Developmental disturbances affecting structure of teeth- Dentinogenesis Imperfecta

2.  Dentinogenesis Imperfecta
 Dentin dysplasia
 Regional Odontodysplasia

3.  Inherited disorder of dentin formation
 Autosomal dominant condition
 affects deciduous and permanent teeth
Genetic alteration:
DSPP- chromosome 4;
encoding dentin phosphoprotein & dentin
sialoprotein

4. SHIELDS CLASSIFICATION
1. TYPE –I
2. TYPE –II
3. TYPE-III
WITKOP CLASSIFICATION
1. DENTINOGENESIS IMPERFECTA
2. HEREDITARY OPALASCENT DENTIN
3. BRANDYWINE ISOLATE
REVISED CLASSIFICATION
1. DENTINOGENESIS IMPERFECTA 1
2. DENTINOGENESIS IMPERFECTA 2

5. SYNONYMS
 OPALESCENT DENTIN
 DI WITHOUT OSTEOGENESIS IMPERFECTA
 OPALASCENT TEETH WITHOUT OSTEOGENESIS
IMPERFECTA
 SHIELDS TYPE II
 CAPDEPONT TEETH

6.  Teeth are blue gray or amber brown and opalescent
 Affected teeth have bulbous crowns- Tulip shape
 Enamel may split readily from dentin when subjected
to occlusal stress.
 Severe attrition of teeth

7. Synonyms
 SHIELDS TYPE III
 BRANDYWINE TYPE DENTINOGENESISIMPERFECTA
C/F:
 This disorder was found in the Brandywine triracial
isolate in Maryland.
 Affects both dentitions
 characterized by too little dentin formation.

8.  Type I -bulb shaped or bell shaped crowns with constricted
cervical areas
 Roots  thin and spiked
 obliteration of coronal and radicular pulp chamber depending
on age
 Type II large pulp chambers with thin shell of dentin and
enamel “SHELL TEETH”

9. H/P:
 ENAMEL  normal
 DENTINAL TUBULES -reduced number
 TUBULES  distorted, irregular in shape,
widely spaced, larger in size.
 Areas of uncalcified matrix.
 Pulpal spaces either smaller than
normal or completely obliterated.
Ground section of tooth

10. “Rootless Teeth”
 hereditary disease
 rare disturbance of dentin formation
 Enamel- normal
 abnormal pulpal morphology
Classification:
 Type I (Radicular Type)
 Type II (Coronal Type)

11. Type I (Radicular Type)
 both dentitions are of normal colour
 premature tooth loss may occur because of short roots
or periapical inflammatory lesions.
Radiographically:
 roots are extremely short
 pulps almost /completely obliterated
periapical radiolucencies:
• granulomas
• cysts
• chronic abscesses

12. H/P:
 Pulp is obliterated by calcified tubular dentin,
osteodentin and fused denticles.
 Normal dentin formation is blocked so that new
dentin forms around obstacles.
 Producing the characteristic lava flowing around boulders
appearance and cascades of dentin.

13. Type II (Coronal Type)
color of primary dentition is opalescent.
permanent dentition is normal.
Radiographically:
Deciduous
roots are extremely short
pulps almost completely obliterated
Permanent
abnormally large pulp chambers in coronal
portion of tooth (thistle tube appearance)

14. H/P:
 Amorphous and atubular dentin in the radicular
portion.
 Permanent teeth also show multiple pulp stones/
denticles.

15.  Odontogenic Dysplasia/ Odontogenesis Imperfecta/
Ghost Teeth
 one or more teeth in a localized area are affected
 maxillary teeth are involved more frequently than
mandibular. Eg, incisors, cuspids
 etiology is unknown

16.  teeth affected may exhibit a delay or total failure in
eruption
 shape is altered, irregular in appearance

17. H/P:
 Enamel thickness varies.
 Marked reduction in the amount of dentin.
Widening of the pre-dentin layer. Large areas of
interglobular dentin and irregular tubular pattern.
 Pulp tissue contains free or attached pulp stones.
 dental follicular tissue may be enlarged & exhibits focal
enamel-like calcifications called enameloid
conglomerates.

18. Radiographically:
 reduction in radiodensity
 teeth assume a “ghost” appearance
 both enamel & dentin appear very thin
 pulp chamber is exceedingly large

19. Treatment:
 poor cosmetic appearance of teeth
 Extraction
And prosthetic rehabilitation