Disorders of Development of

the Oral Region & Teeth I & II

Reference for this topic:
Contemporary Oral and Maxillofacial Pathology, 2nd
edition, Mosby By Sapp, Eversole & Wysocki

Dr. Huda Hammad

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 Objectives
By the end of this lecture students should:
Be familiar with different types of developmental
disorders.
Be able to classify dental developmental disorders
according to type.
Be able to realize specific features of each
developmental disorder.
Be able to differentiate among similar
developmental dental disorders.

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 Disorders of Development of
the Oral Region & Teeth
Teeth
Disturbances in Size
Disturbances in Number
Disturbances in Eruption
Disturbances in Shape
Disturbances in Structure
Soft tissues
Bone

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 TEETH: Disturbances in size
Microdontia
Teeth which are smaller than normal
(usual range of variation).
1. True generalized
All the teeth are smaller than normal.
Teeth are well formed but small in size.
Seen in uncommon conditions like
pituitary dwarfism.

2. Relative generalized
Jaws somewhat larger than normal but
teeth are ofnormal size.
Teeth are spaced.

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 TEETH: Disturbances in size
3. Involving a single tooth
More common than generalized.
Crowns often conical in shape.
Most frequently affects:
1. Maxillary lateral incisors (peg laterals)
2. Maxillary third molars
3. Supernumerary teeth
teeth in addition to the regular number of teeth

5 Peg-shaped lateral incisor Microdontic 3rd molar

 TEETH: Disturbances in size
Macrodontia
Teeth that are larger than normal.
1. True generalized
All the teeth are larger than normal.
Seen in rare conditions e.g. pituitary gigantism.

2. Relative generalized
Jaws somewhat smaller than normal but teeth
are of normal size.
Teeth are crowded.

3. Localized/regional
Affected segment of jaw in:
Hemifacial hypertrophy
Segmental odontomaxillary dysplasia

4. Involving a single tooth

Rare, should not be confused with fusion of 2
teeth.

5. Rhizomegaly=radiculomegaly Which of the 2 molars is abnormal?

Root considerably longer than normal. - The one on the right of the
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Most commonly mandibular canines. patients mouth
 Teeth: Disturbances In Number
Anodontia
Congenital absence of all teeth; deciduous
and permanent.

This patient did not develop any

deciduous or permanent teeth.
Hypodontia (partial anodontia, oligodontia)
Congenital absence of one or more teeth.
More common than anodontia.
Familial tendency.
Most common congenitally absent permanent
teeth are:
Third molars
Maxillary laterals
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Mandubular 2nd premolars
Hypodontia
 Teeth: Disturbances In Number
Hypodontia (partial anodontia, oligodontia)
Uncommon in deciduous:
Mostly maxillary laterals.
Close correlation between absence of a deciduous
tooth and its permanent successor.

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 Teeth: Disturbances In Number
Hereditary Hypohidrotic Ectodermal Dysplasia

X-linked recessive affecting males or

autosomal recessive.
Defect in gene that encodes a
transmembrane protein expressed in
keratinocytes, hair follicles and sweat
glands (ectodermally derived structures).
Absent or lanugo type hair.
Reduction or absence of sweat glands:
inability to regulate body temperature.
Anodontia, or more commonly,
hypodontia: canines and 1st molars usually
present.
Abnormally shaped teeth: conical.
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 This child has sparse, fine hair, some
congenitally missing teeth, and thermal
regulation problems. What is the most likely
cause?
-Hereditary Hypohidrotic Ectodermal Dysplasia

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 Teeth: Disturbances In Number
Supernumerary Teeth
Teeth in excess of the normal number.
Single or multiple.
Erupted or impacted.
Shape: conical or like normal counterpart.
Maxilla >>>>mandible.
Mesiodens > paramolars (maxillary fourth molars or
distomolars) > maxillary lateral incisors.
In deciduous teeth: maxillary lateral incisor
Multiple and impacted in:
Cleidocranial dysplasia
Gardner syndrome

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 Mesiodense
a supernumerary teeth developing
between the maxillary central incisors
and is the most ommon of all
supernumerary teeth

ParaMolar
( 4th molar )
paramolars are usually buccally
places

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 Teeth: Disturbances In Eruption
To be discussed with Other Disorders of Teeth

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 Teeth: Disturbances In Shape
Dilaceration:
A sharp bend or angulation
involving the root of tooth.
Causes:
Trauma during tooth
development.
Continued root formation during a
curved or tortuous path of
eruption.
Idiopathic.
May complicate tooth
extraction:
Importance of preoperative
14 radiographs.
 Teeth: Disturbances In Shape
Taurodontism (Bull-like Teeth):

Elongated crown and apically placed

root furcation of the roots in molars
and occasionally premolars, resulting
in an enlarged rectangular coronal
pulp chamber.

Permanent, deciduous occasionally.

Probably a result of late invagination

of Hertwigs root sheath during
development.

Associated with:
Amelogenesis imperfecta
Down syndrome
Klinefelter syndrome

May
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complicate root canal treatment
 Teeth: Disturbances In Shape
Dens Invaginatus (Dens in Dente) tooth within a tooth
A developmental dental anomaly
characterized by a deep enamel-lined pit
that extends for varying depths into
underlying dentin, often displacing pulp
chamber and sometimes expanding the
root.
Mainly maxillary lateral incisor.
Mild form: deep lingual pit, common.
Cases that involve peg lateral: pit at tip of
conical crown.
Extreme form: dilated odontome.
Base of invagination:
Defective enamel and dentin.
Vulnerable to caries, pulpitis and pulp
necrosis.
Seldom reliable candidates for successful
RCT.
Deep lingual pits:
Early radiographic diagnosis and
prophylactic restoration.
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 Teeth: Disturbances In Shape
Dens Invaginatus (Dens in Dente)
Extreme form: dilated odontome. ( Pictures )
Base of invagination:
Defective enamel and dentin.
Vulnerable to caries, pulpitis and pulp necrosis.
Seldom reliable candidates for successful RCT.
Deep lingual pits:
Early radiographic diagnosis and prophylactic restoration.

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 Teeth: Disturbances in Shape
Supernumerary Cusps:
1. Cusp of Carabelli
Mesiolingual surface of permanent
maxillary 1st molar.
Causes no problems; requires no
treatment.
Normal anatomic variation.

2. Dens Evaginatus (Leong Premolar)

Cusp-like supernumerary focal
enamel protrusion on occlusal or
lingual surface of crown.
Present on region of central groove
between buccal and lingual cusps
of premolars.
Mostly in oriental races (Chinese,
Japanese, Malaysian,etc.).
May interfere with eruption.
Contains a pulp horn, attrition or
fracture results in exposure with
pulpal inflammation and necrosis.
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 Teeth: Disturbances in Shape
Supernumerary Cusps:

3. Talon Cusp
Uncommon but clinically significant.
Lingual aspect of maxillary centrals.
Cingulum portion and extends to incisal
edge.
Pits on lateral aspects should be
restored.
Contains prominent pulp horn.
If it interferes with occlusion, reduction
and endodontic treatment.
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 Teeth: Disturbances in Shape
Supernumerary Roots:
Most common in mandibular
premolars, canines and
mandibular third molars.

Significance: radiographs before

extraction or endodontic
treatment.

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 Teeth: Disturbances in Shape
Gemination
A single-rooted tooth with an
unusually wide, partly divided
crown or two separate crowns.
Incompletely divided tooth germ
(twinning).
Usually anterior teeth.
Clinically similar to fusion.
Affects deciduous and
permanent teeth.

Q: If we count the doubly crowned teeth as

one tooth, the patients have a normal
number of teeth. What is the condition?
- Gemination
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 Teeth: Disturbances in Shape
Fusion
Union between adjacent tooth germs at least
by dentin.
Unusually wide crown.
Complete or incomplete according to the
stage of development.
Fusion before calcification: involves enamel,
dentin, pulp and cementum.
Fusion at later stage: may be limited to roots.
Affects deciduous and permanent teeth.
Differentiated from gemination by counting
number of teeth.
Clinical significance: esthetic, crowding,
periodontal disease

Q: If we count the doubly crowned teeth as one

tooth, the patients have less than the normal
number of teeth by one tooth. What is the
condition?
- Fusion
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 Teeth: Disturbances in Shape
Concrescence
Union of roots of 2 or more teeth
caused by confluence of their
cementum.

Trauma or crowding with interseptal

bone loss.

Can occur before or after eruption.

Primarily affects permanent maxillary

molars.

Rarely more than 2 teeth affected.

Significance: tooth extraction

problems, importance of pre-
operative radiographs.

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 Teeth: Disturbances in Shape
Hypercementosis
Excessive deposits of cementum on root.
Bulbous or pear-shaped root:
Difficult extraction, bone removal
needed.
Common in:
Teeth with increased or decreased occlusal
forces.
Hyperpituitarism.
Paget disease of bone.
Chronic inflammation-periapical.
May cause concrescence.

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 Teeth: Disturbances in Shape
Cervical Enamel Extensions
Focal extensions of enamel
beyond the normally smooth
cervical cementoenamel
junction onto the root.
Primarily affect molars.
Contribute to periodontal
pocket formation.
Thought to play a role in
development of the buccal
bifurcation cyst.
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 Teeth: Disturbances in Shape
Enamel Pearls
Ectopic droplets of enamel that
primarily occur in bifurcation
and trifurcation areas on molar
roots.
Relatively uncommon.
Radiographically seen as 1-3
mm round radiopacities.
May exhibit a central core of
dentin.
Treatment not recommended;
leads to development of root
caries, external resorption, or
pulpitis.
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 Teeth: Disturbances in
Structure of Enamel
Acquired or Environmental Disturbances
Resultant defect: focal or generalized.
Defect related to:
Specific causative factor.
Duration of injury.
Stage of enamel formation at time of injury.
Usually affect either deciduous or permanent dentition.
Affect both enamel and dentin.

Hereditary
Genetically determined.
Both deciduous and permanent dentitions.
Either enamel or dentin.
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 Teeth: Disturbances in Structure of
Enamel
Acquired or Environmental Disturbances
1. Bacterial and viral infections (e.g. syphilis,
scarlet fever).
2. Inflammation.
3. Nutritional deficiencies (e.g. vitamins A, C, D,
calcium).
4. Chemical injuries (e.g. fluoride).
5. Trauma.
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Teeth: Disturbances In Structure
Of Enamel
Acquired or Environmental Disturbances
Focal or Localized Enamel Hypoplasia
Involving 1-2 teeth.
Often idiopathic.

Turner tooth ( Pictures )

Results from localized inflammation or
trauma during tooth development, e.g.
deciduous tooth develops caries or
trauma related abscess that affects
underlying permanent successor.

Depending on severity of injury affected

crown:
May have focal enamel hypoplasia
(enamel opacity) that may be smooth
with pitted areas.
or it may be grossly deformed with
yellowish or brownish discoloration.
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 Teeth: Disturbances In Structure Of
Enamel
Acquired or Environmental
Disturbances
Generalized Enamel Hypoplasia
1. Chronologic enamel hypoplasia
Short-term systemic factors
inhibit functioning ameloblasts
at a specific period:
Horizontal line of pits or grooves
that correspond to time of
development and duration of
insult.
Most cases involve teeth that
develop during the 1st year of
life: permanent incisors,
canines, and 1st molars.
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 Teeth: Disturbances In Structure Of
Enamel
Acquired or Environmental Disturbances
Generalized Enamel Hypoplasia
2. Congenital syphilis

Mulberry molars (Moon molars):

globular occlusal surfaces of 1st
molars.

Hutchinson incisors: notched,

screwdrivershaped incisors.

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 Teeth: Disturbances In Structure Of
Enamel
Acquired or Environmental Disturbances
Generalized Enamel Hypoplasia
3. Vitamin D, A and C deficiencies
Pitted type.

4. Exanthematous diseases: measles, chickenpox, scarlet fever.

Also pitted type.

5. Neonatal line:
Microscopically affecting deciduous teeth and first permanent
molars
Systemic insult during birth.
Enamel hypoplasia is more common in prematurely born children.

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 Teeth: Disturbances in 0

Structure of Enamel :
Acquired or Environmental Disturbances
Generalized Enamel Hypoplasia
6. Dental Fluorosis (Mottled Enamel):
Usually inconspicuous at levels < 1 ppm in
drinking water.
Chemically induced, interferes with
ameloblast function:
Adversely affects enamel matrix formation
and mineralization.
Effect proportionate to fluoride levels.
Teeth are usually resistant to dental caries.
Mild cases: smooth enamel with opaque
areas.
Moderate to severe mottling: pitting and
brownish discoloration.
Severe cases: soft and weak enamel, easy
wear and fracture.
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 Teeth: Disturbances in Structure of
Enamel
Hereditary Disturbances:
Amelogenesis Imperfecta
A heterogeneous group of hereditary disorders of
enamel formation.
Affect both deciduous and permanent dentition.
Confined to enamel, other dental tissues are normal.

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 Teeth: Disturbances in Structure of
Enamel
Hereditary Disturbances:
Amelogenesis Imperfecta (AI)
Normal enamel formation progresses through three stages:
1. Formative stage: deposition of organic matrix.
2. Mineralization stage: primary mineralization of matrix.
3. Maturation stage: secondary mineralization; crystallites mature and
enlarge.
Witkop and Sauk classification of AI: 3 major types correlate with
defects in these stages.
1. Hypoplastic type: decreased enamel matrix formation caused by
disturbance in ameloblast function.
2. Hypocalcified: severe defect in mineralization of matrix.
3. Hypomaturation: less severe alteration in mineralization with focal
or generalized areas of immature enamel crystallites.

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 Teeth: Disturbances in Structure of
Enamel
Hereditary Disturbances:
Amelogenesis Imperfecta (AI)
Witkop and Sauk classification of AI: 3 major types correlate with defects
in these stages.
1. Hypoplastic type: decreased enamel matrix formation caused by
disturbance in ameloblast function.

Smooth localized Rough generalized

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 Hypoplastic amelogenesis imperfecta
Q: Hypoplastic amelogenesis imperfecta. What are the radiographic
features?
-The radiodensity of the enamel is greater than that of dentin but the
quantity of enamel is much lesser than that of dentine

Enamel thinner than normal in focal or generalized areas.

Radiodensity > dentin.

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 Teeth: Disturbances in Structure of Enamel
Hereditary Disturbances:
Amelogenesis Imperfecta (AI)
Witkop and Sauk classification of AI: 3 major
types correlate with defects in these stages.

2. Hypocalcified: severe defect in

mineralization of matrix.

Q: Hypocalcified amelogenesis
imperfecta. What are the radiographic
features?
-The radiodensity of the enamel is less than
that of dentin

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 Teeth: Disturbances in Structure of
Enamel
Hereditary Disturbances:
Amelogenesis Imperfecta (AI)
Witkop and Sauk classification of AI: 3 major types correlate
with defects in these stages.
3. Hypomaturation: less severe alteration in mineralization with
focal or generalized areas of immature enamel crystallites.

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Snow-capped pattern Pigmented pattern
Hypomaturation amelogenesis imperfecta
Q: hypomaturation amelogenesis imperfecta.
What are the radiographic features?
- The radiodensity of the enamel is the same as
that of dentin .

This is another example

of snow-capped pattern

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 Witkop/Sauk Classification of
Amelogenesis Imperfecta
Hypoplastic:
Pitted, autosomal dominant
Local, autosomal dominant
Smooth, autosomal dominant
Rough, autosomal dominant
Rough, autosomal recessive
Smooth, X-linked dominant

Hypocalcified:
Autosomal dominant
Autosomal recessive

Hypomaturation:
Hypomaturation - hypoplastic with taurodontism, autosomal dominant.
X-linked recessive.
Pigmented, autosomal recessive
Snow capped teeth.
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 Teeth: Fisturbances in
Structure of Enamel
Hereditary Disturbances:
Amelogenesis Imperfecta (AI)
Clinical Features
Can be remarkably different among types.
In some types teeth appear essentially normal.
In other types, they may be extremely unsightly.
Both dentitions commonly affected.
In x-linked subtypes, appearance differs between
males and females.
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 Teeth: Disturbances in Structure
of Enamel
Hereditary Disturbances:
Amelogenesis Imperfecta (AI)

Clinical features useful for differentiating the 3 basic types:

1. Hypoplastic type:
Enamel thinner than normal in focal or generalized areas.
Radiodensity > dentin.

2. Hypocalcified type:
Enamel of normal thickness but softer than normal.
Can be easily removed with blunt instrument.
Less radiodense than dentin.

3. Hypomaturation type:
Enamel of normal thickness but not of normal hardness and translucency.
Can be pierced by an explorer point with firm pressure and can be chipped away
from underlying normal dentin.
Radiodensity about the same as dentin.
Mildest form: snow-capped teeth.
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 Required :
Table 1.2 Etiology of
developmental
abnormalities of
enamel in your main
textbook (Soames &
Southam 4th edition).

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 Teeth: Disturbances in Structure of Dentin
Hereditary Disturbances:
Dentinogenesis Imperfecta (DI)
A group of inherited disorders
of dentin formation . Enamel
chips off due to lack of support by
weak dentin.

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 Teeth: Disturbances in Structure of
Dentin
Hereditary Disturbances:
Dentinogenesis Imperfecta (DI)
A group of inherited disorders of dentin formation
classified into 3 types:
Type I: always occur with osteogenesis imperfecta (OI)
- autosomal dominant.

Type II: not associated with OI hereditary opalescent

dentin autosomal dominant 1 in 8000.

Type III: Brandywine type, occurs in a racial isolate in

Maryland similar to I and II along with multiple pulp
exposures in deciduous teeth autosomal dominant.
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Teeth: Disturbances in Structure of Dentin
Hereditary Disturbances:
Dentinogenesis Imperfecta (DI)
Clinical Features
Both dentitions affected.
Teeth are opalescent with color ranging from bluishgray to brown to yellowish.
Dentin is abnormally soft.
Enamel is normal but chips away easily:
Inadequate support by dentin.
DEJ lacks the usual scalloping.
Exposed dentin undergoes rapid attrition.

Radiographic Features
Teeth in types I and II are similar:
Bulbous crowns with constricted CEJ
and thin roots.
Varying degrees of pulp chamber
obliteration.
Teeth in type III may be similar to
those in types I and III, or may exhibit
extremely large pulp chambers surrounded by a thin shell of dentin (shell teeth). Pic
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Teeth: Disturbances in Structure of Dentin
Hereditary Disturbances:
Dentinogenesis Imperfecta (DI)
Histopathologic Features
Enamel is normal.
Severely dysplastic dentin: focal areas of
amorphous matrix with globular and
interglobular areas of mineralization.
Dentinal tubules are disoriented, irregular, widely
spaced and larger than normal.

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Teeth: Disturbances in Structure of Dentin
Hereditary Disturbances:
Dentin Dysplasia
A rare autosomal dominant hereditary disorder characterized by
abnormal dentin formation and abnormal pulp morphology.
Classified into 2 types:
1. Type I: Radicular dentin dysplasia (rootless teeth)

2. Type II: Coronal dentin dysplasia.

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Teeth: Disturbances in Structure of Dentin
Hereditary Disturbances: Dentin Dysplasia type I:
Radicular dentin dysplasia (rootless teeth)

More common than type II.

Both deciduous and permanent dentitions
affected.
Color within normal range, sometimes cervical
areas may how slight bluish or brownish
translucency.
Often normal eruption pattern.
Increased mobility and premature exfoliation.
Short blunt roots.
W-shaped roots of mandibular molars.
Obliterated or chevron- shaped pulp chambers.
Periapical radiolucencies representing abscesses,
granulomas or cysts.
Deciduous teeth exhibit obliterated pulp
chambers.
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Teeth: Disturbances in Structure of Dentin
Hereditary Disturbances:
Dentin Dysplasia
Type II: Coronal dentin dysplasia
Both dentitions are affected
Clinical appearance of deciduous is
different from permanent.
Deciduous teeth:
Features similar to dentinogenesis
imperfecta:
Translucent hue.
Pulp obliteration.
Normal root shape and length
Permanent teeth:
Normal color.
Normal root length.
Abnormally large pulp chambers with
abnormal radicular extension: thistle
or flame-shaped root pulp.
Pulp stones and narrowed canals.
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Teeth: Disturbances in
Structure of Dentin
Regional Odontodysplasia (Ghost Teeth)
Non-hereditary, sporadic.
Local ischemic cause postulated.
Localized developmental abnormality of
several adjacent teeth.
Defective formation of enamel and
dentin.
Abnormal pulp and follicle calcifications.
Maxilla > mandible.
Affects several contiguous teeth in a
single quadrant.
Most commonly in permanent dentition.
Delay or failure of eruption.
Deformed, discolored, soft teeth.
Marked decrease in radiodensity-ghost
teeth.
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 Teeth: Disturbances in
Structure of Cementum
Hypophosphatasia
Autosomal recessive or dominant condition.
Deficiency of alkaline phosphatase in serum and tissues.
Disorder of bone mineralization: rickets and osteomalacia-like picture.

Dental manifestation:
Absence (inadequate formation) of cementum
Enamel normal, pulp is large.
Alveolar bone loss.
+/- hypoplastic enamel defects.
Delayed formation and eruption of dentition.
Premature loss of primary teeth.
Spontaneous loss of permanent teeth.

End of parts I & II

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