Amelogenesis imperfecta
INDIAN DENTAL ACADEMY
Leader in continuing Dental Education
www.indiandentalacademy.com
OBJECTIVES
 At the end of the lecture student should describe--
– Various developmental disorders affecting the structure of teeth
– Classification of Amelogenesis Imperfecta & enamel hypoplasia
– Etiopathogenesis & Clinical manifestations
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Developmental disturbances
in structure of teeth
Amelogenesis imperfecta
Environmental enamel hypoplasia
Dentinogenesis imperfecta
Dentin dysplasia
www.indiandentalacademy.com
Amelogenesis Imperfecta:-
It encompasses a complicated group of conditions that
demonstrate developmental alterations in the structure of the
enamel in the absence of a systemic disorder.
www.indiandentalacademy.com
Environmental alterations of the
structure of enamel
During amelogenesis an external stimuli can result in
abnormalities in the enamel
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Amelogenesis Imperfecta:-
Development of enamel takes place in three stages:-
 Formative stage- deposition of organic matrix.
 Calcification stage- matrix mineralization.
 Maturation stage- crystal enlargement and
maturation.
Three types of amelogenesis imperfecta:-
I. Hypoplastic
II. Hypocalcified
III. Hypomaturation
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Classification by Witkop(1989)
I. Hypoplastic:-
Pitted autosomal dominant
Local autosomal dominant
Local autosomal recessive
Smooth, autosomal dominant
Smooth, X-linked dominant
Rough autosomal dominant
Enamel agenesis, autosomal recessive
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Hypoplastic Amelogenesis
Imperfecta generalised pitted
pattern
Hypoplastic Amelogenesis
Imperfecta smooth pattern
Hypoplastic Amelogenesis
Imperfecta rough pattern
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Amelogenesis Imperfecta
II. Hypomaturation:-
Pigmented autosomal recessive
X-linked recessive
Snow-capped teeth, autosomal dominant
III. Hypocalcified:-
Autosomal dominant
Autosomal recessive
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Hypomaturation Amelogenesis
Imperfecta
Hypomaturation
Amelogenesis Imperfecta
snow-capped pattern
Hypocalcified Amelogenesis Imperfectawww.indiandentalacademy.com
IV. Hypomaturation- hypoplastic with taurodontism
Hypomaturation- hypoplastic with taurodontism, autosomal
dominant
Hypoplastic- hypomaturation with taurodontism, autosomal
dominant
Hypomaturation-
hypoplastic
with taurodontism
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Amelogenesis Imperfecta
Clinical Features:-
Hypoplastic:- enamel not formed to full thickness.
Hypocalcified:- soft enamel.
Hypomaturation:- enamel can be pierced by explorer and
chipped away.
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Clinical Features:-
Variable clinical appearance
Range from normal appearing to
unsightly teeth.
Yellow to dark brown appearance.
Smooth or wrinkled
No contact points between teeth
with incisal & proximal wear.
www.indiandentalacademy.com
Radiographic features
Absence or thinning of enamel
Radiographic opacity may become similar to dentin
www.indiandentalacademy.com
Histopathology:-
Depends on type.
Defects in matrix formation to absence of matrix formation.
Defective mineral deposition.
Alteration in enamel rod and rod sheath structures.
www.indiandentalacademy.com
Enamel Hypoplasia
Incomplete or defective formation of the organic enamel
matrix
Two types-
Hereditary type ( Amelogenesis Imperfecta)
 Affects both dentition
 Only enamel is affected
 Environmental
 Either dentition or only single tooth
 Both enamel and dentin
www.indiandentalacademy.com
Environmental Enamel Hypoplasia
Different factors are :
 Nutritional deficiency
 Exanthematous diseases
 Congenital syphilis
 Hypocalcemia
 Birth injuries
 Local infection/ trauma
 Ingestion of chemicals
 Idiopathic
www.indiandentalacademy.com
Nutritional deficiency &
Exanthematous fevers
Rickets during time of tooth
development
Deficiency of vit. A & vit. C
Exanthematous disease like measles,
chickenpox, scarlet fever
www.indiandentalacademy.com
Clinically manifest as
 Horizontal rows of pits
 Diminished enamel
 Location of the defects correlates well with the
development stage of the affected teeth.
www.indiandentalacademy.com
Most commonly involved teeth are –central & lateral incisors,
canines & first molars
www.indiandentalacademy.com
 Maxillary and mandibular permanent incisor and 1st
molars.
 Hutchinson’s incisor
 Mulberry, Moons, Fournier’s molars
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Hutchinson’s incisor
Permanent central incisor –
Screw driver shaped due to
convergence of mesial and
distal sides and notched incisal
edge due to absence of central
calcification centre.
www.indiandentalacademy.com
Mulberry molars
Molars- enamel of
occlusal surface and
occlusal 1/3rd shows mass
of globules instead of
cusps.
www.indiandentalacademy.com
Enamel hypoplasia due to
hypocalcemia
Tetany due to decreased level of
calcium in blood.
Due to vitamin D deficiency and
parathyroid deficiency.
Clinically manifest as pitting variety
www.indiandentalacademy.com
SUMMARY
– Various developmental disorders affecting the structure of
teeth
– Classification of Amelogenesis Imperfecta & enamel
hypoplasia

– Etiopathogenesis & Clinical manifestations
www.indiandentalacademy.com
Bibliography
Shafer’s text book of oral pathology 5th
& 6th
edition
Oral and maxillofacial pathology Neville, 2nd
edition
Color atlas of oral diseases Cawson, R. A.
8th
edition
www.indiandentalacademy.com
www.indiandentalacademy.com

Developmental disturbances in structure of teeth Amelogenesis imperfecta /endodontic courses

  • 1.
    Amelogenesis imperfecta INDIAN DENTALACADEMY Leader in continuing Dental Education www.indiandentalacademy.com
  • 2.
    OBJECTIVES  At theend of the lecture student should describe-- – Various developmental disorders affecting the structure of teeth – Classification of Amelogenesis Imperfecta & enamel hypoplasia – Etiopathogenesis & Clinical manifestations www.indiandentalacademy.com
  • 3.
    Developmental disturbances in structureof teeth Amelogenesis imperfecta Environmental enamel hypoplasia Dentinogenesis imperfecta Dentin dysplasia www.indiandentalacademy.com
  • 4.
    Amelogenesis Imperfecta:- It encompassesa complicated group of conditions that demonstrate developmental alterations in the structure of the enamel in the absence of a systemic disorder. www.indiandentalacademy.com
  • 5.
    Environmental alterations ofthe structure of enamel During amelogenesis an external stimuli can result in abnormalities in the enamel www.indiandentalacademy.com
  • 6.
    Amelogenesis Imperfecta:- Development ofenamel takes place in three stages:-  Formative stage- deposition of organic matrix.  Calcification stage- matrix mineralization.  Maturation stage- crystal enlargement and maturation. Three types of amelogenesis imperfecta:- I. Hypoplastic II. Hypocalcified III. Hypomaturation www.indiandentalacademy.com
  • 7.
    Classification by Witkop(1989) I.Hypoplastic:- Pitted autosomal dominant Local autosomal dominant Local autosomal recessive Smooth, autosomal dominant Smooth, X-linked dominant Rough autosomal dominant Enamel agenesis, autosomal recessive www.indiandentalacademy.com
  • 8.
    Hypoplastic Amelogenesis Imperfecta generalisedpitted pattern Hypoplastic Amelogenesis Imperfecta smooth pattern Hypoplastic Amelogenesis Imperfecta rough pattern www.indiandentalacademy.com
  • 9.
    Amelogenesis Imperfecta II. Hypomaturation:- Pigmentedautosomal recessive X-linked recessive Snow-capped teeth, autosomal dominant III. Hypocalcified:- Autosomal dominant Autosomal recessive www.indiandentalacademy.com
  • 10.
    Hypomaturation Amelogenesis Imperfecta Hypomaturation Amelogenesis Imperfecta snow-cappedpattern Hypocalcified Amelogenesis Imperfectawww.indiandentalacademy.com
  • 11.
    IV. Hypomaturation- hypoplasticwith taurodontism Hypomaturation- hypoplastic with taurodontism, autosomal dominant Hypoplastic- hypomaturation with taurodontism, autosomal dominant Hypomaturation- hypoplastic with taurodontism www.indiandentalacademy.com
  • 12.
    Amelogenesis Imperfecta Clinical Features:- Hypoplastic:-enamel not formed to full thickness. Hypocalcified:- soft enamel. Hypomaturation:- enamel can be pierced by explorer and chipped away. www.indiandentalacademy.com
  • 13.
    Clinical Features:- Variable clinicalappearance Range from normal appearing to unsightly teeth. Yellow to dark brown appearance. Smooth or wrinkled No contact points between teeth with incisal & proximal wear. www.indiandentalacademy.com
  • 14.
    Radiographic features Absence orthinning of enamel Radiographic opacity may become similar to dentin www.indiandentalacademy.com
  • 15.
    Histopathology:- Depends on type. Defectsin matrix formation to absence of matrix formation. Defective mineral deposition. Alteration in enamel rod and rod sheath structures. www.indiandentalacademy.com
  • 16.
    Enamel Hypoplasia Incomplete ordefective formation of the organic enamel matrix Two types- Hereditary type ( Amelogenesis Imperfecta)  Affects both dentition  Only enamel is affected  Environmental  Either dentition or only single tooth  Both enamel and dentin www.indiandentalacademy.com
  • 17.
    Environmental Enamel Hypoplasia Differentfactors are :  Nutritional deficiency  Exanthematous diseases  Congenital syphilis  Hypocalcemia  Birth injuries  Local infection/ trauma  Ingestion of chemicals  Idiopathic www.indiandentalacademy.com
  • 18.
    Nutritional deficiency & Exanthematousfevers Rickets during time of tooth development Deficiency of vit. A & vit. C Exanthematous disease like measles, chickenpox, scarlet fever www.indiandentalacademy.com
  • 19.
    Clinically manifest as Horizontal rows of pits  Diminished enamel  Location of the defects correlates well with the development stage of the affected teeth. www.indiandentalacademy.com
  • 20.
    Most commonly involvedteeth are –central & lateral incisors, canines & first molars www.indiandentalacademy.com
  • 21.
     Maxillary andmandibular permanent incisor and 1st molars.  Hutchinson’s incisor  Mulberry, Moons, Fournier’s molars www.indiandentalacademy.com
  • 22.
    Hutchinson’s incisor Permanent centralincisor – Screw driver shaped due to convergence of mesial and distal sides and notched incisal edge due to absence of central calcification centre. www.indiandentalacademy.com
  • 23.
    Mulberry molars Molars- enamelof occlusal surface and occlusal 1/3rd shows mass of globules instead of cusps. www.indiandentalacademy.com
  • 24.
    Enamel hypoplasia dueto hypocalcemia Tetany due to decreased level of calcium in blood. Due to vitamin D deficiency and parathyroid deficiency. Clinically manifest as pitting variety www.indiandentalacademy.com
  • 25.
    SUMMARY – Various developmentaldisorders affecting the structure of teeth – Classification of Amelogenesis Imperfecta & enamel hypoplasia  – Etiopathogenesis & Clinical manifestations www.indiandentalacademy.com
  • 26.
    Bibliography Shafer’s text bookof oral pathology 5th & 6th edition Oral and maxillofacial pathology Neville, 2nd edition Color atlas of oral diseases Cawson, R. A. 8th edition www.indiandentalacademy.com
  • 27.

Editor's Notes

  • #19 The crowns of the deciduous teeth begin to develop at approx.14 week of gestation & continue until child is 12 months old. Crowns of permanent