Occurrence:
(1) as a component of some types of osteogenesis imperfecta (seen in Types IB, IIIB and IVB)
(2) inherited as an isolated autosomal dominant trait
(3) as a component of another inherited syndrome (Ehlers-Danlos syndrome type II, Goldblatt syndrome, Schimke immuno-osseous dysplasia, skeletal dysplasia with opalescent and rootless teeth, cortical defects with wormian bones)
Features:
(1) dental discoloration (an opalescent blue or brown)
(2) dental deformities
(3) malocclusion of the teeth and crossbite
(4) excessive wearing away of the teeth due to flaking off of the dentine
(5) dental fracture associated with poor mechanical strength of the dentine
Dental deformities:
(1) pulpal obliteration (narrow and "flame-shaped")
(2) short and narrow roots
(3) bulbous, bell-shaped crowns
(4) constriction at the cementoenamel junction (on radiographs)
The defect may affect:
(1) both the deciduous and permanent dentition
(2) only the deciduous dentition
(3) only the permanent dentition
Caries are may not be a problem in untreated teeth that erode before the caries can develop.
