Dentinogenesis imperfecta is a disorder of tooth growth. This circumstance is inherited in an autosomal predominant form, which means one transcript of the altered gene in each cubicle is adequate to induce the disorder. There are three types of dentinogenesis imperfecta. Type I is associated with osteogenesis imperfecta. Type II was previously found to be linked to altered glycosaminoglycan concentrations. Type III is the brandywine form, named for the city brandywine, maryland, where there was a large population of patients with this disorder. Type III tends to be less severe than type II. The severity of discoloration and enamel fracturing in all dentinogenesis imperfecta types is highly variable even within the same family. Genetic mutations, or alterations in an individual’s genome, can be inherited, affecting cells that perpetually divide (germ-line mutations), or they can occur at any point during a person’s life.
In most cases, a stricken individual has one parent with the circumstance. This circumstance causes the teeth to be discolored (almost frequently a blue-gray or yellow-brown tone) and translucent. It is usually an autosomal dominant trait with variable expressivity but can be recessive if the associated osteogenesis imperfecta is of recessive type. 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. Due to the lack of support of the poorly mineralized underlying dentin, the enamel frequently fractures from the teeth leading to rapid wear and attrition of the teeth. 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.
Early and accurate diagnosis of dentinogenesis imperfecta is urgent to enable proper preventative interventions and optimum dental handling. If left raw it is not rare to view the whole DI affected dentition ratty away to the gingiva. Providing optimal oral health treatment for dentinogenesis imperfecta frequently includes preventing severe attrition associated with enamel loss and rapid wear of the poorly mineralized dentin, rehabilitating dentitions that have undergone severe wear, optimizing esthetics,and preventing the common dental problems associated with caries and periodontal disease. Although pulpal pathosis is rarely reported with dentinogenesis imperfecta, endodontic treatment is occasionally necessary and has a guarded prognosis if initiated after pulp canal obliteration has occurred. Management of permanent dentinogenesis imperfecta teeth with fracturing and excessive wear can be treated with porcelain fused to metal crowns.
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