DENTINOGENESIS IMPERFECTA(HEREDITARY OPALESCENT DENTIN) ESMINO, PRECIOUS GRACE H. DMD2D
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.
• 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
TYPE 1Type of dentinogenesis imperfectawith similar dental formalitiesusually an autosomal dominanttrait with variable expressivity butcan be recessive if the associatedosteogenesis imperfecta isof recessive type.
TYPE 2Occurs 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.
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.
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
Histology• Dentinal tubules are irregular and are bigger in diameter.• Areas of uncalcified matrix are seen.• Sometimes odontoblasts are seen in dentin.
Treatment One treatment option is bonding, putting lighter enamel on theweakened 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 underthat cover are still the same. Due to the weakened condition of the teeth, many commoncosmetic procedures such as braces and bridges areinappropriate for patients with Dentinogenesis imperfecta and arelikely to cause even more damage than the situation theywere intended to correct. before after