DIKIOHS, date: 26-october-2009
Dr. Arsala Urooj
MDS Oral Pathology Trainee
DIKIOHS, date: 26-october-2009
 Dentinogenesis Imperfecta is a hereditory
developmental disturbance of dentin in the absence of
any systemic syndrome.
 Similar dental changes may be seen in conjunction
with systemic hereditary disorder of bone ,
Osteogenesis Imperfecta ( Osteogenesis Imperfecta
with Opalescent Teeth), which is clearly a separate
disease from dentiogenesis Imperfecta.
DIKIOHS, date: 26-october-2009
 DI
 Mutation of the
 Dentin sialophosphoprotien gene
 DSPP
 Currently 8 mutations of DSPP are known 7 are ass.
With DI, 8th is ass. With Dentin Dysplasia Type II
DIKIOHS, date: 26-october-2009
Sheilds
Clinical Presentation
Witkop
Dentinogenesis Imperfecta I
Osteogenesis Imperfecta with
Opalescent Teeth
Dentinogenesis Imperfecta
Dentinogenesis Imperfecta II
Isolated opalescent Teeth
Hereditary Opalescent teeth
Dentinogenesis Imperfecta III
Isolated opalescent Teeth
Brandywine Isolate
DIKIOHS, date: 26-october-2009
 1: 8000 ( whites in United states)
 DI & OI with opalescent Teeth are similar clinically,
radiographycally & histopathologically
 Both Primary & Secondary Dentition are affected
 Severity of dental alteration varies with AGE at which
tooth develops
 Decidous teeth > permanent Incisors > 1st molar> 2nd &
3rd molar
DIKIOHS, date: 26-october-2009
 Dentition has blue to brown discoloration
 Distinct translucency
 Enamel easily separates from underlying dentine
 Exposed dentine  accelerated attrition
 Enamel hypoplasia (some pt.), 2ndry defect not a
direct expression of DI gene
DIKIOHS, date: 26-october-2009
DIKIOHS, date: 26-october-2009
 Teeth has bulbous crowns
 Cervical constrictions
 Thin roots
 Early obliteration of root canal & pulp chamber
 Normal sized pulp / pulp enlargement shell teeth
DIKIOHS, date: 26-october-2009
 Rare abnormality
 Deciduous teeth (mostly)
 Normal thickness enamel
 Extremely thin dentin (may involve entire tooth or
isolated to root)
 Dramatically enlarged pulp
 Alteration may be un associated With DI as an isolated
finding in both dentition
 Normal tooth shape and coloration
DIKIOHS, date: 26-october-2009
Radiographic appearance of shell teeth
DIKIOHS, date: 26-october-2009
 Sensorineural ,high frequency hear loss
 Jaw position affect the anatomy of inner ear
 Premature tooth loss associated with hearing deficit
 Unclear that if hearing is associated with DSPP
mutation or alteration in primary gnathic changes
DIKIOHS, date: 26-october-2009
 Altered dentin
 Appear normal dentine  adjacent to enamel junction
 Remaining is distinctly abnormal
 Short misshapen tubules course through atypical
granular dentin matrix which often demonstrate
interglobular calcification
 Scanty atypical odontoblast line the pulp surface &
cells can be seen entrapped with in the defective
dentin
 Enamel is normal
 1/3 pt. hypoplastic/ hypocalcified defects of enamel
DIKIOHS, date: 26-october-2009
Obliterated pulp chamber
Water content more than 50%
Enlarged dentinal tubules
DIKIOHS, date: 26-october-2009
 Root canal  may develop micro exposure 





periapical inflammation
Crowns  cervical fracture
Overlay denture with Fl releasing GIC
Attrition  non precious metal casting
Occlusal wear composite with dentine bonding
agent
Implant
DIKIOHS, date: 26-october-2009
DIKIOHS, date: 26-october-2009