Dentinogenesis imperfecta

Dentinogenesis imperfecta
Dentinogenesis imperfecta
Classification and external resources
ICD-10 K00.5
ICD-9 520.5
MeSH D003811

Dentinogenesis imperfecta (hereditary Opalescent Dentin) is a genetic disorder of tooth development. This condition causes teeth to be discolored (most often a blue-gray or yellow-brown color) and translucent. Teeth are also weaker than normal, making them prone to rapid wear, breakage, and loss. These problems can affect both primary (baby) teeth and permanent teeth. This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. Dentinogenesis imperfecta affects an estimated 1 in 6,000 to 8,000 people.



Types of dentinogenesis imperfecta with similar dental formalities usually an autosomal dominant trait with variable expressivity but can be recessive if the associated osteogenesis imperfecta is of recessive type. This type is no l

Type II : Occurs in people without other inherited disorders (i.e. Osteogenesis imperfecta).It is an autosomal dominant trait. A few families with type II have progressive hearing loss in addition to dental abnormalities.

Mutations in the DSPP gene have been identified in people with type II and type III dentinogenesis imperfecta. Type I occurs as part of osteogenesis imperfecta.

Clinical features

Clinical appearance is variable. However, the teeth usually involved and more severely affected are deciduous teeth in type 1; whereas in type 2 both the dentitions are equally affected.

The teeth may be gray to yellowish brown. They exhibit translucent or opalescent hue. Enamel is usually lost early due to loss of scalloping at the DEJ. However, the teeth are not more susceptible to dental caries than normal ones.

Radiographic features

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

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


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


One treatment option is bonding, putting lighter enamel on the weakened enamel of the teeth and with lots of treatments of this bonding, the teeth appear whiter to the eye, but the teeth on the inside and under that cover are still the same.

Due to the weakened condition of the teeth, many common cosmetic procedures such as braces and bridges are inappropriate for patients with Dentinogenesis imperfecta and are likely to cause even more damage than the situation they were intended to correct.

This article incorporates public domain text from The U.S. National Library of Medicine

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Look at other dictionaries:

  • dentinogenesis imperfecta — dentinogenesis im·per·fec·ta .im pər fek tə n a disorder of tooth development inherited as an autosomal dominant and characterized by relatively soft enamel in both the primary and permanent teeth that makes the teeth abnormally vulnerable to… …   Medical dictionary

  • dentinogénesis imperfecta — Displasia hereditaria de la dentina de los dientes deciduos y permanentes, en la que crece un exceso de dentina opalescente, que oblitera la pulpa. Los dientes tienen raíces cortas y se desprenden rápidamente. Diccionario Mosby Medicina,… …   Diccionario médico

  • Dentinogenesis imperfecta — Klassifikation nach ICD 10 K00.5 Hereditäre Störungen der Zahnstruktur, anderenorts nicht klassifiziert Dentinogenesis imperfecta …   Deutsch Wikipedia


  • dentinogenesis — The process of dentin formation in the development of teeth. [dentin + G. genesis, production] d. imperfecta [MIM*125490 & MIM*125500] an autosomal dominant disorder of the teeth characterized clinically by translucent …   Medical dictionary

  • dentinogenesis — n. the formation of dentine by odontoblasts. Although dentinogenesis continues throughout life, very little dentine is formed later than a few years after tooth eruption unless it is stimulated by caries, abrasion, or trauma. Dentinogenesis… …   The new mediacal dictionary

  • odontogenesis imperfecta — dentinogenesis imperfecta …   Medical dictionary

  • Osteogenesis imperfecta — Classification and external resources The classic blue sclerae of a person with osteogenesis imperfecta ICD 10 Q …   Wikipedia

  • Osteogenesis imperfecta — Klassifikation nach ICD 10 Q78.0 Osteogenesis imperfecta …   Deutsch Wikipedia

  • Amelogenesis imperfecta — Classification and external resources Amelogenesis imperfecta, hypoplastic type. Note the association of pitted enamel and open bite. ICD 10 K …   Wikipedia

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