dentinogenesis imperfecta

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Presented by: Anupama Mukundan

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Hi friends, this is my presentation on Dentinogenesis Im

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Page 1: Dentinogenesis imperfecta

Presented by:Anupama Mukundan

Page 2: Dentinogenesis imperfecta

What is dentinogenesis Introduction to dentinogenesis

imperfectaClassificationEtiologyClinical featuresRadiographic featuresHistopathologic featuresTreatment

Page 3: Dentinogenesis imperfecta

Dentinogenesis is the formation of dentin, which starts before amelogenesis.

Dentin is formed by odontoblast cells.

Dentinogenesis takes place in two phases:

1. formation of organic collagen matrix 2.deposition of hydroxyapetite crystals

Page 4: Dentinogenesis imperfecta

Is an inherited disorder of dentin formation

Autosomal dominant condition

Affects deciduous and permanent teeth

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SHIELDS CLASSIFICATION1.TYPE - I2.TYPE - II3.TYPE- III REVISED CLASSIFICATION

DENTINOGENESIS IMPERFECTA 11.DENTINOGENESIS IMPERFECTA 2

WITKOP CLASSIFICATION1.DENTINOGENESIS IMPERFECTA2.HEREDITARY OPALASCENT DENTIN3.BRANDYWINE ISOLATE

Page 6: Dentinogenesis imperfecta

TYPE I: Occurs in patients affected with osteogenesis imperfecta

TYPE II : Is not associated with osteogenesis imperfecta

TYPE III: “Brandywine type” rare condition, seen in racial isolate of Maryland, exhibits multiple pulp exposures and periapical lesions in deciduous dentition.

Page 7: Dentinogenesis imperfecta

DENTINOGENESIS IMPERFECTA 1 Without osteogenesis imperfecta Corresponds to type II of shields classification

DENTINOGENESIS IMPERFECTA 2 Corresponds to type III of shields classification

THERE IS NO SUBSTITUTE IN THE PRESENT CLASSIFICATION FOR THE CATEGORY DESIGNATED AS TYPE I IN THE SHIELDS CLASSIFICATION

Page 8: Dentinogenesis imperfecta

SYNONYMS OPALESCENT DENTIN

DENTINOGENESIS IMPERFECTA WITHOUT OSTEOGENESIS IMPERFECTA

OPALESCENT TEETH WITHOUT OSTEOGENESIS IMPERFECTA

SHIELDS TYPE II

CAPDEPONT TEETH

Page 9: Dentinogenesis imperfecta

MUTATION IN THE DENTIN SIALO PHOSPHO PROTEIN (DSPP) gene ENCODING DENTIN PHOSPHOPROTEIN AND DENTIN SIALOPROTEIN

Page 10: Dentinogenesis imperfecta

Clearly distinct from osteogenesis imperfecta with opalescent teeth & affects only the teeth

No increased frequency of bone fracture is seen

Frequency: 1 in 6000-8000

Page 11: Dentinogenesis imperfecta

Synonyms

SHIELDS TYPE IIIBRANDYWINE TYPE

DENTINOGENESIS IMPERFECTA

Page 12: Dentinogenesis imperfecta

Some researchers say it is a separate mutation from DGI 1

Shield et al 1973 stated that markedly enlarged pulp chambers and pulp exposures occurs in deciduous teeth do not occur in DGI 1

Witkop 1975 suggested both are same

Recent studies suggests both are result of mutation in two tightly linked genes

MacDougall et al 1999 stated DGI 2 differ from DGI 1 by the presence of multiple pulp exposures normal non mineralized pulp chambers ,and general appearance of shell teeth

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Affects males and females equally Teeth are blue gray or amber brown and

opalescent Few days after eruption teeth may achieve a

normal color, following which they become translucent

Page 14: Dentinogenesis imperfecta

Finally become gray or brown with bluish reflection from enamel

Enamel may split readily from dentin when subjected to occlusal stress

Severe attrition of teeth

Obliterated pulp chamber

Sauk et al (1976)increase in glycosaminoglycans in edta soluble dentin in teeth from patients with this disorder as compared to controls and less gag in edta insoluble residue

Page 15: Dentinogenesis imperfecta

Teeth are not sensitive

Dentin is soft and easily penetrable but not caries prone because of structural change in dentin

In some case there may be hypomineralised area on the enamel

Page 16: Dentinogenesis imperfecta

Bulb shaped or bell shaped crowns of teeth with constricted cervical areas

Roots - thin and spiked

Obliteration of coronal and radicular pulp chamber depending on age

Cementum, alveolar bone and PDL appears normal

Type 2large pulp chambers with thin shell of dentin and enamel “shell teeth”

Page 17: Dentinogenesis imperfecta
Page 18: Dentinogenesis imperfecta

Enamelnormal

Mantle dentin (narrow zone of dentin below enamel)normal

Remaining dentin severely dysplastic with vast areas of amorphous matrix with globular or interglobular foci of mineralization

Reduced number of dentinal tubules

Page 19: Dentinogenesis imperfecta

Tubules distorted, irregular in shape, widely spaced ,larger in size

Absence of odontoblastic processes and presence

of degenerating cellular debris instead

Large area of atubular dentin

Pulp chamber and root canal obliterated by abnormal dentin deposition

DEJ smooth or flattened instead of scalloped (responsible for early chipping of enamel)

Page 20: Dentinogenesis imperfecta

Increased water content (60 % than normal)

Decreased mineral content Density, x-ray absorption and

hardness are lowMicro hardness near to cementum

Page 21: Dentinogenesis imperfecta

AIMED AT PREVENTING LOSS OF ENAMEL AND DENTIN THROUGH ATTRITION

Mild –moderate cases (no enamel loss or rapid wear of teeth)

1. Routine restorative techniques Eg:amalgam, composite

2. Bonding of veneers for esthetics as they mask opalescence of anterior teeth

3. Bleaching to an extend lightens the color

Page 22: Dentinogenesis imperfecta

Severe cases: (significant enamel loss and rapid wear)

1.Full coverage crown restoration

2.Primary teeth stainless steel in posteriors stainless steel with open face composite for

anterior teeth 3.Permanent teeth porcelain fused metal

crowns

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Shafer’s text book of oral pathology (6th edition)

Oral and maxillofacial pathology-Neville (3rd edition)

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THANK YOU