DENTINOGENESIS IMPERFECTA

A hereditary developmental disturbance of the dentin

Similar dental changes may be seen in conjunction with the systemic
hereditary disorder of bone osteogenesis imperfecta
Morphologically:
oThe dentitions have a blue-to-brown discoloration often with a
distinctive translucence
o The enamel frequently separates easily fro m the underlying defective
dentin. Once exposed, the dentin often demonstrates significantly
accelerate d attrition
Radiographically:
o the teeth have bulbous crowns
oCervicalconstriction
othin roo ts
oobli teration of the root canals and pulp chambe rs
oSome teeth may show normal sized pulps or pulpal enlargement
Histologically:
oShort misshapen tubules course through an atypical granular dentin matrix which often
demonstrates interglobular calcification
Treatment
Overlay dentures placed on teeth that are covered
with fluoride-releasing glass-ionomer cement
Additional therapeutic approaches
VITAMIN D-RESISTANT RICKETS

Inherited as an X- linked(male) dominant trait
The disorder is caused by mutations in a zinc
metalloproteina se gene known as PHEX (phosphate
regulating gene)
Radiographically :
large pulp chambers with pulp horns extending almost to the
dentinoenamel junction
In some cases ,the cuspal enamel may be worn down by
attrition to the level of the pulp horn causing pulpal
exposure and pulp death
Morphologically:
The tooth appear dead
Histologically:
The dentin appears abnormal and is characterized by the
deposition of globular dentin , which often exhibits clefting.
Treatment
early treatment with calcitriol and multiple daily
doses of phosphate.
Endodontic therapy is necessary for the pulpally
involved teeth.

Dentin Dysplasia
Thereare two major pattern s: type I and type II
Caused by genetic disorder
Dentine dysplasia type I
Normal shape, form and consistency.
radiographically :little or no detectable pulp and
roots that are markedly short or absent.
Dentin Dysplasia Type II
exhibits numerous features of dentinogenesis
imperfecta
Radiographically:bulbous crowns, cervical
constriction, thin roots, and obliteration of the
pulp.
Treatment:
Excessive care of oral hygiene must be established
and maintained
If periapical inflammato ry lesions develop the
therapeutic choice is use and is guided by the root
length.

Regional odontodysplasia
localized nonhereditary developmental abnormality of teeth with
extensive adverse effects on the formation of enamel, dentin ,and pulp.
Most cases are idiopathic, but a number have been related to various
syndromes,Growth abnormalities, Neural disorders. and vascular
malformations
Morphology:Many of the affected teeth fail to erupt. Erupted teeth
demonstrate small irregular crowns that are yellow to brown
Radiographically:the altered teeth demonstrate extremely thin enamel
and dentin surrounding an enlarged radiolucent pulp(ghost teeth)
Histologically:
structure of the enamel is irregular or lacking with a laminated appearance
dentin contains clefts scattered through a mixture of interglobular dentin and
amorphous material
pulp tissue contains free or attached stones that may exhibit tubules or consist
of laminated calcification
Treatment:
Endodontic therapy on non vital teeth that have
sufficient hard tissue
Erupted teeth can be covered with etched-
retained restorations or stainless steel crowns

SEGMENTAL ODONTOMAXILLARY
DYSPLASIA

developmental disorder that affects the jaw and
(sometimes) the overlying facial tissues
Etiology:unknown
Morphology:
unilateral enlargement of the maxillary bone, along with fibrous
hyperplasia of the overlying gingival soft tissues .
maxillary premolars frequently are missing and the primary
teeth in the affected area may be hypoplastic or show enamel
defects
Histologically:
The affected maxillary bone consists of irregular trabeculae with
a woven appearance. This bone shows numerous resting and
reversal lines but it lacks significant osteoblastic and osteoclastic
activity.

Treatment:
not much is known about its natural evolution.
Once diagnosed, the condition seems to remain
stable and may not require surgical intervention.
However, orthodontic therapy and orthognathic
surgery may be considered in some cases.

DENS EVAGINATUS

an abnormal proliferation and folding of a portion the IEE
and subjacent ectomesenchymal cells of the dental papilla
into the stellate reticulum during the bell stage of tooth
formation
Morphology:
cusp-like elevation of enamel located in the central groove or
lingual ridge of the buccal cusp of permanent premolar or
molar teeth
Radiographically
the occlusal surface exhibits a tuberculated appearance. and
often a pulpal extension is seen in the cusp
Etiology:
Unknown,can result from many factors, including genetic and
environmental ones .


Treatment:
Type I: Normal pulp, mature apex
Type II: Normal pulp, immature apex
Type III: Inflamed pulp, mature apex
Type IV: Inflamed pulp, immature apex
Type V: Necrotic pulp, mature apex
Type VI: Necrotic pulp, immature apex