4. Introduction!
A diverse group of
hereditary conditions that
primarily affect the quality
and/or the quantity of
Dental ENAMEL, resulting in
poor development or
complete absence of the
enamel of the teeth.
It is an Ectodermal disease
& the mesodermal
component is normal
5. CLASSIFICATION:
Based on the stage at which the disease occurs:
HYPOPLASTIC TYPE:
Normal or reduced enamel thickness throughout
the whole surface, or in isolated areas.
HYPOCALCIFICATION TYPE:
Inadequate mineralization, resulting in enamel
that gets lost a few years after eruption.
Regular enamel thickness at the time of tooth
eruption.
HYPOMATURATION TYPE:
Adequate deposition of enamel, but the enamel
crystal does not mature normally.
The result is soft pigmented enamel that chips
easily. Opaque mottled enamel.
8. HYPOCALCIFICATION TYPE:
AUTOSOMAL DOMINANT
AUTOSOMAL RECESSIVE
Enamel Thickness: Normal.
Hypoplasia can be seen in the
middle third of the tooth.
Enamel Consistency: Very Soft. Lost
soon after eruption leaving only the
dentin exposed.
Color: Newly erupted are covered
with dull lusterless opaque, white,
honey colored or yellowish to brown
shade
Dentinal hypersensitivity present.
9. HYPOMATURATION TYPE:
AUTOSOMAL DOMINANT
AUTOSOMAL RECESSIVE
SNOW-CAPPED TEETH
Enamel Thickness: Normal. Point of
explorer can be forced into enamel
Color: Permanent are mottled
yellow white in color, but may be
darkened with stain absorption.
Primary have ground glass
opaque white appearance.
10. Maxillary Teeth are more commonly
affected.
Varying amount of enamel on incisal or
occlusal aspect of crown is present and has
opaque white appearance.
Opacity may be solid or flecked and may
involve enamel surface.
Junctional line of opaque white and
translucent enamel is sharp
“Dipped into white paint appearance”
Snow-Capped Teeth
11. HYPOMATURATION – HYPOPLASTIC
WITH TAURODONTISM TYPE:
AUTOSOMAL DOMINANT
Resembles the same as in Tricho-dento-
osseous syndrome, which comprises of
kinky hair, brittle nails and
osteosclerosis, in addition to dental
abnormalities.
*Several cases of AI are associated
with Nephrocalcinosis (Dystrophic
Calcification in Kidney)
12. Radiographic Features:
Square Crown with relatively thin opaque layer of enamel with low or abscent cusp.
Pitted enamel appears as sharply localized area of mottled dentisty.
Hypomaturation demonstrates normal thickness but density is that of dentin
Hypocalcified has normal thickness but density is even less than dentin.
With advanced abrasion, obliteration of pulp chamber can also be seen.
Amelogenesis Imperfecta
Normal IOPA
14. DIFFERENTIAL DIAGNOSIS:
Dentinogenesis Imperfecta: Normal shape, size of crown &
root with relatively normal density, obliteration of pulp
chamber and root canals in absence of marked abrasion.
DIAGNOSIS:
• CLINICAL: Cheesy consistency of enamel with enamel loss
• RADIOGRAPHIC: Missing enamel cap with low or abscent cusp
MANAGEMENT:
Cosmetic Improvement
Desensitizing Agents
Overdenture
16. Introduction!
An autosomal dominant condition, DI
results in gray to yellowish-brown teeth
having broad crowns with constricted
cervical areas resulting in a “Tulip Shape”
The gene maps to chromosome number 4
which encodes for DSPP. This protein
constitutes 50% of non-collagenous
component of dentin matrix.
17. Shield’s Classification
TYPE I
(Dentinogenesis
Imperfecta)
• DI associated
with
Osteogenesis
Imperfecta.
• Roots & Pulp
Chambers are
small and
underdeveloped
TYPE II
(Hereditary
Opalescent Teeth)
• DI not associated
with
Osteogenesis
Imperfecta
• Progressive
hearing loss +
dental
abnormalities
TYPE III
(Brandywine
Isolate)
• Bell-Shaped
crowns especially
in permanent
dentition.
• Shell like teeth &
multiple pulp
exposure
19. Radiographic Features:
Crown size is normal giving a bulbous
appearance due to cervical constriction.
Marked occlusal attrition.
Short & Slender roots.
Type I & II have partial or complete
obliteration of pulp chamber.
Root canals may be absent or thread-like.
Periapical radiolucency may be seen
occasionally.
Shell-Like Tooth (Type III)
21. DIAGNOSIS:
Clinical: Rapid loss of enamel with attrition. Multiple pulp exposure seen.
Radiographic: Pulp Chamber obliteration. Presence of periapical
radioluscencies without pulp involvement
DIFFERENTIAL DIAGNOSIS:
Dentinal Dysplasia: Crown size and shape is in proportion.
Amelogenesis Imperfecta
MANAGEMENT:
Overdentures or Crown Placement
23. Rare disturbance of dentin formation
characterized by normal enamel but
atypical dentin formation with
abnormal pulp morphology.
ETIOLOGY:
Hereditary Disease, transmitted as
autosomal dominant characteristic.
Mutation rate is extremely low.
Introduction!
24. Classification:
Shield
Clinical
Classification
Witkop
Radiologic
Classification
Type I – Dentin Dysplasia
Normal shape, size & consistency with sligh amber & translucent
shade
Mobility of teeth present causing malalignment & malpositioning
Minor trauma may result in exfoliation
Type II – Anomalous Dysplasia
Primary are yellow, brown, blue, grey-amber, translucent
Permanent are normal coloured.
Root size is normal
• Type I - Radicular Dentin Dysplasia
• Type II - Coronal Dentin Dysplasia
25. Radiographic Features:
TYPE I (Radicular):
The roots of primary and permanent teeth are either short or abnormally shaped.
Roots of primary teeth appear to be thin spicules. Pulp chambers and root canals are
completely filled before eruption and appear half moon-shaped. Periapical
radiolucency present which may be cyst or granulomas
TYPE II (Coronal):
Obliteration of pulp chamber and reduction in calibration of root canals occur after
tooth eruption (5-6 yrs.). Pulp chambers of molars are filled with hypertrophic
dentin, they become flame shaped & have multiple pulp stones. Anterior and
premolars develop ‘thistle tube’ shaped pulp chambers because of its extension into
the root.
29. Introduction!
An unusual developmental anomaly in
which ectodermal and mesodermal tooth
component are affected. It is a localized
arrest in tooth development. All the
elements of tooth are hypocalcified and
hypomineralized.
ETIOLOGY:
Uncertain. Trauma, infection, local
Ischemia, local vascular defect,
Genetic transmission, local
Somatic mutation, etc.
30. Clinical Features:
Gingival swelling due to calcification in adjacent soft tissue
Exhibit a delay or total failure in eruption
Periapical abscess is very frequent
The tooth is friable & susciptable to caries &
fracture
Very irregular in appearance. Affected teeth can
be mottled brown
Small, Asymmetrical morphology.
ASSOCIATED DISEASES:
1. Hemangioma
2. Hydrocephalus
3. Vascular Nevi
4. Epidermal Nevus Syndrome
31. Radiographic Features:
Marked reduction in the radiodensity giving the teeth a pale whispy “Ghost”
appearance.
Enamel is very thin, less dense and poorly mineralized
that it may not even appear on the radiograph
The tooth appear like thin shells &
unerupted ones appear to be resorbing
The pulp chambers are large and root
canals wide due to hypoplastic dentin.
32.
33. DIAGNOSIS:
Clinical: Irregular shaped teeth with brown discoloration
Radiographic: Shell Tooth appearance
DIFFERENTIAL DIAGNOSIS:
Shell Tooth of Dentinogenesis Imperfecta: Familial
Involvement + Non-Hypoplastic Enamel. Only few
affected teeth.
MANAGEMENT:
Prosthetic Replacement
Restorative Procedure
35. Normal dentin is calcified by
deposition of calcium salts in the organic
matrix in the form of globules, which
increase in size and finally unite into a
homogenous structure.
In Dentin Hypocalcification there is
failure of union between these globules
leaving interglobular areas of uncalcified
matrix
CAUSES: Parathyroid Deficiency or Rickets
36. CREDITS: This presentation template was created by
Slidesgo, and includes icons by Flaticon, and infographics
& images by Freepik
RESOURCES:
Sivapathasundharam B Shafer WG. Shafer's Textbook of
Oral Pathology. Ninth ed. New Delhi: Elsevier; 2020.
Ghom A Ghom SA Verma M. Textbook of Oral Medicine
with Free Book on Basic Oral Radiology. Third ed. New
Delhi: Jaypee Brothers Medical Publishers (P); 2014.
Karjodkar FR. Essentials of Oral and Maxillofacial
Radiology. Second ed. New Delhi: Jaypee Brothers
Medical; 2019.
Thank
You!
GUIDED BY:
Dr. Mukta Vanjani
