36: Multiple missing and abnormally shaped teeth

36 Multiple missing and abnormally shaped teeth


Ellen is a regular attender with no caries. Her oral hygiene is excellent. There is no history of tooth extraction in either the primary or permanent dentitions.

Medical history

Ellen has no medical problems. She is an active girl who is involved in orienteering with her family as well as being in the local amateur dramatic society.

image There are a significant number of syndromes of the head and neck that manifest with missing teeth. Can you name some?

Ectodermal dysplasia describes a group of inherited disorders involving ectodermally derived structures, i.e. hair, teeth, nails, skin and sweat glands. The most common form is the hypohidrotic X-linked form. The usual presentation is a male child with:

Down syndrome affects 1 in 700 births and is commoner in offspring of older mothers, and is a result of chromosomal translocation. Apart from the morphological features characteristic of the syndrome, there is commonly delayed eruption and generalized microdontia as well as hypodontia of some teeth.

The diagnosis and management of all dental anomalies is extremely important and should not be delayed. Genetic consultation is often desirable to not only confirm the diagnosis but also to help parents understand the risk of future offspring and generations being affected. Conversely, geneticists may require help from paediatric dentists to clarify a diagnosis.

Jan 2, 2015 | Posted by in Orthodontics | Comments Off on 36: Multiple missing and abnormally shaped teeth
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