Clinical presentation Clinical features include: *<unk> scoloured 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<unk> mension (OVD) - this is secondary to the tooth wear/NCTSL. A reduced OVD can lead to craniofacial dysgnathia, poor tooth aesthetics, and<unk> sorders during chewing, swallowing, speaking and eating. The baby (primary) teeth are usually more severely affected than adult (permanent) teeth. Enamel is usually lost early because it is further inclined to attrition due to loss of scalloping at the dentinoenamel junction (DEJ). It was suggested that the scalloping is beneficial for the mechanical properties of teeth as it reinforces the anchor between enamel and dentine. However, the teeth are not more susceptible to dental caries than normal ones. Periodontal<unk> sease, or gum<unk> sease, is a common finding amongst individuals with<unk>  despite no clinical findings of tooth decay (dental caries). The reason for this is currently not well understood. Certain patients with<unk>  will suffer from multiple periapical abscesses apparently resulting from pulpal strangulation secondary to pulpal obliteration or from pulp exposure due to extensive coronal wear. They may need apical surgery to save the involved teeth. Note that, although dentine exposure is a common clinical finding, individuals with<unk>  usually do not experience tooth sensitivity as the exposed dentine is typically sclerosed (hardened), thereby appearing glassy/shiny. Radiographic presentation Radiographic features include: * Bulbous shape of tooth crown with pronounced cervical constriction * Small pulp, or total pulp obliteration * Small or obliterated root canal * Presence of pulp stones * Narrow and small roots * Periapical radiolucency without any evidence of clinical pathology such as tooth decay (dental caries) Presentation by sub-type of<unk>  Clinical and radiographic features can be categorised by the sub-type of<unk>  (see Shield's Classification in the Classification section): Type I Clinically, both the baby (primary) and adult (permanent) teeth often appear amber coloured and translucent, and show signs of severe attrition. Primary teeth have a more obvious appearance as they have a thinner layer of enamel overlying dentine, hence the abnormal color of dentine is more noticeable. Radiographically, affected teeth have short and narrow roots, and obliterated pulps due to dentine hypertrophy before or shortly after tooth eruption. The severity of these features is variable, with some teeth presenting with total obliteration of the pulp, while other teeth appear to have normal, healthy dentine. Some type I cases present with no clinical findings, with only radiographic abnormalities. Type II Type II has a similar clinical and radiographic appearance to type I with some<unk> stinguishing features: * Bulbous crowns are common with pronounced cervical constriction * All teeth in the mouth are affected, with severe abnormalities present in both the baby (primary) and adult (permanent) teeth. This is in contrast to type I where the presentation is more variable *Rarely, individuals exhibit sensorineural hearing loss. It is proposed this hearing loss is a secondary feature to attrition; this type of tooth wear can cause jaw overclosure with subsequent changes to the shape of the inner ear, thus causing hearing loss. However, the true cause remains unknown. Type III Similar clinical and radiographic features to that of type I and II are apparent for the adult (permanent) teeth. The main<unk> stinguishing feature is "shell teeth", a term used to describe the unique appearance of the baby (primary) teeth; the primary teeth have multiple pulp exposures and radiographically appear hollow as the dentine layer is thin (dentine hypotrophy) and the pulp chamber is very large.
Dentinogenesis imperfecta
Kyrle disease
Osteogenesis imperfecta
Rhizomelic chondrodysplasia punctata
Chondrodysplasia punctata
Fuchs' dystrophy
Thiel–Behnke dystrophy
Iododerma