Sonya had her first dentist appointment this morning with our local pediatric dentist and what we suspected was confirmed. Sonya has moderate to severe
Dentinogenesis Imperfecta which is caused by her
Osteogenesis Imperfecta. I'm so glad we have such a fantastic local pediatric dentist (he is just 10 minutes down the street!) who is willing to take care of her follow up appointments, unfortunately he is not equipped to put her under and perform her capping. We will be traveling down to
Children's Hospital of Philadelphia to meet a pediatric dentist whom our dentist
referred us to and is
knowledgeable in this field to perform her capping. Capping is necessary for her ASAP as her teeth are already showing signs of fast wearing away. If left untreated she will wear away her teeth by the age of 2. So far she has 8 teeth, 4 on top and 4 on bottom, all of which are affected. I am
sooooo nervous about her being put under again, but this is what's needed and what is best for my baby!
For a little background here is some information on
Dentinogenesis Imperfecta if you are interested in learning some more.
Osteogenesis imperfecta (OI) is always associated with bone fragility. In addition, OI may affect the growth of the jaws and may or may not affect the teeth. About half of the people who have OI have teeth that appear normal, and their major concerns are routine care. However, the other half has a defect in the teeth called
dentinogenesis imperfecta (DI), sometimes referred to as opalescent teeth or brittle teeth. These teeth may be misshapen, may chip or break easily, and will require special care. Oral cavity problems related to
osteogenesis imperfecta may
include the following:
• A skeletal Class III malocclusion. The teeth do not correctly match up making biting difficult. This is caused by the size and/or position of the upper jaw or the lower jaw.
• An open bite. There is a vertical gap between some of the upper and lower teeth.
• Impacted teeth. The first or second permanent molars do not erupt, or they erupt out of the usual location (ectopic).
• Dental development. Tooth development may be delayed or advanced in some individuals affected by OI. OI does not affect the presence or absence of gum disease (
periodontitis).
Major Parts of the TeethThe teeth are made up of four distinct parts.
• Enamel is the outside part of the crown. It is the hardest substance in the body and the point of contact for chewing.
• Dentin is the substance under the enamel that forms the rest of the crown and surrounding the pulp chamber and almost all of the root structure. It is similar to bone.
• The Pulp Chamber is the inner hollow part of the tooth containing blood vessels and nerves.
• The
Dentinoenamel Junction (
DEJ) is the term for where the enamel and dentin are attached to each other.
Dentinogenesis Imperfecta (DI)
Dentinogenesis imperfecta can be part of
osteogenesis imperfecta (DI type I) or it can be a separate inherited dominant trait without OI (DI type II). DI occurring with OI seems to run in families but can vary in severity from one member to another. DI has a variable affect on the color, shape, and wear of both primary and permanent teeth. If someone has OI and DI, all of their teeth may not be affected to the same degree. Teeth affected by DI have essentially normal enamel, but the
DEJ and the dentin are not normal. The enamel tends to crack away from the dentin, which will wear away more quickly than enamel. The dentin makes the teeth look darker or opalescent. The dentin also grows to fill in the pulp chamber, causing a loss of feeling in the tooth. Affected teeth will have an increased incidence of fracture, wear and decay.
Dentinogenesis imperfecta may be diagnosed with the first baby tooth. If the tooth looks gray, bluish, or brown, DI should be suspected. Children should be taken to a dentist (if possible a specialist in pediatric dentistry) when the first teeth are erupting. This may happen as early as 6 months to 1 year of age.
Radiographs, or X-rays, can be useful but may be difficult to obtain until the child is older. Sometimes there are changes visible on the X-rays that are not obvious just by looking at the teeth. Crowns appear bulbous and roots may be shorter and more slender than standard. Primary teeth are usually more affected than the permanent teeth. When, for any reason, crowns are not feasible, a “tooth color” dental material may be used, such as composites or glass
ionomers in conjunction with composites. The sand abrasion method may also be useful because it removes carious
dentine only and thus spares dental tissue. In any case, amalgam restorations should not be used because they impose an additional stress on the teeth.
General Care for People With OI Plus DIChildren with OI and
dentinogenesis imperfecta need the same basic care children without DI, but they also need to be monitored for cracking, chipping and abrasion of the teeth. Special care will be needed even with the baby teeth. All of the teeth may not be affected by DI, and primary teeth usually are affected to a greater extent than the permanent teeth. Restorative treatment may be needed at some point. Regular care is needed so the teeth will last as long as possible and to prevent abscesses and pain. Brushing and cleaning has not been shown to cause damage, but will not make teeth affected by DI white. Sealants should be effective on teeth affected with DI as long as the enamel is intact. Older children and especially adolescents with DI are often embarrassed by their discolored teeth. Different types of veneers can sometimes hide the problem. Bleaching is not recommended because the discoloration is not in the enamel. If the teeth are wearing excessively, caps (also called crowns), will probably need to be placed on at least some of the teeth. Caps serve to keep the teeth in place and encourage proper development of the jaw. More specialized treatment may be more appropriate for permanent teeth.
Treating
Malocclusions with Orthodontia or
Orthognathic Surgery A malocclusion is an abnormal relationship between the upper and lower teeth, which creates problems with how the teeth come together. This may be due to the relationship of the upper and lower jaws to each other, the alignment of the teeth, or both. This type of problem includes crooked teeth, “
underbite,” “overbite” and “open bite.” Treatment is usually provided by an orthodontist. The particular treatment plan depends on the specific problem(s) with the bite and the teeth. If the malocclusion is caused by skeletal discrepancies, then
orthognathic (jaw) surgery may be required along with orthodontia. An orthodontist should examine each child with OI around the age of 7 years. At that time early orthodontic interventions in children who are developing a relatively small upper jaw compared to the lower jaw may help decrease the need for later
orthognathic surgery.
Sounds like fun huh? Well, for now I am just praying that we get in soon to the specialist at CHOP and that the
procedure goes well! Then I will be praying that her permanent teeth will be less severely affected by her DI. Thanks for your support everyone, as always I am so grateful that I have you all to lean on! Since you are all simply the best, here is your reward for listening.... Sonya photos!!!

I love bath time in the kitchen sink!

Woo Hooooooo!!!!!

Hey! Who put that washcloth there?

Rockin' the mowhawk!

Handful of bubbles!

Mommy!!! That's enough already! Now I look like Little Cindy Lou Who!