Dentin Dysplasia Type II 1

Definition: Genetic disturbances in dentin formation leading to altered morphology of dentin (very similar to dentinogenesis imperfecta).  There are two types: type I is radicular and type II is coronal indicating the portion of the tooth with the most changes.  This is an autosomal-dominant abnormality of the DSPP gene.  Dentin dysplasia is less common than dentinogenesis imperfecta.

Radiographic Features:

Location: Dentin and pulp chamber/root canal space.

Edge: Well-defined.

Shape: Obliteration of the pulp chamber and root canal space after eruption of the tooth. The pulp chamber may have a ‘thistle’ or ‘flame’ shape.

Internal: Radiopaque, same radiopacity as dentin.  No or small pulp chamber and root canals present.

Other: May or may not have pulp stones present.

Number: Associated with all teeth in the oral cavity.

(click image to enlarge)

Dentin Dysplasia Type II

(note the ‘flame’ shaped pulp chamber and obliterated root canal space)

Dentin Dysplasia Type II

(note the ‘thistle’ or ‘flame’ shaped pulp chamber and obliterated root canal space)