Radiographically, evidence of partial pulp obliteration with a "thistle-shaped appearance". Moderate type
Teeth are moderately discoloured (blue, grey or amber opalescent). More attrition is evident with shortening of crown height. Crowns may appear bulbous with prominent constriction at the CEJ. Radiographically, the pulp is small or is totally obliterated. Roots appear thinner and shorter than average. There may be periapical pathology. Severe type
Teeth are markedly discoloured (brown opalescent). The crowns are very short due to severe attrition. Crowns may appear bulbous with prominent constriction at the CEJ. Radiographically, pulp appears large and the dentine layer is thin ("shell teeth" as described in Presentation section). Roots are thin and short. There may be multiple periapical pathologies. Radicular dentin dysplasia
This sub-type is used in place of Shields dentine dysplasia type I, in which only the roots of the teeth are affected. Both primary and permanent teeth are affected. The teeth appear normal clinically. Radiographically, the roots are shorter and fused together with a rounded apex. Presentation
Clinical presentation
Clinical features include:

Discoloured teeth - teeth may be amber, brown, blue or opalescent
Bulbous shape to the tooth crown due to cervical constriction
Tooth wear/Non-carious tooth surface loss (NCTSL) - due to the poorly mineralised dentine, the enamel of the tooth is unsupported and subsequently shears or chips off as it is subjected to biting forces. This exposes the underlying poorly mineralised dentine which is less resistant to wear. Therefore, features of abrasion and attrition may become apparent. Reduction in occlusal vertical dimension (OVD) - this is secondary to the tooth wear/NCTSL.
Dental whitening (bleaching) is contraindicated although it has been reported to lighten the color of DI teeth with some success; however, because the discoloration is caused primarily by the underlying yellow-brown dentin, this alone is unlikely to produce normal appearance in cases of significant discoloration.If there is considerable attrition, overdentures may be prescribed to prevent further attrition of remaining teeth and for preserving the occlusal face height. Management of DI associated with OI
Bisphosphonates have recently been introduced to treat several bone disorders, which include osteogenesis imperfecta.A recognized risk of this drug relevant to dental treatments is bisphosphonate-associated osteonecrosis of the jaw (BRONJ). Occurrences of this risk is associated with dental surgical procedures such as extractions. Dental professionals should therefore proceed with caution when carrying out any dental procedures in patients who have Type 2 DI who may be on bisphosphonate drug therapy. See also
Dentin
Dentinogenesis
Tooth development
Osteogenesis imperfecta

References

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


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