Dentinogenesis Dentinogenesis imperfectaimperfecta

What is dentinogenesis What is dentinogenesis imperfecta?imperfecta?

Dentinogenesis imperfecta is a disorder of Dentinogenesis imperfecta is a disorder of tooth development. This condition causes tooth development. This condition causes the teeth to be discolored (most often a the teeth to be discolored (most often a blue-gray or yellow-brown color) and blue-gray or yellow-brown color) and translucent. Teeth are also weaker than translucent. Teeth are also weaker than normal, making them prone to rapid wear, normal, making them prone to rapid wear, breakage, and loss. These problems can breakage, and loss. These problems can affect both primary (baby) teeth and affect both primary (baby) teeth and permanent teeth. permanent teeth.

How common is dentinogenesis How common is dentinogenesis imperfecta?imperfecta?

Dentinogenesis imperfecta affects an Dentinogenesis imperfecta affects an estimated 1 in 6,000 to 8,000 people. estimated 1 in 6,000 to 8,000 people.

TypesTypes

Types of dentinogenesis imperfecta with similar dental Types of dentinogenesis imperfecta with similar dental formalities usually an autosomal dominant trait with formalities usually an autosomal dominant trait with variable expressivity but can be recessive if the variable expressivity but can be recessive if the associated osteogenesis imperfecta is of recessive type. associated osteogenesis imperfecta is of recessive type. This type is no lThis type is no lType II : Occurs in people without other inherited Type II : Occurs in people without other inherited disorders.It is an autosomal dominant trait. A few disorders.It is an autosomal dominant trait. A few families with type II have progressive hearing loss in families with type II have progressive hearing loss in addition to dental abnormalities.addition to dental abnormalities.Mutations in the Mutations in the DSPPDSPP genegene have been identified in have been identified in people with type II and type III dentinogenesis people with type II and type III dentinogenesis imperfecta. Type I occurs as part of osteogenesis imperfecta. Type I occurs as part of osteogenesis imperfecta.imperfecta.

HistologyHistology

Dentinal tubules are irregular and are Dentinal tubules are irregular and are bigger in diameter. Areas of uncalcified bigger in diameter. Areas of uncalcified matrix are seen. Sometimes odontoblasts matrix are seen. Sometimes odontoblasts are seen in dentin.are seen in dentin.

Radiographic featuresRadiographic features

Type I and II show total obliteration of the Type I and II show total obliteration of the pulp chamber.pulp chamber.

Type III shows thin dentin and extremely Type III shows thin dentin and extremely enormous pulp chamber.These teeth are enormous pulp chamber.These teeth are usually known as usually known as Shell TeethShell Teeth..

Type I Type I

Type IIType II

Type IIIType III

How do people inherit How do people inherit dentinogenesis imperfecta?dentinogenesis imperfecta?

This condition is inherited in an autosomal This condition is inherited in an autosomal dominant pattern, which means one copy dominant pattern, which means one copy of the altered gene in each cell is sufficient of the altered gene in each cell is sufficient to cause the disorder.to cause the disorder.

In most cases, an affected person has one In most cases, an affected person has one parent with the condition.parent with the condition.

TreatmentTreatment

One treatment option is bonding, putting lighter One treatment option is bonding, putting lighter enamel on the weakened enamel of the teeth enamel on the weakened enamel of the teeth and with lots of treatments of this bonding, the and with lots of treatments of this bonding, the teeth appear whiter to the eye, but the teeth on teeth appear whiter to the eye, but the teeth on the inside and under that cover are still the the inside and under that cover are still the same.same.Due to the weakened condition of the teeth, Due to the weakened condition of the teeth, many common cosmetic procedures such as many common cosmetic procedures such as braces and bridges are inappropriate for patients braces and bridges are inappropriate for patients with Dentinogenesis imperfecta and are likely to with Dentinogenesis imperfecta and are likely to cause even more damage than the situation they cause even more damage than the situation they were intended to correct.were intended to correct.