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Disorders of Dentine and Eruptive Defects

Inherited Disorders of Dentine

All the different anomalies of dentine are inherited and are principally related to disorders of collagen metabolism but are phenotypically very similar.

Dentinogenesis Imperfecta (DI) and Hereditary Opalescent Dentine

These are inherited disorders of dentine and collagen metabolism. Clinically identical conditions, the term dentinogenesis imperfecta is usually reserved for the condition associated with osteogenesis imperfecta and hereditary opalescent dentine for the isolated condition. The appearance is characteristic with an amber or grey to blue opalescent colour of the crown (Fig. 44.1). The enamel is normal but will chip and expose the weaker and abnormal dentine that wears rapidly. Over time there is pulp canal obliteration and radiographically the crowns are bulbous (Fig. 44.2).

Figure 44.1 Dentinogenesis imperfecta with the typical appearance of grey–bluish opalescence of the teeth with enamel present and yellow–brown exposed dentine.

Figure 44.2 Classical appearance of an adolescent with dentinogenesis imperfecta. Note the bulbous crowns and shortened roots. Over time the initially wide-open apices and thin root canals become obliterated.

Management

It is essential that the enamel of these teeth is protected and early placement of stainless steel crowns on primary molars is recommended (Fig. 44.3). The anterior teeth may be built up with composite resin strip crowns although over time these are lost and some form of full-coverage restoration is required. Initially, the appearance of the permanent dentition is good, however, these teeth tend to deteriorate in early adult life necessitating complex restorative work throughout a lifetime.

Figure 44.3 Stainless steel crowns are invaluable in the restoration of these teeth. Once the enamel is lost the dentine wears rapidly making long-term retention poor.

Osteogenesis Imperfecta (OI)

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