Amelogenesis Imperfecta Final

Unversity of kirkuk Oral Histology
College of Dentistry Final Report
C C
.‫ عبدهللا محمد خليل حسن‬: ‫اسم الطالب‬

.‫ الثانية‬: ‫المرحلة‬
2019/2020
0
Amelogenesis imperfecta (AI): Oral Histology
Amelogenesis imperfecta (AI) is a group of conditions caused by defects in the

genes that encode enamel matrix proteins or other proteins or enzymes required to
process or mineralize the matrix affecting all or most of the teeth of both deciduous and
permanent dentitions to some extent.
 Amelogenesis imperfecta is due to the malfunction of the proteins in the enamel

(ameloblastin, enamelin, tuftelin and amelogenin) as a result of abnormal
enamel formation via amelogenesis.
 The dentin and cementum are not affected. Systemic illness, malnutrition, and
environmental factors play no role in its cause; instead, the condition is caused by
the defective development of enamel, which has a genetic basis.
 For unknown reasons, the deciduous dentition is often less severely affected than
the permanent dentition; hence, in many cases, the condition may not be
identified until the eruption of the permanent teeth.
Classification
 Based on type of defect (phenotype)
 Hypoplastic Amelogenesis imperfecta
 Hypocalcified amelogenesis imperfecta:
 Hypomaturation amelogenesis imperfecta
 Hypomature hypoplastic enamel with taurodontism
 Based on pattern of inheritance (genotype)

 Autosomal dominant trait
 Autosomal recessive. trait
 X-linked trait.
1
Enamel development is a three-stage process that begins with enamel matrix

deposition, transitioning to mineralization of the matrix, and ending with
final hardening (maturation) of the enamel layers. Each stage is controlled by
separate genetic mechanisms, and a defect in the genetic control at any of the
three stages will produce amelogenesis imperfecta. Therefore, amelogenesis
imperfecta can be subdivided into groups depending on which stage of enamel
production is disrupted by the genetic defect:-
1. TypeI Hypoplastic Amelogenesis imperfecta:
 The enamel is reduced in amount but normally mineralized, because the

main defect in this type is deficient formation of matrix.
 The enamel is randomly pitted, grooved or uniformly very thin, but hard and
translucent. The defects tend to become stained.
 The teeth are not especially susceptible to caries unless the enamel is scanty
and fractures to expose dentine.
 The main patterns of inheritance are autosomal dominant and autosomal

recessive, X-linked and (a genetic rarity) an X-linked dominant type. In the
last type, there is almost complete failure of enamel formation in affected
males, whereas in females the enamel is vertically ridged.
Hypoplastic vertical grooves in a female with X-linked Amelogenesis imperfecta, hypoplastic pitted
amelogenesis imperfecta. type. Enamel between pits appears normal.
2
2. TypeII Hypocalcified amelogenesis imperfecta:
 Enamel matrix is formed in normal quantity but is poorly calcified. When

newly erupted, the enamel is normal in thickness and form,
 The enamel is extremely brittle,weak or chalky and opaque in appearance.

 Teeth are prone to staining and rapid wear, exposing dentine.
 Hypocalcified forms of amelogenesis imperfecta affect the deposition of

inorganic elements onto the organic enamel matrix.
 This is an uncommon form of amelogenesis imperfecta, and only two types

have been identified. Both types tend to show diffuse involvement of the
teeth.
 Autosomal dominant and autosomal recessive inheritances are seen in these

two forms. Both tend to manifest clinically as a diffuse, mottled color change
of the enamel.
Amelogenesis imperfecta, hypocalcified type. The soft chalky

enamel was virtually of normal thickness and form but has
chipped away during mastication leaving a characteristic shoulder,
seen best on the upper left central incisor.
3
3. Type III Hypomaturation amelogenesis imperfecta:
 The enamel is normal in thickness on eruption but with opaque, white to

brownish-yellow patches caused by failure of maturation, a process of matrix
removal and increasing mineralisation that is partly developmental and partly
posteruptive.
 The appearance can mimic fluorotic mottling if the spots are small.
 However, affected enamel is soft and vulnerable to attrition, though not as
severely as the hypocalcified type.
 Pitting or cavitation is generally not present in the hypomaturation types.
 Two of the genetic patterns produce a distinctive “snow-capped” appearance
in the enamel.
 Autosomal recessive and X-linked recessive inheritance patterns have been
confirmed in two of the types of Hypomaturation.
Amelogenesis imperfecta, one of the several Amelogenesis imperfecta, hypomaturation type. Tooth
morphology is normal, but there are opaque white and
hypomaturation types. In this form there are
discolored patches
opaque white flecks and patches affecting the
occlusal half of the tooth surface.
4. Type IV - Hypomature hypoplastic enamel with taurodontism
 Enamel has a variation in appearance, with mixed features from Type I and
Type II AI.
 All Type 4 AI has taurodontism in common.
 Condition is of autosomal dominant pattern.
 Other common features may include an anterior open bite, , sensitivity of teeth.
4
Clinical features
 The clinical appearance of the various types of amelogenesis

imperfecta can be remarkably different.
 In some types the teeth appear essentially normal, whereas in others they
may be extremely unsightly and obviously abnormal.
 Both dentitions are commonly affected to some degree.
 In the X-linked subtypes the clinical appearance differs between males
and females.
Radiographic features
The radiologic appearance of amelogenesis imperfecta varies with the type.
 In the smooth hypoplastic type, the enamel layer is conspicuously thin

and its radiodensity is greater than the adjacent dentin.
 In the hypocalcified type, the enamel layer appears wispy or absent and is
usually less radiodense than the adjacent dentin.
 In the hypomaturation type, the radio-density of the enamel is almost

equal to that of normal dentin.
 In general, the enamel seen on the radiographs appears thinner than

normal, and its density more closely approximates that of the dentin in
many of the forms. Therefore, no sharp demarcation between enamel and
dentin may be evident.
 Older patients may exhibit complete loss of evidence of enamel.
5
Treatment
 Long-term treatment planning needs to be undertaken as the condition

will require treatment and re-treatment throughout life.
 Where there is severe hypoplasia so that the crowns are smaller than
normal or where the teeth are subject to occlusal wear as a result of a
mineralization defect, placement of stainless steel crowns on the first
molars should be considered once these teeth are fully erupted.
 Acid-etch composite facings on the labial aspects of the maxillary central

and lateral incisors can improve the aesthetics
 More definitive treatment can include porcelain veneers on the anterior

teeth as well as full crowns on premolars and molars.
 Attention may need to be paid to gingival health; some females with XAI
have difficulty in maintaining oral hygiene because of plaque
accumulation in hypoplastic grooves at the gingival margin.
 If there is taurodontism and endodontic treatment is required, navigation

into the apical canals can pose a problem in some patients.
 In the worst-case scenario, the teeth may have to be extracted and

implants or dentures are required. Loss of nerves in the affected teeth may
occur.
6
References
 Dental and Oral Pathology.

 General and Oral Pathology for the Dental Hygienist Second
Edition (December 27, 2012) - Leslie DeLong (Author), Nancy
Burkhart (Author)
 Cawson's Essentials of Oral Pathology and Oral Medicine.
 Wikipedia,

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Unversity of kirkuk Oral Histology

College of Dentistry Final Report

.‫ عبدهللا محمد خليل حسن‬: ‫اسم الطالب‬

Amelogenesis imperfecta (AI) is a group of conditions caused by defects in the

 Amelogenesis imperfecta is due to the malfunction of the proteins in the enamel

 Based on pattern of inheritance (genotype)

Enamel development is a three-stage process that begins with enamel matrix

1. TypeI Hypoplastic Amelogenesis imperfecta:

 The enamel is reduced in amount but normally mineralized, because the

 The main patterns of inheritance are autosomal dominant and autosomal

2. TypeII Hypocalcified amelogenesis imperfecta:

 Enamel matrix is formed in normal quantity but is poorly calcified. When

 The enamel is extremely brittle,weak or chalky and opaque in appearance.

 Hypocalcified forms of amelogenesis imperfecta affect the deposition of

 This is an uncommon form of amelogenesis imperfecta, and only two types

 Autosomal dominant and autosomal recessive inheritances are seen in these

Amelogenesis imperfecta, hypocalcified type. The soft chalky

3. Type III Hypomaturation amelogenesis imperfecta:

 The enamel is normal in thickness on eruption but with opaque, white to

4. Type IV - Hypomature hypoplastic enamel with taurodontism

 The clinical appearance of the various types of amelogenesis

The radiologic appearance of amelogenesis imperfecta varies with the type.

 In the smooth hypoplastic type, the enamel layer is conspicuously thin

 In the hypomaturation type, the radio-density of the enamel is almost

 In general, the enamel seen on the radiographs appears thinner than

 Older patients may exhibit complete loss of evidence of enamel.

 Long-term treatment planning needs to be undertaken as the condition

 Acid-etch composite facings on the labial aspects of the maxillary central

 More deﬁnitive treatment can include porcelain veneers on the anterior

 If there is taurodontism and endodontic treatment is required, navigation

 In the worst-case scenario, the teeth may have to be extracted and

 Dental and Oral Pathology.

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