth for signs of glaucoma. MR perfusion imaging is used to spot brain areas at high risk of ischemic damage, and quantitative EEG to predict who’ll have severe disease.
“If you delay treatment, you get thickening of the skin and soft-tissue changes that makes it more difficult to treat,” Cohen says. “Early treatment can prevent those complications.” |
