DEPOSITION OF ORGANIC MATRIXteDExt CALCIFICATION MATURATION

Hereditary developmental disorder (x-linked, autosomal

dominant or recessive, or sporadic inheritance.)

Interferes with enamel formation

Absence of any systemic involvement

Affects both the primary and permanent dentitions.

3 types: Hypoplastic AI (60– 73%) , hypomaturation AI (20–40%),

and hypocalcification ( 7%)

Each type has subtypes differentiated by mode of inheritance and

alteration of genes involved in the process of formation and
maturation of 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
• Accumulation of 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.