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DEPOSITION OF ORGANIC MATRIXteDExt CALCIFICATION MATURATION
Hereditary developmental disorder(x-linked, autosomal
dominantor recessive, or sporadic inheritance.)
Interferes with enamel formation
Absence of any systemic involvement
Affects both the primary and permanent dentitions.
3 types: HypoplasticAI (60– 73%) , hypomaturation AI (20–40%),
and hypocalcification( 7%)
Each type has subtypes differentiatedby mode of inheritance and
alteration of genes involvedin the process of formation and
maturationof the enamel
Genes involved
• Amelogenin
• Ameloblastin
• ENAM gene Enamelin
• Enamelysin
• Kalikerin
• Tuftelin
Defective gene for X-linked AI type closely linked to the locus DXS85 at Xp22. Which is also
location for amelogenin
Hypoplastic AI
• Enamel that is pitted, has grooves or
furrows, has large areas of missing, or
enamel that is very thin over the entire
tooth crown.
• Clinically, the crown size varies from small
to normal and small teeth may
lack proximalcontacts.
• Enamel may be rough, smooth, pitted,
grooved, locally hypoplastic or the
complete tooth crown may appear with
thin enamel.
Hypocalcified AI:
• Dental sensitivity
• Poor aesthetics.
• Early loss of enamel.
• The thickness of enamel appears to
be normal at eruption that often chips and
but, tends to abrade easily post eruptively.
• Delayed eruption of teeth.
• Anterior open bite
• Accumulationof a large amount of
supragingival calculus
Hypomaturation AI
•Normal morphologically at the
time of eruption, but eventually
chip away, especially in
the occlusal areas.
•Sensitivity
•Open bite malocclusion
•The enamel thickness is normal
but often chips off and abrades
away easily.
Hypomaturation-Hypoplastic
with Taurodontism:
• Clinically, white/yellow- brown mottled.
• The teeth appear smaller than
normal and lack proximal contacts.
• The crowns show pitting and tend to
have hypo mineralized areas.
• Radiographically, The enamel contrast is
normal to slightly greater than dentin.
• shows large or bulbous pulp chambers
which appear taurodontic
TREATMENT
•Three stages
•Temporary phase -during the primary and mixed dentition
Stainless steel crowns on primary molars
Polycarbonate crowns, resin modified glass ionomers (RMGI), prefabricated crowns (stainless steel
crowns with or without esthetic facing) or direct composite resin can be used.
• Transitional phase – when all permanent teeth have erupted
Stainless steel crowns for permanent molars
Composite resin restorations on occlusal surface
•Permanent phase – in adulthood.
Crown lengthening and gingival recontouring,Orthodontic treatments may be used to close interdental
spaces and for anterior open bite ,Root canal therapy,Orthognathic surgery.
amelogenesis imperfecta
amelogenesis imperfecta

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amelogenesis imperfecta

  • 1.
  • 2.
  • 3. DEPOSITION OF ORGANIC MATRIXteDExt CALCIFICATION MATURATION
  • 4. Hereditary developmental disorder(x-linked, autosomal dominantor recessive, or sporadic inheritance.) Interferes with enamel formation Absence of any systemic involvement Affects both the primary and permanent dentitions. 3 types: HypoplasticAI (60– 73%) , hypomaturation AI (20–40%), and hypocalcification( 7%) Each type has subtypes differentiatedby mode of inheritance and alteration of genes involvedin the process of formation and maturationof the enamel
  • 5. Genes involved • Amelogenin • Ameloblastin • ENAM gene Enamelin • Enamelysin • Kalikerin • Tuftelin Defective gene for X-linked AI type closely linked to the locus DXS85 at Xp22. Which is also location for amelogenin
  • 6.
  • 7.
  • 8. Hypoplastic AI • Enamel that is pitted, has grooves or furrows, has large areas of missing, or enamel that is very thin over the entire tooth crown. • Clinically, the crown size varies from small to normal and small teeth may lack proximalcontacts. • Enamel may be rough, smooth, pitted, grooved, locally hypoplastic or the complete tooth crown may appear with thin enamel.
  • 9. Hypocalcified AI: • Dental sensitivity • Poor aesthetics. • Early loss of enamel. • The thickness of enamel appears to be normal at eruption that often chips and but, tends to abrade easily post eruptively. • Delayed eruption of teeth. • Anterior open bite • Accumulationof a large amount of supragingival calculus
  • 10. Hypomaturation AI •Normal morphologically at the time of eruption, but eventually chip away, especially in the occlusal areas. •Sensitivity •Open bite malocclusion •The enamel thickness is normal but often chips off and abrades away easily.
  • 11. Hypomaturation-Hypoplastic with Taurodontism: • Clinically, white/yellow- brown mottled. • The teeth appear smaller than normal and lack proximal contacts. • The crowns show pitting and tend to have hypo mineralized areas. • Radiographically, The enamel contrast is normal to slightly greater than dentin. • shows large or bulbous pulp chambers which appear taurodontic
  • 12. TREATMENT •Three stages •Temporary phase -during the primary and mixed dentition Stainless steel crowns on primary molars Polycarbonate crowns, resin modified glass ionomers (RMGI), prefabricated crowns (stainless steel crowns with or without esthetic facing) or direct composite resin can be used. • Transitional phase – when all permanent teeth have erupted Stainless steel crowns for permanent molars Composite resin restorations on occlusal surface •Permanent phase – in adulthood. Crown lengthening and gingival recontouring,Orthodontic treatments may be used to close interdental spaces and for anterior open bite ,Root canal therapy,Orthognathic surgery.