By Tim Wright, D.D.S., M.S. Department of Pediatric Dentistry
Thank you Brenda for typing this for us! 🙂
Individuals with EB may have teeth with severely malformed enamel (enamel hypoplasia) and/or dental caries depending on the EB type. The enamel is usually normal in simplex and dystrophic EB types. Generalized enamel hypoplasia is typically limited to Junctional EB. Rarely individuals with non-Junctional EB types may have generalized enamel hypoplasia. Rampant dental caries occurs in Junctional EB partly because of the enamel hypoplasia. Dental decay also is frequently seen in patients with severe recessive dystrophic EB. This excessive dental caries results from severe soft tissue involvement which leads to dietary changes (soft and high carbohydrate), increased oral clearance time (secondary to limited tongue mobility and oral scarring), and creates an abnormal tooth/soft tissue relationship. Oral involvement also reduces the ability to practice preventive measures directed at reducing caries.
Because dental caries can form rapidly in individuals with recessive dystrophic and Junctional EB, dental examinations should begin by 1 year of age and be conducted at least twice a year. If caries becomes a problem then more frequent visits (4 times a year) are indicated for preventive treatments and examination. Individuals with mild EB can be treated much as any other patient. The dentist should, however, be made aware of any history of mucosal fragility and oral blistering since dental therapy can precipitate oral lesions even in mildly affected patients. Many dentists are not familiar with EB and the patient or parent must help educate the health care team. An altered approach to treatment may be required in individuals with enamel hypoplasia or rampant caries, extreme fragility of the mucosa and/or the presence of microstomia (a decreased oral opening size). Individuals with severe soft tissue involvement requiring multiple restorative and/or surgical procedures are often best managed with general anesthesia.
Preventing tooth decay is most challenging for individuals with severe mucosal involvement. In patients prone to oral blistering, oral hygiene may best be accomplished with a soft bristled, small headed toothbrush. Many small headed children’s toothbrushes are available, some of which have special grip handles that may be helpful to individuals with hands involvement. Running the bristles under hot water prior to brushing makes the