AMELOGENESIS IMPERFECTA

Presented by:

HARON, Sittie Aainaa M.

AMELOGENESIS IMPERFECTA (AI)

DEFINITION:• disorder of tooth development, abnormal enamel formation.•AI defects include abnormalities that are classified as hypoplastic (defect in amount of enamel), hypomaturation (defect in final growth and maturation of enamel crystallites), and hypocalcified (defect in initial crystallite formation followed by defective growth)• It is due to the malfunction of the proteins in the enamel: ameloblastin, enamelin, tuftelin and amelogenin.

ALSO CALLED: ENAMEL DYSPLASIA

SIGNS AND SYMPTOMS

• The enamel of the tooth is soft and thin.• Teeth appear yellow and are easily damaged.• Both deciduous teeth and permanent teeth are affected.• brown or white chalky discoloration of the teeth

This condition causes teeth to be unusually small, discolored, pitted or grooved, and prone to rapid wear and breakage

DENTAL CORRELATION

• Full crowns will improve the appearance of the teeth and protect them from damage.

compensate for the soft enamel stainless steel crowns are used

in children

porcelain for adults• teeth may have to be extracted and implants or dentures are required.

As a dentist, we are expected to diagnose and treat this condition:

CLASSIFICATION:MULTIFACTORIAL - six genes are known to cause Amelogenesis Imperfecta• AMELX, ENAM, MMP20, KLK4, FAM83H, and WDR72

MENDELIAN - the different types of AI are classified according to their mode of inheritance.

• Autosomal Dominant Inheritance• Autosomal Recessive Inheritance• X-linked Recessive Inheritance

Type Clinical Appearance Enamel Thickness Radiographic Appearance

Inheritance

Hypoplastic (Type I)

Crowns size varies from small to normal, small teeth may lack proxmial contacts, color varies from normal to opaque white – yellow brown

Varies from thin and smooth to normal thickness with grooves, furrows and/or pits

Enamel has normal to slightly reduced contrast/ thin

Autosomal dominant, recessive, or X-linked

Hypomaturation(Type II)

Varies from creamy opaque to marked yellow/brown, surface of teeth soft and rough, dental sensitivity and open bite common

Normal thickness with enamel that often chips and abrades easily

Enamel has contrast similar to or > than dentin, unerupted crowns have normal morphology

Autosomal dominant, recessive, or X-linked

Hypocalcified(Type III)

Opaque white to yellow-brown, soft rough enamel surface, dental sensitivity and open bite common, heavy calculus formation common

Normal thickness with enamel that often chips and abrades easily

Enamel has contrast similar to or < dentin, unerupted crowns have normal morphology

Autosomal dominant, recessive

Hypomaturation/ Hypoplasia/

Taurodontism(Type IV)

White/Yellow- Brown mottled, teeth can appear small and

lack proximal contact

Reduced, hypomineralized

areas and pits

Enamel contrast normal to slightly > dentin, large

pulp chambers

Autosomal dominant