Chapter 5

Developmental Disorders

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 Outline
Embryonic Development of the Face, Oral
Cavity, and Teeth
Developmental Soft Tissue Abnormalities
Developmental Cysts
Developmental Abnormalities of Teeth

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 Developmental Disorders
(pg. 156)
A failure during the process of cell division
and differentiation into various tissues and
structures
Some may be identified clinically, by
radiographic examination, biopsy, or histologic
examination.

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 Developmental Disorders (cont.)
(pg. 156)
Inherited disorder
Caused by an abnormality in genetic makeup
Congenital disorder
Present at birth
May be inherited or developmental
The cause of most congenital abnormalities is
unknown.

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 Embryonic Development of the
Face, Oral Cavity, and Teeth
Face
Oral and Nasal Cavities
Teeth

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 Face
(pgs. 156-157)
During the third week, ectoderm infolds to
form the stomodeum, the primitive oral
cavity.
The frontal process is above and the first
branchial arch is below.
The first branchial arch divides into two
maxillary processes and the mandibular
process.

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 Face (cont.)

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 Face (cont.)
Two pits develop on the frontal process.
They divide the frontal process into three parts.
The median nasal process
The right lateral nasal process
The left lateral nasal process
The median nasal process grows downward
between the maxillary processes to form the
globular process.
This will form the philtrum.

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 Oral and Nasal Cavities
(pg. 157)
Premaxilla
Forms from the globular process
Lateral palatine processes
Form from the maxillary process
The lateral palatine processes fuse with
the premaxilla, creating a Y-shaped
pattern.
The body of the tongue develops from the
first branchial arch.
The base of the tongue forms from the
second and third branchial arches.
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 Teeth
(pgs. 157-158)
Odontogenesis
Takes place in about the fifth week of life
Involves ectoderm and ectomesenchyme
Begins with formation of a band of ectoderm in
each jaw called the primary dental lamina
Ten small knoblike proliferations develop on
the primary dental lamina in each jaw.
Each extends into underlying mesenchyme.

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 Teeth (cont.)

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 Teeth (cont.)
The tooth germ has three parts.
The enamel organ
Produces enamel
The dental papilla
Forms the dental pulp
The dental sac or follicle
The follicle provides cells that form cementum, the
periodontal ligament, and alveolar bone.
Cementum is formed after the crown is complete.

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 Developmental Soft Tissue
Abnormalities
(pgs. 158-159)
Ankyloglossia
Commissural Lip Pits
Lingual Thyroid

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 Ankyloglossia
(pg. 158)
An extensive adhesion of the tongue to the floor
of the mouth
Due to the complete or partial fusion of the lingual
frenum
Some patients may have no adverse effects, while others
may have difficulty with speech.
It may just involve mucosa, or it may be muscular and
thick.
Treatment
Frenectomy
This works nicely with a laser.

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Ankyloglossia (cont.)

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 Commissural Lip Pits
(pgs. 158-159)
Epithelium-lined blind tracts located at the
corners of the mouth (commissure)
May be shallow or several millimeters deep.
Congenital lip pits may also be observed near
the midline of the vermilion border.
Treatment
None

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Commissural Lip Pits (cont.)

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 Lingual Thyroid
(pg. 159)
A small mass of thyroid tissue located on
the tongue
Results from the failure of the primitive thyroid
tissue to migrate from its developmental
location in the area of foramen cecum on the
posterior portion of the tongue to its normal
position in the neck

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 Lingual Thyroid (cont.)
Appears as a smooth nodular mass posterior
to circumvallate papillae at the base of the
tongue.
TreatmentS
It may be removed if it is obstructive, providing
the patient has other functioning thyroid tissue.

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 Developmental Cysts
Odontogenic Cysts
NonodontogenicS Cysts
Pseudocysts

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 Developmental Cysts (cont.)
(pg. 159) (Box 5-1)
An abnormal fluid-filled epithelium-lined
sac or cavity
Found throughout the body, including the
head and neck region

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 Developmental Cysts (cont.)
Developmental cysts are classified as to
whether they are odontogenic or
nonodontogenic.
They are also classified according to location,
cause, origin of the epithelial cells, and
histologic appearance.

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 Developmental Cysts (cont.)
They can cause expansion of bone.
Intraosseous cysts
Occur within bone
Extraosseous cysts
Occur in soft tissue
Cysts within bone generally appear as
well-circumscribed radiolucencies.
They may appear as unilocular or multilocular.

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 Odontogenic Cysts
Dentigerous Cyst
Eruption Cyst
Primordial Cyst
Odontogenic Keratocyst
Calcifying Odontogenic Cyst
Lateral Periodontal Cyst and Gingival Cyst

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 Dentigerous Cyst
(Follicular Cyst)
(pgs. 159-160)
Forms around the crown of an unerupted
or developing tooth
The epithelial lining originates from the
reduced enamel epithelium after the crown has
formed and calcified.
Most commonly around the crown of an
unerupted or impacted third molar

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Dentigerous Cyst
(Follicular Cyst) (cont.)

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 Dentigerous Cyst
(pgs. 160-161)
Radiographic
A well-defined, unilocular radiolucency around the crown
of an unerupted or impacted tooth
Histologic
The lumen is most characteristically lined with cuboidal
epithelium surrounded by a wall of connective tissue.
Treatment
Removal of the cyst
There is some risk of cystic transformation into a
neoplasm.

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Dentigerous Cyst (cont.)

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 Eruption Cyst
(pg. 160)
Similar to a dentigerous cyst
Found in the soft tissue around the crown of an
erupting tooth
Treatment
None

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 Primordial Cyst
(pgs. 160-161)
Develops in the place of a tooth
Most commonly in place of a third molar
Most often seen in young adults and
discovered on radiographic examination

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 Primordial Cyst

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 Primordial Cyst (cont.)
Histologic
The lumen is lined by stratified squamous
epithelium surrounded by parallel bundles of
collagen fibers.
It may prove to be an odontogenic keratocyst
or a lateral periodontal cyst.
Treatment
Surgical removal
The risk of recurrence depends on the
diagnosis.

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Odontogenic Keratocyst (OKC)
(pgs. 161-163)
Characterized by histologic appearance
and frequent recurrence
The lumen of the cyst contains perakeratin.
Most often seen in the mandibular third
molar region
Can move teeth and cause resorption

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 Odontogenic Keratocyst
(pgs. 161-162)
Histologic
The lumen is lined by epithelium that is 8 to 10
cell layers thick and surfaced by parakeratin.
Radiographic
Frequently appears as a well-defined,
multilocular, radiolucent lesion

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Odontogenic Keratocyst (cont.)
(pgs. 161-162)
Treatment
Due to a high recurrence rate, surgical excision and
osseous curettage are recommended.

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 Calcifying Odontogenic Cyst
(COC)
(pg. 163)
A nonaggressive, cystic lesion lined by
odontogenic epithelium
Closely resembles an ameloblastoma
Has a characteristic feature called ghost cells

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 Lateral Periodontal Cyst and
Gingival Cyst
(pgs. 163-164)

Most often seen in the mandibular cuspid and premolar

area
An asymptomatic, unilocular or multilocular radiolucent
lesion on the lateral surface of a tooth root
Found most often in males

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 Lateral Periodontal Cyst and
Gingival Cyst (cont.)
(pgs. 163-164)
Histologic
A gingival cyst has the same type of lining, but
is located in the soft tissue.
A thin band of stratified squamous epithelium
lines the cyst
Treatment
Surgical excision

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Lateral Periodontal Cyst and
Gingival Cyst (cont.)

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 Nonodontogenic Cysts
Nasopalatine Canal Cyst
Median Palatine Cyst
Globulomaxillary Cyst
Median Mandibular Cyst
Nasolabial Cyst
Branchial Cleft Cyst (Lymphoepithelial
Cyst)
Epidermal Cyst
Dermoid Cyst and Benign Cystic Teratoma
Thyroglossal Tract Cyst
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 Nasopalatine Canal Cyst
(Incisive Canal Cyst)
(pg. 164)
Located within the nasopalatine canal or
the incisive papilla
Most commonly seen in men between 40 and
60 years old
Usually asymptomatic
May see a small, pink bulge near the apices
and between the roots of the maxillary central
incisors on the lingual surface

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 Nasopalatine Canal Cyst
(pg. 164)
Radiographic
A well-defined, radiolucent lesion
May be oval or heart-shaped
Histologic
Lined by epithelium varying from stratified
squamous to pseudostratified ciliated columnar
epithelium
Treatment
Surgical excision

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Nasopalatine Canal Cyst (cont.)

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 Median Palatine Cyst
(pgs. 164-165)
A well-defined, unilocular radiolucency
Located in the midline of the hard palate
Histologic
Lined with stratified, squamous epithelium
surrounded by dense fibrous connective tissue
Treatment
Surgical removal

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Median Palatine Cyst (cont.)

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 Globulomaxillary Cyst
(pg. 165)
A well-defined, pear-shaped radiolucency
found between the roots of the maxillary
lateral incisor and cuspid
Was once thought to be a fissural cyst, now
believed to be of odontogenic epithelial origin
Treatment
Surgical removal

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Globulomaxillary Cyst (cont.)

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 Median Mandibular Cyst
(pg. 165)
A rare lesion located in the midline of the
mandible
Lined with squamous epithelium
Radiographic
A well-defined radiolucency below the apices of
mandibular incisors
Treatment
Surgical removal

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 Nasolabial Cyst
(pgs. 165-166)
A soft tissue cyst
Thought to originate from the lower anterior
portion of the nasolacrimal duct
Observed in adults from 40 to 50 years of age
4:1 ratio in favor of females

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 Nasolabial Cyst (cont.)
(pgs. 165-166)
Clinical
An expansion or swelling in the mucobuccal
fold in the area of the maxillary canine and the
floor of the nose
Histologic
Lined with pseudostratified, ciliated columnar
epithelium and multiple goblet cells
Treatment
Surgical excisions

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Nasolabial Cyst (cont.)

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 Branchial Cleft Cyst
(Lymphoepithelial Cyst)
(pgs. 165-166)
Most commonly found in major salivary
glands
A stratified squamous epithelial lining
surrounded by a well-circumscribed
component of lymphoid tissue
Appears to arise from epithelium trapped
in a lymph node during development

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 Branchial Cleft Cyst
(Lymphoepithelial Cyst) (cont.)

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 Branchial Cleft Cyst
(Lymphoepithelial Cyst) (cont.)
Most commonly found intraorally on the
floor of the mouth and the lateral borders
of the tongue
Appears as a pinkish, yellow raised nodule
Treatment
Surgical excision

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 Epidermal Cyst
(pg. 166)
A raised nodule on the skin of the face or
neck
May be noted intraorally on occasion
Histologic
Lined by keratinizing epithelium the resembles
the epithelium of the skin
The lumen is usually filled with keratin scales
Treatment
Surgical excision

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 Dermoid Cyst and Benign Cystic
Teratoma
(pg. 166)
A developmental cyst often present at birth
or noted in young children
It is usually found on the floor of the mouth
when it is located in the oral cavity.
May have a doughy consistency when
palpated

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 Dermoid Cyst
Histologic
Lined by orthokeratinized, stratified squamous
epithelium surrounded by a connective tissue
wall
The lumen is usually filled with keratin
Hair follicles, sebaceous glands, and sweat
glands may be seen in the cyst wall
Benign cystic teratoma
Resembles a dermoid cyst
Treatment
Surgical excision

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 Thyroglossal Tract Cyst
(pgs. 166-167)
Forms along the tract the thyroid gland
follows in development
Most often found in young individuals under 20
years of age
No sex predilection
Treatment
Excision of the cyst and tract

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Thyroglossal Tract Cyst (cont.)

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 Pseudocysts
Static Bone Cyst
Simple Bone Cyst
Aneurysmal Bone Cyst

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 Static Bone Cyst (Lingual Mandibular
Bone Cavity) (Stafne Bone Cyst)
(pgs. 166, 168)
A pseudocyst (not a true cyst)
A well-defined cystlike radiolucency may be
observed on radiograph in the posterior region
of the mandible inferior to the mandibular
canal.
Caused by a lingual depression in the
mandible
Treatment
None

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Static Bone Cyst (Lingual Mandibular
Bone Cavity) (Stafne Bone Cyst)
(cont.)

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 Simple Bone Cyst
(Traumatic Bone Cyst)
(pg. 168)
A pathologic cavity in bone that is not lined with
epithelium
May be associated with trauma
Radiographic
A well-defined unilocular or multilocular radiolucency
Characteristically shows scalloping around roots of teeth
Treatment
Curettage on the wall lining the void

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 Simple Bone Cyst
(Traumatic Bone Cyst) (cont.)

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 Aneurysmal Bone Cyst
(pg. 168)
A pseudocyst
Consists of blood filled spaces surrounded by
multinucleated giant cells and fibrous
connective tissue

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Aneurysmal Bone Cyst (cont.)
Radiographic
Multilocular appearance honeycomb, soap
bubble
Usually seen in persons less than 30 years old
Slight predilection for females
Treatment
Surgical excision

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 Developmental Abnormalities of
Teeth
Abnormalities in the Number of Teeth
Abnormalities in the Size of Teeth
Abnormalities in the Shape of Teeth
Abnormalities of Tooth Structure
Abnormalities of Tooth Eruption

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 Abnormalities in the Number of
Teeth
Anodontia
Hypodontia
Supernumerary Teeth

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 Anodontia
(pgs. 168-169)
The congenital lack of teeth
Total anodontia is lack of all teeth.
May be associated with ectodermal dysplasia

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 Hypodontia
(pg. 169)
The lack of one or more teeth
May affect either deciduous or permanent teeth
The most common missing permanent
teeth are
Mandibular and maxillary third molars
Maxillary lateral incisors
Mandibular second premolars
The most common missing deciduous
tooth is the mandibular incisor.

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 Hypodontia (cont.)

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 Hypodontia (cont.)
Usually identified during clinical and
radiographic examination
Tends to be familial
Treatment
May require prosthetic replacement
Orthodontic evaluation and treatment may be
necessary
May be a component of a syndrome

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 Supernumerary Teeth
(pgs. 169-170)
Extra teeth found in the dental arches
May result from formation of extra tooth buds
in the dental lamina or from the cleavage of
already existing tooth buds
May occur in either deciduous or
permanent dentition
Most often seen in the maxilla
Most are found on radiographs

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Supernumerary Teeth (cont.)

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 Supernumerary Teeth (cont.)
(pgs. 169, 171-172)
Mesiodens
The most common supernumerary tooth
Located between maxillary incisors
May be inverted when seen on radiographs
Distomolar
The second most common supernumerary
tooth
Located distal to the third molar

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Supernumerary Teeth (cont.)

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 Supernumerary Teeth (cont.)
Treatment
Erupted teeth may require removal if they
cause crowding, malposition of adjacent teeth,
or noneruption of normal teeth.
Nonerupted teeth should be extracted
because a risk exists for cyst development
around the crown.
Multiple supernumerary teeth may be
associated with cleidocranial dysplasia or
Gardner syndrome.

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Abnormalities in the Size of Teeths
Microdontia
Macrodontia

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 Microdontia
(pgs. 170, 172) (Fig. 5-24)
One or more teeth are smaller than normal.
True generalized microdontia
Seen in a pituitary dwarf; all teeth are smaller than
normal
Generalized relative microdontia
Normal-sized teeth appear small in a large jaw.
Microdontia involving a single tooth
Maxillary lateral incisor and maxillary third molar are
the most commonly involved teeth.

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 Microdontia (Cont.)

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 Macrodontia
(pg. 170)
One or more teeth are larger than normal.
True generalized macrodontia
Seen in cases of pituitary giantism
Relative generalized macrodontia
Large teeth in a small jaw
Macrodontia affecting a single tooth
May be seen in cases of facial hemihypertrophy

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 Abnormalities in the Shape of
Teeths
Gemination
Fusion
Concrescence
Dilaceration
Enamel Pearl
Talon Cusp
Taurodontism
Dens in Dente
Dens Evaginatus
Supernumerary Rootsss
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 Gemination
(pgs. 170-171, 173)
Occurs when a single tooth germ attempts
to divide in two
Appears as two crowns joined together by a
notched incisal area
Radiographically, usually one single root and
one common pulp canal exist
The patient has a full complement of teeth.

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 Gemination (cont.)

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 Fusion
(pgs. 171-173)
The union of two normally separate
adjacent tooth germs
Appears as a single large crown that occurs in
place of two normal teeth
Radiographically, either separate or fused roots
and root canals are seen.
The patient is usually short one tooth.

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 Fusion (cont.)

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 Concrescences
(pgs. 172, 174)
Two adjacent teeth are united by
cementum.
Usually discovered on radiograph
If one of the teeth needs to be removed, both
usually come out (two for the price of one).

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Concrescences (cont.)

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 Dilaceration
(pgs. 172, 174-175)
An abnormal curve or bend in the root of a
tooth
Usually discovered on radiograph
May cause a problem if the tooth must be
removed or a root canal performed

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 Dilaceration (cont.)

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 Enamel Pearl
(pgs. 174, 176)
A small, spherical enamel projection on a
root surface
Usually found on maxillary molars
Radiographically, it appears as a small,
spherical radiopacity.
Difficult to instrument with curettes or scalers
Removal may be necessary if periodontal
problems occur in the furcation.

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Enamel Pearl (cont.)

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 Talon Cusp
(pgs. 174, 176)
An accessory cusp located in the cingulum
area of a maxillary or mandibular
permanent incisor
Contains a pulp horn
May interfere with occlusion

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 Talon Cusp (cont.)

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 Taurodontism
(pgs. 174, 176)
The teeth have elongated pulp chambers
and short roots.
May occur in both deciduous and permanent
dentition
Identified on radiographs

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Taurodontism (cont.)

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 Dens in Dente
(pgs. 175, 177)
Occurs when the enamel organ
invaginates into the crown of a tooth
before mineralization
Radiographically, it appears as a toothlike
structure within a tooth.
Vulnerable to caries, pulpal infection, and
necrosis
A restoration can be placed in the pit if the
tooth is vital.

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Dens in Dente (cont.)

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 Dens Evaginatus
(pgs. 176-177)
An accessory enamel cusp found on the
occlusal tooth surface
Most often seen on mandibular premolars
May cause occlusal problems

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Dens Evaginatus (cont.)

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 Supernumerary Roots
(pgs. 176-177)
May involve any tooth
Most commonly, maxillary and mandibular third
molars if multirooted teeth are involved.
May become clinically significant if removal or
endodontia is necessary

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Supernumerary Roots

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 Abnormalities of Tooth Structure
Enamel Hypoplasia
Enamel Hypocalcification
Endogenous Staining of Teeth
Regional Odontodysplasia

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 Enamel Hypoplasia
(pgs. 177-179)
The incomplete or defective formation of enamel
Can affect either deciduous or permanent dentition
May be due to many factors
Amelogenesis imperfecta
Febrile illness (measles, chickenpox, scarlet fever)
Vitamin deficiency
Infection of a deciduous tooth
Ingestion of fluoride
Congenital syphilis
Birth injury, premature birth
Idiopathic factors

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Enamel Hypoplasia Caused by Febrile
Illness or Vitamin Deficiency
(pgs. 177-178)
Ameloblasts are one of the most sensitive
cell groups in the body.
Any serious systemic disease or severe
nutritional deficiency can produce enamel
hypoplasia.
One or more horizontal rows of tiny, deep pits
are seen traversing the affected tooth surface.

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Enamel Hypoplasia Caused by Febrile
Illness or Vitamin Deficiency (cont.)

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Enamel Hypoplasia Resulting from
Local Infection or Trauma
(pg. 178)
Enamel hypoplasia of an adult tooth may
result from infection of a deciduous tooth.
A single tooth is usually affected; it is referred
to as a Turner tooth.
The color of the enamel may range from yellow
to brown, or severe pitting and deformity may
be involved.

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Enamel Hypoplasia Resulting from
Fluoride Ingestion
(pg. 178)
Affected teeth exhibit a mottled
discoloration of enamel.
Ingestion of water with 2 to 3 times the
recommended amount leads to white flecks
and chalky opaque areas of enamel.
Four times the recommended amount of
fluoride causes brown or black staining.

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Enamel Hypoplasia Resulting from
Fluoride Ingestion (cont.)

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Enamel Hypoplasia Resulting from
Congenital Syphilis
(pgs. 178-179)
Congenital syphilis is transmitted from an
infected mother to her fetus via the
placenta.
This may result in enamel hypoplasia of adult
incisors and first molars.
Hutchinson incisors are shaped like screwdrivers.
Mulberry molars have a berrylike appearance.

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Enamel Hypoplasia Resulting from
Congenital Syphilis (cont.)

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 Enamel Hypoplasia Resulting from Birth
Injury, Premature Birth, or Idiopathic Factors
(pg. 179)
Enamel hypoplasia may occur due to
trauma or injury at the time of birth.
Even a mild illness or systemic problem can
result in enamel hypoplasia.

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 Enamel Hypocalcification
(pg. 179)
A developmental anomaly resulting in a
disturbance or the maturation of the
enamel matrix
Usually appears as a chalky, white spot on the middle
third of smooth crowns
The underlying enamel may be soft and susceptible to
caries.

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Endogenous Staining of Teeth
(pg. 179)
The result of deposition of substances
circulating systemically during tooth
development
May be due to
Tetracycline stain
Erythroblastosis fetalis Rh incompatibility
Neonatal liver disease
Congenital porphyria an inherited metabolic
disease

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 Regional Odontodysplasia
(Ghost Teeth)
(pgs. 179-180)
One or several teeth in the same quadrant
exhibit a marked reduction in radiodensity
and a characteristic ghostlike appearance.
Very thin enamel and dentin are present
May affect primary or adult dentition
Usually treated by extraction

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Regional Odontodysplasia
(Ghost Teeth) (cont.)

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Abnormalities of Tooth Eruption
(pgs. 180-181)
Impacted and embedded teeth
Impacted teeth cannot erupt due to an
obstruction.
Embedded teeth do not erupt due to lack of
eruptive force.
Ankylosed teeth

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Impacted and Embedded Teeth
(pg. 180)
Any tooth can be impacted.
Third molar impactions are classified according
to the position of the tooth.
Teeth can be completely impacted in bone
or they may be partially impacted.
Partially impacted teeth are prone to infection.

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Impacted and Embedded Teeth
(cont.)

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 Impacted and Embedded Teeth
(cont.)
Impacted teeth may be surgically removed
to prevent odontogenic cyst and tumor
formation or damage to adjacent teeth.
Partially impacted third molar teeth are
removed to prevent infections.
The optimal time is between 12 and 24 years
of age.

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 Ankylosed Teeth
(pgs. 180-181)
Tooth cementum fused to bone
Prevents exfoliation of the deciduous tooth and
eruption of the underlying adult tooth
The ankylosed deciduous tooth appears
submerged and has a different sound when
percussed (kind of a dull thud).

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Ankylosed Teeth (cont.)

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 Ankylosed Teeth (cont.)
The periodontal ligament space is lacking.
Difficult to extract
Removal of deciduous teeth is necessary for
eruption of the adult successor.
Removal of adult teeth may be necessary to
prevent malocclusion, caries, and periodontal
disease.

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 Discussion Questions
What developmental soft tissue abnormalities
may be observed within the oral cavity?
What developmental cysts may be observed
within the oral cavity?
What developmental abnormalities of teeth
may be observed within the oral cavity?

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