Sturge-Weber Syndrome - Oral Manifestations

Sturge-Weber Syndrome (SWS), also known as encephalotrigeminal angiomatosis occurs in about 1 of 50,000 births as reported in Nelson's Textbook of Pediatrics.

SWS usually occurs at birth and sometimes is associated with seizures or mental retardation. It's visual characteristic is a facial port wine stain. Few intraoral photos are available of SWS, so I took the following images with permission from one of our patients at the Care Free Dental Clinic in Escanaba, Michigan:

Most apparent on the hard and soft palate are the bright red striations caused by oversized blood vessels.

He reports being told that he is very fortunate, because he has no mental retardation or seizures.