Saturday, March 13, 2010

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.











Caries, Decay, Cavities, Acid Erosion

Caries and acid erosion can be seen between these two teeth below:





I recently cared for a young woman who has had recurrent episodes of decay (caries) between her back teeth. She's a soda drinker, and admits that she isn't flossing enough. When I began to remove the decay from between her teeth, I saw what is in the image above. I showed the patient this area, and she remarked that she "would really start flossing and stop drinking pop."


I thought that if the image was helpful for her, perhaps others would benefit by seeing the damage caused by acid attack between teeth. These areas are usually invisible to the patient, and even to the dentist without an x-ray.


The photo is of the area between two teeth. The caries is in it's initial state, as it doesn't show a hole between the teeth yet. Decalcification occurs first, which is the whiter area in the photo. This is the outer layer of the enamel of the tooth that has been thinned or weekened. The next layer of the tooth as you proceed deeper is the dentin. The tooth on the right has begun to have caries form in the dentinal layer, while the tooth on the left has progressed much farther. This second layer of the tooth is much softer than the enamel, and decays and washes out much faster, even traveling laterally undermining sound enamel.


Acid erosion from lack of flossing or from too much soda or sports drinks can cause destruction in a tooth from the inside out. Seeing is believing in this case.