Amelogenesis Imperfecta

Definition:
Amelogenesis imperfecta is a tooth development disorder in which the teeth are covered with thin, abnormally formed enamel.

Amelogenesis imperfecta presents with abnormal formation of the enamel.
Enamel is composed mostly of mineral, that is formed and regulated by the proteins in it.
Amelogenesis imperfecta is due to the malfunction of the proteins in the enamel: ameloblastin, enamelin, tuftelin and amelogenin.
People afflicted with amelogenesis imperfecta have teeth with abnormal color: yellow, brown or grey.
The teeth have a higher risk for dental cavities and are hypersensitive to temperature changes.
This disorder can affect any number of teeth.

Types:
1- Hypoplastic AI:
Inadequate deposition of enamel matrix.
Enamel matrix present is normal.
Enamel may be "Thin or Pitted".
The thin enamel may be "Smooth or Rough".
Generalized small pits scattered across surface of teeth or localized large area of hypoplastic enamel typically on the buccal middle third of the tooth.
Smooth enamel exhibits a smooth surface which is thin, hard and glossy or rough.
The enamel is more dense than that of smooth pattern.
Most commonly is autosomal dominant inheritance.

2- Hypmaturation AI:
Adequate deposition and mineralization of enamel matrix but inadequate maturation of crystal structure (mineral).
Soft enamel with similar radiodensity to underlying dentin .
Pigmented enamel has mottled , brown appearance .
Snow-Capped Teeth: Zone of opaque white enamel on the incisal / occlusal end of the teeth

3- Hypocalcified :
No mineralization occurs.
Enamel is soft and easily lost.
Enamel is dull, lustreless, opaque white, honey or browned colour.
Enamel and dentin appear radiographically indistinct.

4- hypomaturation hypoplastic:

Causes:
Amelogenesis imperfecta is a hereditary Disorder.

Symptoms:
The teeth enamel become soft and thin.
The teeth appear yellow.
The teeth become easily damaged.
Both baby teeth and permanent teeth are affected.

Differential Diagnosis:
The most common differential diagnosis with the Amelogenesis Imperfecta is the Dental Fluorosis.
- The variability of the fluorosis make it very difficult to differentiate.
- It varies from mild white "flecking" of the enamel to profoundly dense white colouration with random, disfiguring areas of staining and hypoplasia.
- Requires careful questioning to distinguish from Amelogenesis Imperfecta.
- Fluorosis may present with areas of horizontal white banding corresponding to periods of more intense fluoride intake and may show the premolars or second permanent molars to be spared (chronological distribution).
- The history will often reveal excessive fluoride intake either in terms of a habit such as eating toothpaste in childhood, or related to a local water supply.

Treatment:
The treatment depends on the severity of the problem.
The treatment starts from simple composite restorations until a Full crown restorations that will improve the appearance of the teeth and protect them from damage.
Treatment is often successful in protecting the teeth.
The teeth may have to be extracted and implants or dentures are required.
Generally, treatment is veneers , full coverage , overdenture, full denture or extraction and implant.

Complications:
The enamel is easily fractured and damaged, which affects the appearance of the teeth, especially if left untreated.

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Authored by :: World of Dentistry :: Team