Amelogenesis Imperfecta
Author :
Trishul Allareddy BDS, MS, Dipl. ABOMR.
Clinical Professor and Director of Oral and Maxillofacial Radiology
veeratrishul-allareddy@uiowa.edu
Published on : 17 Sep 2019
Amelogenesis Imperfecta:
- Developmental condition which results in alterations of enamel formation in teeth.
- Results due to mutations in enamel forming genes.
- Localized or generalized.
- In the literature at least 14 variants of this condition have been reported.
The most common ones are:
- Hypoplastic
- Hypomineralized
- Hypomaturation
- Hypoplastic-hypomaturation
Hypoplastic:
- There is an inadequate deposition of enamel matrix.
- The enamel is thin due to the failure to develop to its normal expected thickness.
- Crowns appear heavily abraded and are often square shaped with increased interproximal contact space due to the lack of enamel thickness.
Hypomineralized:
- There is an inadequate mineralization of the enamel matrix.
- The enamel has a similar or less density to that of dentin.
- The enamel is soft and is susceptible to easily being worn away.
- Often the dentin is also soft and subject to easy wear and/or fracture of the crown.
- Teeth are often discolored in this condition.
Hypomaturation:
- The enamel matrix shows an inadequate maturation of the enamel matrix resulting in a mottled enamel which is of normal thickness.
- Enamel has similar density to that of dentin.
- Teeth are often discolored and may be cloudy white, yellow or brown.
Hypomaturation/hypoplastic:
- There is a combination of both inadequate deposition and mineralization of the enamel matrix.
- Taurodontism may be seen.
Radiographic features:
- Enamel is thin.
- Enamel have same density as that of dentin in the hypomaturation type.
- Interproximal spaces between teeth with square shaped crowns resulting in a “picket fence” appearance in the hypoplastic type.
- Teeth are often heavily worn away.
- Teeth may show pitted or irregular enamel areas in the hypomineralized type.
Management:
- Full mouth rehabilitation with crowns as preferred choice.
- Often the crowns will fail requiring extraction and replacement of teeth.
- It is preferred to not extract teeth until somatic growth ceases.
- Mild cases may be treated with veneers.
Hypoplastic Amelogenesis Imperfecta
Panoramic reconstruction of the CBCT: Shows multiple unerupted
Panoramic reconstruction from a CBCT
Note the lack of enamel with a picket fence appearance. The molars show taurodontism
Full mouth series
Multiple teeth with poorly formed enamel requiring full mouth crowns