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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

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