2. DENTINOGENESIS IMPERFECTA
• 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 teeth and
permanent teeth.
3. • 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.
• Dentinogenesis imperfecta represents a group of hereditary
conditions that are characterized by abnormal dentin formation.
• These conditions are genetically and clinically heterogenous and can
affect only the teeth or can be associated with the condition
osteogenesis imperfecta.
DENTINOGENESIS
IMPERFECTA
5. TYPE 1
Type 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.
6. TYPE 2
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.
7. C L I N I C A L F E AT U R E S
• Clinical appearance is variable.
• 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.
8. R A D I O G R A P H I C F E AT U R E S
• 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
9. Histology
• Dentinal tubules are irregular and are
bigger in diameter.
• Areas of uncalcified matrix are seen.
• Sometimes odontoblasts are seen in dentin.
10. Treatment
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.
before after
