Aesthetic Rehabilitation of Young Female Patient with Congenital Amelogenesis Imperfecta

Amelogenesis Imperfecta a rare condition associated with the Teeth Do you know -

                                           

 

• Whats that ......

• Amelogenesis imperfecta (AI) is a congenital disorder which presents with a rare abnormal formation of the enamel or external layer of the crown of teeth, unrelated to any systemic or generalized conditions
• 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 .

•  Teeth with abnormal color: yellow, brown or grey; this disorder can afflict any number of teeth of both dentitions.

• The teeth have a higher risk for dental cavities and are hypersensitive to temperature changes as well as rapid attrition, excessive calculus.

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Genetic Predisposition ..AMELX and ENAM encode extracellular matrix proteins of the developing tooth enamel , MMP20 encode proteases that help degrade organic matter from the enamel matrix during the maturation stage of amelogenesis.SLC24A4 encodes a calcium transporter that mediates calcium transport to developing enamel during tooth development.







Type 1 - Hypoplastic-


 Enamel is very thin but hard & translucent, and may have random pits & grooves.


Type 2 - Hypomaturation-


Enamel has sound thickness, with a pitted appearance. It is less hard compared to normal enamel, and are prone to rapid wear


Type 3 - Hypocalcified


Enamel defect due to malfunction of enamel calcification, therefore enamel is of normal thickness but is extremely brittle, with an opaque/chalky presentation.


Type 4 - Hypomature hypoplastic enamel with taurodontism 
 ( The crown of the teeth is enlarged at the expense of the root.
Bull Like tooth )




 Type 1 and Type 2 AI. All Type 4 AI has taurodontism in common

Treatment-

Preventive and restorative dental care is very important as well as considerations for esthetic issues since the crown are yellow from exposure of dentin due to enamel loss . 

Full-coverage crowns are sometimes being used to compensate for the abraded enamel in adults, tackling the sensitivity the patient experiences

Epidemiology-

The exact incidence of amelogenesis imperfecta is 1 in 14,000 people in the United States

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