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 even softer. Parents need to brush children's teeth until
about the age of 6 or 7 years because children lack the manual dexterity
to properly clean their teeth. Parents should be very careful not to
damage the gums or make the brushing experience negative and unpleasant.
It is important, however, that the teeth be cleaned at least once a day
preferably just prior to bedtime.
Be sure
and use a fluoridated toothpaste. In small children a pea size amount is
adequate to deliver the fluoride to the teeth. For individuals prone to
developing cavities there are special high strength prescription
fluoride toothpaste. Strongly flavored toothpaste (mint) may be
irritating to the individuals with severe oral involvement, however,
there are numerous non-mint flavors available. Bubble gum flavor is a
big hit with children (and some adults). In addition to the systemic
fluorides that we get in the water or alcoholic fluoride rinses
available for the EB patients that are sensitive to strong flavoring
agents and alcohol. Non-alcoholic rinses with greater amounts of
fluoride are available by prescription. Chlorhexidine mouth rinses (an
antibacterial rinse) also ma assist in controlling dental caries,
however, sensitivity due to the high alcohol content can be problematic.
This may be overcome by swabbing it directly on the teeth. Chlorhexidine
rinse is a prescription item.
There are a
variety of fluoride treatments applied by dentists. The most common
treatment consists of placing a 1.23% acidified gel in a tray that is
held in the mouth for 4 minutes. The high concentration of flavoring
agents and acid nature of the gel make it unacceptable to some EB
patients. Milder flavored neutral sodium fluoride gels are available
that may be less irritating to fragile mucosa. Recently, a high
concentration fluoride varnish has become available in the United States
offering a wonderful option for delivering maximum fluoride protection
to the teeth of individuals with even the most sensitive mucosa. This
varnish is simply painted on the teeth by the dentist.
The diet
constitutes major difficulty in caries control, and due to the complex
systemic nutritional demands of individuals with sever EB types, this
may be best managed with the assistance of a dietician. The effects of
any diet planning should be considered with regards to dental health and
tooth friendly foods (cheese, vegetables, fresh fruits) eaten as much as
possible. Be careful of the less obvious cavity producing foods such as
highly sweetened breakfast cereals, raisins and dried fruits. Cavity
producing oral bacteria can ferment carbohydrates from a wide variety of
foods. Other helpful hints are to rinse the mouth or drink water after
eating if brushing is not possible. Bottle or breast feeding infants at
bedtime can result in nursing caries after the teeth are present (1 year
of age). If nursing continues after 1 year great care should be taken
not to give the bottle while the infant is going to bed. Virtually any
bottle fed liquid, except water, can cause rapid tooth decay of the baby
teeth.
Individuals
with even the severest forms of EB can now maintain their natural
dentition providing them the ability to chew, eat a healthy diet and
have a pretty smile. In the not too distant past, dental extraction was
considered the treatment of choice for individuals severely affected
with EB. Now we are able to prevent tooth decay, restore malformed
enamel and help produce good alignment of the teeth. The future looks
even brighter as dental prevention continues to improve, new dental
materials are stronger and more esthetic than ever and new technologies,
such as dental implants, continue to improve. Dental health for all EB
patients has become a reality and that is really something to smile
about. .
