Dentinal dysplasia

Date: 2015-10-07; view: 741.

Dentinal dysplasia was first described in the 1920s; a 'rootless teeth' is an alternative, descriptive title. The condition is an autosomal dominant trait with both dentitions being affected. The teeth may be clinically normal but root formation is abnormal to varying degrees. Some teeth may have extremely short blunt roots, others taper markedly towards the apex. The pulp is partially obliterated, appearing in the molar teeth as a small demilune. Under the microscope the coronal dentine is normal but the root dentine is not, with masses of abnormal dentine obliterating the pulp space. The microscopic appearance has been compared a flowing stream. The short roots may cause problems because of mobility and this may lead to the condition being identified. The above mentioned condition is often referred to as a dentinal dysplasia type I to distinguish it from a condition referred to by some as a dentinal dysplasia type II (coronal dentinal dysplasia). The latter is most likely to represent an allelic variant of dentinogenesis imperfecta as genetic linkage studies have shown it to map to the same region of chromosome 4 as dentinogenesis imperfecta and a mutation in the DSPP gene has also been identified in one family diagnosed as having dentinal dysplasia type II. It remains to be seen whether dentinal dysplasia (type I) is also allelic to dentinogenesis imperfecta.

Treatment

As with enamel defects there may be severe psycho-social problems as a result of the appearance of the teeth. Many of the arguments presented in the consideration of enamel conditions will apply to dentine also. In dentinogenesis imperfecta, management is focused on the prevention of tooth wear, the maintenance of the vertical dimension and improvement of the appearance.

Thus, dental anomalies may have both a functional and psycho-social impact on the child and their family. The presence of one dental anomaly may be associated with others. Thorough clinical examination and radiographic investigations are essential. An anomaly in the primary dentition may be associated with a similar anomaly in the permanent dentition. All cases of missing teeth require treatment planning with multidisciplinary input. Dental professionals have an important part to play in the diagnosis and care of children with these conditions. Careful monitoring of dental development, together with interception when appropriate, may reduce the impact of these conditions.