1. AMELOGENESIS
IMPERFECTA
Presented by:
HARON, Sittie Aainaa M.

2. AMELOGENESIS IMPERFECTA
(AI)
ALSO CALLED: ENAMEL DYSPLASIA
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.

3. SIGNS AND SYMPTOMS
This condition causes teeth to be unusually small, discolored,
pitted or grooved, and prone to rapid wear and breakage
• 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

4. DENTAL CORRELATION
As a dentist, we are expected to diagnose and treat this
condition: • 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.

6. 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

7. Type Clinical Appearance Enamel Thickness Radiographic Inheritance
Appearance
Hypoplastic Crowns size varies from small Varies from thin and Enamel has normal to Autosomal
(Type I) to normal, small teeth may smooth to normal slightly reduced contrast/ dominant, recessive,
lack proxmial contacts, color thickness with thin or X-linked
varies from normal to opaque grooves, furrows
white – yellow brown and/or pits
Hypomaturation Varies from creamy opaque to Normal thickness Enamel has contrast Autosomal
(Type II) marked yellow/brown, surface with enamel that similar to or > than dominant, recessive,
of teeth soft and rough, dental often chips and dentin, unerupted crowns or X-linked
sensitivity and open bite abrades easily have normal morphology
common
Hypocalcified Opaque white to yellow- Normal thickness Enamel has contrast Autosomal
(Type III) brown, soft rough enamel with enamel that similar to or < dentin, dominant, recessive
surface, dental sensitivity and often chips and unerupted crowns have
open bite common, heavy abrades easily normal morphology
calculus formation common
Hypomaturation/ White/Yellow- Brown mottled, Reduced, Enamel contrast normal Autosomal
Hypoplasia/ teeth can appear small and hypomineralized to slightly > dentin, large dominant
Taurodontism lack proximal contact areas and pits pulp chambers
(Type IV)