Dentinogenesis imperfecta

I just took my 4 year old daughter to the dentist today. My daughter has discolored teeth, and we were told she has dentinogenesis imperfecta. I have the same condition, as does my mother and grandmother. I was always told that I had colored teeth, and I had to have alot of cosmetic work done. I would like as much information as possible on this genetic condition.

Is there anything we can do now to keep her adult teeth from having this same condition when they come in, such as fluoride, calcium, vitamins, etc? If not, is there anything we can do when she becomes a young teenager to make her teeth look a little better until her teeth stop growing? I would appreciate as much information as you can give me.


Dear Frank,

Dentinogenesis imperfecta (DI) is an inherited disorder of the dentin (layer of tooth beneath the enamel). This disorder is sometimes linked to osteogenesis imperfecta. The presence of blue sclera or a history of bone fractures are signs of osteogenesis imperfecta. Bone density measurement can be done to determine the presence of osteogenesis imperfecta. Dentinogenesis imperfecta affects only the teeth. This is a rare disorder found in about 1:7000 children.

Both dentinogenesis imperfecta and osteogenesis imperfecta are autosomal dominant but are mapped to two different chromosomes. DI is mapped to a mutation at 4q13-21, and osteogenesis imperfecta is a mutation at 7q22. It is apparently not sex-linked, and appears equally in males and females.

Teeth affected by DI have an amber, gray, or purple opalescence (translucence) or discoloration. The pulp chamber (area within the tooth containing the nerve and blood vessels) may be completely obliterated. This is due to the continued deposition of dentin by the cells which produce it. The crowns of the teeth are generally short and bulbous in appearance, while the roots are narrow. The enamel can chip away within about 2-4 years following eruption of the teeth. This phenomenon is most likely attributed to a defect at the junction between the enamel and dentin. This exposes the dentin underneath, which is soft and wears rapidly. The dentin is also more susceptible to decay than enamel. These effects can be seen in both the primary and permanent dentition as both are affected by the genetic mutation.

Chemical analysis of these teeth reveals that the water content is greatly increased, as much as 60% above normal. The inorganic content, usually the majority of the composition of both dentin and enamel, is much less than in normal dentin. The density and hardness of the dentin is low; this is one explanation for the rapid wear of the dentin once it is exposed.

Treatment for this condition usually involves placement of crowns on the teeth, at least in the posterior region. This helps decrease the wear on the teeth and maintains proper vertical dimension between the upper and lower jaws. For primary teeth, this may mean placement of stainless steel crowns on the posterior teeth and composite resin (tooth colored filling material) for anterior teeth. Crowns may be necessary for all permanent teeth. Stainless steel crowns and/or composite resin may be used to maintain vertical dimension in the posterior region during maturation. In general, fillings are not a successful long-term solution because the dentin is so soft. If tooth loss should occur, care should be taken in the fabrication of appliances which may exert pressure on remaining teeth because the roots fracture easily. With severe cases of DI, the placement of a complete denture over the worn teeth (an overdenture) may be beneficial.

You and your dentist should treatment plan carefully. The severity of your daughter's condition must be assessed. Regular checkups may help prevent major problems brought on by dentinogenesis imperfecta.

For additional information please see:

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