Anomalies of the

3 Developing Dentition
Clifton O. Dummett, Jr. and Sarat Thikkurissy

A variety of dental anomalies are associated with defects

in tooth development precipitated by hereditary, sys-
temic, traumatic, or local factors. Numerous systems have
OUTLINE

Anomalies of Number
been used to classify dental anomalies, each with merit. The
one used in this text categorizes them in terms of abnormali- Hyperdontia
ties in tooth number, size, shape, structure, and color.1 The Hypodontia
advantage of this system is that the categories can be related Anomalies of Size
to the stages of tooth development in which the respective Microdontia and Macrodontia
anomalies are thought to originate. These stages of dental
development are discussed in Chapter 12. The reader is also Fusion
encouraged to review textbooks on dental histology, dental Gemination
embryology, and orofacial genetics for more in-depth Anomalies of Shape
information. Dens Evaginatus
Dens in Dente
Anomalies of Number Taurodont
Dilaceration
Alterations in tooth number result from problems during Anomalies of Structure
the initiation or dental lamina stage of dental development.
In addition to hereditary patterns producing extra or missing Enamel
teeth, physical disruption of the dental lamina, overactive Amelogenesis Imperfecta
dental lamina, and failure of dental lamina induction by Environmental Enamel Hypoplasia
ectomesenchyme are several examples of etiologic factors Localized Enamel Hypoplasia
that affect tooth number.1 Enamel Hypocalcification
Dentin
HYPERDONTIA Dentin Dysplasia
Hyperdontia and supernumerary teeth are terms describing Regional Odontodysplasia
an excess in tooth number that can occur in both the primary Cementum
and permanent dentitions. Reports on the incidence of Anomalies of Color
hyperdontia include values as high as 3%, with males being
affected twice as frequently as females.2 Ninety percent to
98% of supernumerary teeth occur in the maxilla, with the
permanent dentition being more frequently affected than
the primary dentition. The most common supernumerary
tooth is the mesiodens, which occurs in the palatal midline (Figure 3-1, B), or shapes that duplicate molar anatomy.
and can assume a number of shapes and positions relative From a clinical standpoint, the tuberculate, or barrel-shaped,
to the adjacent teeth. The majority tend to be located palatal supernumeraries generate the most severe complications
to the central incisors.3 with respect to difficulty of removal and adverse effects on
As reported by Primosch in 1981, supernumerary teeth adjacent teeth, such as impaction or ectopic eruption. Addi-
are morphologically classified as either supplemental or tional complications associated with supernumeraries
rudimentary.2 Supplemental supernumerary teeth (Figure include dentigerous cyst formation, pericoronal space ossi-
3-1, A) duplicate the typical anatomy of posterior and ante- fication, and crown resorption.3 It is important in supernu-
rior teeth. Rudimentary supernumerary teeth are dysmor- merary tooth detection to rule out the presence of odontoma
phic and can assume conical forms, tuberculate forms in light of the fact that the morphologic characteristics of a

54
 CHAPTER 3 Anomalies of the Developing Dentition 55

A B
■ FIGURE 3-1 A, Supernumerary teeth: supplemental morphology. B, Supernumerary teeth: rudimentary, tuberculate morphology.

compound odontoma are similar to those of supernumerary ■ TABLE 3-1

teeth.
Cleft lip and palate (CLP) commonly demonstrates an Syndromes Demonstrating
excess or deficiency in the normal complement of teeth and Supernumerary Teeth
provides a clear example of physical disruption of the dental Condition Characteristics
lamina as an etiologic factor. Patients with CLP have been Apert syndrome Scaphocephaly, craniosynostosis, bilateral
reported to have an increased incidence of hyperdontia, with syndactyly, midface hypoplasia
reports indicating an incidence of up to 5%.4 Classic syn- Cleidocranial Aplastic clavicles, frontal bossing,
dromes involving supernumerary teeth are summarized in dysplasia hypoplastic midface
Table 3-1; cleidocranial dysplasia has the highest association Gardner syndrome Osteomas, epidermoid cysts, odontomas,
with this dental anomaly. intestinal polyps
HYPODONTIA Down syndrome Brachycephaly, mental retardation,
epicanthal folds
Hypodontia, or congenital tooth absence, is a deficiency in
Crouzon disease Craniosynostosis, exophthalmos,
tooth number. Familial heredity patterns account for the
hypoplastic midface
largest etiologic correlation with patterns of hypodontia.
Sturge-Weber Angiomatosis and calcification of
Incidence reports identify a range of 1.5% to 10% excluding
syndrome leptomeninges, seizures, port-wine
third molars in U.S. populations.5 The most frequently
nevi of face
occurring congenitally absent permanent tooth, excluding
the third molar, tends to be the mandibular second bicuspid Oral-facial-digital Hypoplastic alar cartilage, cleft tongue,
(3.4%), followed by the maxillary lateral incisor (2.2%).6 syndrome clinodactyly
There is a high correlation between primary tooth absence Hallermann-Streiff Dyscephaly, mandibular hypoplasia,
and permanent tooth absence.7-9 Ectodermal dysplasia (ED) syndrome hypotrichosis
represents a group of classic syndromes characterized by
oligodontia or multiple congenitally missing teeth. The most
common is hypohidrotic ED, followed in descending fre- ■ BOX 3-1
quency of occurrence by hidrotic ED, ectrodactyly ED plus
SYNDROMES MANIFESTING BOTH
cleft lip and palate, Rapp-Hodgkin ED, and Robinson-type
HYPERDONTIA AND HYPODONTIA
ED (Figure 3-2). Other conditions involving hypodontia are
summarized in Table 3-2 and those involving both hyper- • Down syndrome
dontia and hypodontia in Box 3-1. • Oral-facial-digital syndrome type 1
• Crouzon disease
• Hallermann-Streiff syndrome
Anomalies of Size
MICRODONTIA AND MACRODONTIA hypoplasia and ear malformation, less growth occurs in this
Abnormalities in tooth size are epitomized in microdontia less vascularized area, with smaller teeth forming as a result.
and macrodontia. Hemifacial microsomia is thought to result Peg-shaped lateral incisors, an example of microdontia, is
from a hematoma of the stapedial artery during embryologic common in Down syndrome. Other conditions demonstrat-
development giving rise to a deficient nutrient supply to the ing microdontia include ectodermal dysplasia and chondro-
affected side of the face. In addition to unilateral mandibular ectodermal dysplasia. These size abnormalities are thought to
56 PART 1 Fundamentals of Pediatric Dentistry

■ FIGURE 3-2 Hypodontia in a child with ectodermal dysplasia. Note atrophy of alveolar ridge.

■ TABLE 3-2 FUSION

Syndromes Demonstrating Hypodontia Fusion has an incidence of 0.5% and is more common in the
primary dentition.7 The classic definition of fusion is the
Condition Characteristics
dentinal union of two embryologically developing teeth.
Ectodermal dysplasia Hypotrichosis, aplasia of sweat/ Although fused teeth can contain two separate pulp cham-
(hypohidrotic type) sebaceous glands bers, many appear as large bifid crowns with one chamber,
Chondroectodermal Polydactyly, mesomelic dwarfism, which makes them difficult to distinguish from geminated
dysplasia hidrotic ectodermal dysplasia teeth.
Achondroplasia Short-limbed dwarfism,
macrocephaly, frontal bossing GEMINATION
Rieger syndrome Iris dysplasia, midface hypoplasia, Gemination has a frequency of 0.5%, which is similar to that
protruding umbilicus of fusion and is more common in the primary dentition.
Incontinentia pigmenti Alopecia, pigmented macules, Conceptually, a geminated tooth represents an incomplete
mental retardation division of a single tooth bud resulting in a bifid crown with
Seckel syndrome Dwarfism, microcephaly, facial a single pulp chamber. Gemination tends to occur in a famil-
hypoplasia, low-set lobeless ears ial pattern, and its significance is similar to that of fusion in
that both conditions may result in retarded eruption of the
permanent successor. Clinically, fusion and gemination are
usually distinguished by counting the teeth in the arch. If
there is a deficiency in the normal complement including the
originate during the morphodifferentiation stage of tooth bifid crown, the condition is fusion. Fusion with a supernu-
development. merary tooth must also be considered and ruled out because
Facial hemihypertrophy can demonstrate comparatively this would not affect the normal number of teeth.
larger teeth on the affected side (Figure 3-3). Of the many Concrescence is a twinning anomaly involving the union
factors thought to cause this condition, vascular and neuro- of two teeth by cementum only. Its cause is thought to be
genic abnormalities are considered the most likely. In addi- trauma or adjacent tooth malposition. Because it can occur
tion to an increase in crown and root size, affected teeth after root development, concrescence is technically not a
develop more rapidly and erupt earlier than on the unin- developmental anomaly.
volved side. The otodental syndrome (also known as otodental
dysplasia), consisting of high-frequency hearing loss and
globe-shaped fused molar teeth, is another condition that Anomalies of Shape
involves macrodontia. Isolated teeth with macrodontia can
also result from twinning abnormalities that originate during Abnormalities in shape originate during the morphodiffer-
the proliferation stage of tooth development. Fusion and entiation stage of tooth development and are manifested as
gemination are the most common twinning abnormalities, alterations in crown and root form. Modes of inheritance
and both include enlarged crowns. include both autosomal dominant and polygenic patterns.
 CHAPTER 3 Anomalies of the Developing Dentition 57

A B

C
■ FIGURE 3-3 A and B, Hemifacial hypertrophy. Patient’s affected side (right) is larger in all dimensions. C, Hemifacial hypertrophy. Teeth
on the patient’s affected side (right) are larger in all dimensions.

frequency of 1% to 4% and results from the invagination of

inner enamel epithelium cells, which are the precursors of
ameloblasts.1

■ FIGURE 3-4 Dens invaginatus—talon cusp. All three elements
of dental tissues are represented in the extra cusp.
DENS IN DENTE
Dens in dente is a condition resulting from invagination of
the inner enamel epithelium producing the appearance of a
tooth within a tooth. In 1987, Ruprechet and colleagues
reported a 10% prevalence, with the maxillary lateral inci-
sors being most frequently affected.10 The clinical signifi-
cance of this anomaly results from potential carious
involvement through the communication of the invaginated
portion of the lingual surface of the tooth with the outside
environment. The enamel and dentin in the invaginated
portion can be both defective and absent, allowing direct
communication with the pulp.

DENS EVAGINATUS
Dens evaginatus is an extra cusp, usually in the central
groove or ridge of a posterior tooth and in the cingulum area
of the central and lateral incisors (Figure 3-4). In incisors,
these cusps appear talon-shaped and can approach the level
of the incisal edge. This extra portion contains not only
enamel but dentin and pulp tissue; therefore a pulp exposure
can result from radical equilibration. It occurs with a
TAURODONT
Taurodont teeth are characterized by a significantly elon-
gated pulp chamber with short stunted roots, and apical
displacement of the pulpal floor. Taurodontism results from
the failure of the proper level of horizontal invagination of
Hertwig epithelial root sheath (Figure 3-5). The incidence
can range from 0.5% to 5%, with the permanent molar most
often affected. The condition can be classified according to
the extent of the pulp chamber elongation.11 The conditions
58 PART 1 Fundamentals of Pediatric Dentistry

■ FIGURE 3-5 Taurodont teeth. Note that primary teeth as well as permanent teeth can be affected.

A B
■ FIGURE 3-6 A, Dilacerated lateral incisor. B, Dilacerated lateral incisor.

■ TABLE 3-3 episode, usually to the primary dentition (Figure 3-6). The
overall incidence of dilaceration has been estimated at 3%
Conditions Demonstrating Taurodontism of all permanent dentitions.12 Ectopic eruption and trau-
Condition Characteristics matic injury are the most commonly cited causes of dilacera-
Klinefelter syndrome Aspermatogenesis, mental tions. In 1971, Andreasen reported a dilaceration incidence
retardation of 25% in those permanent teeth with developmental dis-
Trichodento-osseous Sclerotic bones, coarse gnarled turbances secondary to primary tooth injury.13 Congenital
syndrome hair, enamel defects ichthyosis, consisting of hyperkeratosis of the knees and
Oral-facial-digital Dystrophic nails, hyperplastic elbows, fishlike scaly skin, and delayed tooth eruption, also
syndrome type 2 frenum, lobed tongue includes root dilaceration as a consistent finding.
Ectodermal dysplasia Hypotrichosis, aplasia of sweat/
(hypohidrotic) sebaceous glands
Amelogenesis
imperfecta type 4
Enamel hypoplasia and
hypomaturation, mottled yellow
Anomalies of Structure
teeth ENAMEL
Down syndrome Brachycephaly, mental retardation,
Tooth structure abnormalities result from disruption during
epicanthal fold
the histodifferentiation, apposition, and/or mineralization
stages of tooth development. Enamel defects are manifested
as hypoplasia or hypocalcification. According to Jorgenson
and Yost, they may be broadly classified as heritable defects
that classically involve taurodontism are summarized in or environmentally induced defects.14
Table 3-3.
AMELOGENESIS IMPERFECTA
DILACERATION Amelogenesis imperfecta (AI) is a classic example of a
Dilaceration refers to an abnormal bend of the root during heritable enamel defect. Estimates of the incidence of this
its development and is thought to result from a traumatic condition include 1 in 14,000,15 1 in 8000,16 and 1 in 4000.17
 CHAPTER 3 Anomalies of the Developing Dentition 59

■ BOX 3-2

CLASSIFICATION OF
AMELOGENESIS IMPERFECTA

Type 1: Hypoplastic
1A Hypoplastic, pitted autosomal dominant
1B Hypoplastic, local autosomal dominant
1C Hypoplastic, local autosomal recessive
1D Hypoplastic, smooth autosomal dominant
1E Hypoplastic, smooth X-linked dominant
1F Hypoplastic, rough autosomal dominant
1G Enamel agenesis, autosomal recessive

Type 2: Hypomaturation ■ FIGURE 3-7 Amelogenesis imperfecta, hypoplastic type.

2A Hypomaturation, pigmented autosomal recessive
2B Hypomaturation, X-linked recessive
2D Snow-capped teeth, autosomal dominant?

Type 3: Hypocalcified
3A Autosomal dominant
3B Autosomal recessive

Type 4: Hypomaturation, Hypoplastic with Taurodontism

4A Hypomaturation-hypoplastic with taurodontism,
autosomal dominant
4B Hypoplastic-hypomaturation with taurodontism,
autosomal dominant

Fourteen subgroup classifications of AI are listed in Box 3-2,
with multiple inheritance patterns represented. Six genes
have been associated with AI: AMELX, ENAM, MMP20,
KLK4, FAM83H, and WDR72.18 It is important to remember
that the sole feature that distinguishes AI from other enamel
defects is its confinement to distinct patterns of inheritance
and its occurrence exclusive of any syndromic, metabolic, or
systemic condition.19 Anterior open bite has been observed
in 60% of reported cases.13 The open bite is thought to result
from a combination of posterior maxillary hyperplasia, a
high palatal vault, a discrepancy in the transverse dimension
of the maxillary arch, a shortened mandibular ramus, and
excessive anterior facial height.20 The four major AI catego-
ries are described according to the stages of tooth develop-
ment in which each is thought to occur. Additional
information on the subgroups can be obtained from more
comprehensive resources.
HYPOPLASTIC TYPE
Heritable enamel defects occurring in the histodifferentia-
tion stage of tooth development are exemplified by the hypo-
plastic type of AI wherein an insufficient quantity of enamel
is formed (Figure 3-7). This occurs because some areas of
the enamel organ are devoid of inner enamel epithelium,
causing a lack of cell differentiation into ameloblasts. Both
primary and permanent dentitions are affected, and the con-
dition is inherited predominantly as an autosomal dominant
trait depending on the subgroup pattern. Affected teeth
■ FIGURE 3-8 Amelogenesis imperfecta, hypomaturation type.
appear small with open contacts, and areas of the clinical
crowns contain very thin or nonexistent enamel resulting in
high sensitivity to thermal stimuli.
HYPOMATURATION TYPE
The hypomaturation type of AI is an example of an inherited
defect in enamel matrix apposition. It is characterized by
teeth having normal enamel thickness but a low value of
radiodensity and mineral content (Figure 3-8). The problem
is related to the persistence of organic content in the rod
sheath resulting in poor calcification, low mineral content,
and a porous surface that becomes stained.
HYPOPLASTIC/HYPOMATURATION
AMELOGENESIS IMPERFECTA
WITH TAURODONTISM
Hypoplastic/hypomaturation AI with taurodontism is an
example of inherited defects in both apposition and histodif-
ferentiation stages of enamel formation. The enamel appears
mottled with a yellow-brown color and is pitted on the facial
surfaces, exemplifying the features of both hypoplasia and
hypomaturation previously described. Molar teeth demon-
strate taurodontism, and other components of the dentition
have enlarged pulp chambers.
60 PART 1 Fundamentals of Pediatric Dentistry
■ FIGURE 3-9 Amelogenesis imperfecta, hypocalcified type.
Hypocalcification Type The hypocalcification type of AI
is an example of an inherited defect in the calcification stage
of enamel formation. Quantitatively, the enamel is normal,
but qualitatively the matrix is poorly calcified with a resul-
tant fracturing of the enamel surface (Figure 3-9). The hypo-
calcified enamel is soft and fragile, especially at the incisal
regions, and is easily fractured, exposing the underlying
dentin that produces an unaesthetic appearance. Increased
calculus formation and marked delay in tooth eruption are
consistent findings. The clinician needs to be cautious in
discerning calculus from hypocalcified enamel, particularly
in the mandibular anterior teeth.
ENVIRONMENTAL ENAMEL HYPOPLASIA
Examples of environmentally induced enamel hypoplasia
can result from systemic or local causes. Examples of sys-
temic causes producing generalized enamel hypoplasia
include nutrition deficiencies, particularly in vitamins A, C,
and D as well as in calcium and phosphorus.14 Severe infec-
tion, such as exanthematous diseases and fever-producing
disorders, particularly during the first year of life, can directly
affect ameloblastic activity, resulting in enamel hypoplasia.
Rubella embryopathy has a high correlation with prenatal
enamel hypoplasia in the primary dentition. Syphilis caused
by the spirochete Treponema pallidum produces classic pat-
terns of hypoplastic dysmorphic permanent teeth. The
tapered and notched incisal edges of anterior teeth with
screwdriver shapes are called Hutchinson incisors, and the
crenated occlusal patterns of posterior teeth known as mul-
berry molars are classic clinical findings for prenatal syphilis
infection.
Neurologic defects as exemplified in children with cere-
bral palsy and Sturge-Weber syndrome have an increased like-
lihood of progressing into generalized enamel hypoplasia.
Children with asthma also demonstrate a higher prevalence
of enamel hypoplasia than do nonafflicted children. Prema-
turity and excess radiation exposure can disrupt ameloblas-
tic matrix formation or subsequent mineralization and are
■ BOX 3-3
SYNDROMES DEMONSTRATING
ENAMEL HYPOPLASIA
• Down syndrome
• Tuberous sclerosis
• Epidermolysis bullosa
• Hurler syndrome
• Hunter syndrome
• Treacher Collins syndrome
• Phenylketonuria
• Pseudohypoparathyroidism
• Trichodento-osseous syndrome
• Vitamin D–dependent rickets
• Lesch-Nyhan syndrome
• Fanconi syndrome
• Sturge-Weber syndrome
• Turner syndrome
additional causes of systemic enamel hypoplasia. A number
of syndromes involve enamel hypoplasia as a consistent
dental characteristic and are listed in Box 3-3.
Excess ingestion of systemic fluoride can produce gener-
alized enamel defects. Dental fluorosis can be manifested as
a defect in the calcification of the teeth in milder forms, with
significant pigmentation and ameloblastic impairment in
the more severe forms. Fluorosis occurs when the concentra-
tion of ingested fluoride is above 1.8 ppm/day.14 There is a
90% chance of some degree of dental fluorosis when the
amount of ingested fluoride is greater than 6 ppm, although
the severity of morphologic defects cannot be predicted
from specific quantities of ingested fluoride. Recent studies
have suggested that an increase in average daily fluoride
intake of 0.1 mg/day from reconstituted powdered formula
in infants aged 3 to 9 months was associated with an increase
in the risk of developing enamel fluorosis in the permanent
maxillary incisors.21
LOCALIZED ENAMEL HYPOPLASIA
Causes of enamel hypoplasia affecting individual teeth
include local infection, local trauma, iatrogenic surgery as
occurs in cleft palate closure, and primary tooth overreten-
tion. Turner hypoplasia is a classic example of hypoplastic
defect in permanent teeth resulting from local infection or
trauma to the primary precursor (Figure 3-10).
ENAMEL HYPOCALCIFICATION
Hypocalcification defects can be directly related to faults in
the mineralization of the organic matrix in enamel forma-
tion. The same factors that cause enamel hypoplasia also
cause hypocalcification. The majority of localized hypocal-
cific defects, as in the case of Turner hypoplasia, are subse-
quent to localized infection and trauma. Excess exposure to
citric acid resulting from habitual sucking on citrus fruits
 CHAPTER 3
can produce generalized erosive hypocalcified lesions that
mimic the hypocalcification type of AI.
DENTIN
DENTINOGENESIS IMPERFECTA
Dentinogenesis imperfecta is an example of an inheritable
dentinal defect originating during the histodifferentiation
stage of tooth development (Figure 3-11, A and B). This
anomaly involves a defect of predentin matrix that results
in amorphic, disorganized, and atubular circumpulpal
dentin. The mantle dentin is normal, in contrast with the
previously described circumpulpal dentin, which is high in
organic content and contains interglobular calcification. Its
■ FIGURE 3-10 Turner hypoplasia. Note that cementum is
formed on the crown areas that are denuded of enamel.
A B
C D
■ FIGURE 3-11 A-D, Dentinogenesis imperfecta, hereditary opalescent dentin.
Anomalies of the Developing Dentition 61
incidence is about 1 in 8000. Dentinogenesis imperfecta can
be subdivided into three basic types.22
Shields type 1 occurs with osteogenesis imperfecta, an
inherited defect in collagen formation that results in osteo-
porotic brittle bones, bowing of the limbs, bitemporal
bossing, and blue sclera. Primary teeth tend to be more
severely affected than permanent teeth. Periapical radiolu-
cencies, bulbous crowns, obliteration of pulp chambers, and
root fractures are evident (Figure 3-11, C). An amber trans-
lucent tooth color is common.
Shields type 2, also known as hereditary opalescent dentin,
tends to occur as a separate entity from osteogenesis imper-
fecta. In this case, both primary and permanent dentitions
are equally affected, and the characteristics previously
described for type 1 are the same. This condition is inherited
as an autosomal dominant trait.
Shields type 3 is rare and represents many of the features
described earlier, with a predominance of bell-shaped
crowns, especially in the permanent dentition. Unlike types
1 and 2, type 3 involves teeth with a shell-like appearance
and multiple pulp exposures. It has occurred exclusively in
a triracial isolated group in Maryland known as the Brandy-
wine population.22 Type 3 has been proposed to be a differ-
ent expression of the same type 2 gene.7
DENTIN DYSPLASIA
Dentin dysplasia represents another group of inherited
dentin disorders resulting in characteristic features involving
the circumpulpal dentin and root morphology. In 1973,
Shields and associates proposed a classification based on
characteristic patterns of dentinal dysplasia.22
62 PART 1 Fundamentals of Pediatric Dentistry

Shields type 1 demonstrates normal primary and perma-
nent crown morphology with an amber translucency (Figure
3-12). The roots tend to be short and sharply constricted.
Primary teeth have obliterated pulps. Both primary and per-
manent dentitions demonstrate multiple periapical radiolu-
cencies and absent pulp chambers. Cascading tubule patterns
result from blockage of normal dentin tubules by calcified
masses.
Shields type 2 involves amber-colored primary teeth
closely resembling dentinogenesis imperfecta types 1 and 2.
Permanent teeth appear normal, but radiographically they
demonstrate thistle tube–shaped pulp chambers with mul-
tiple pulp stones (Figure 3-13). No periapical radiolucencies
are visible.
roots (Figure 3-14).23 The teeth have a ghostlike radiographic
appearance with shortened roots and shell-like crowns and
are dysmorphic in overall appearance. No conclusive etio-
logic factor or inheritance pattern has been identified that
can explain the reported cases.
Additional conditions involving dentin abnormalities
relate to systemic abnormalities that impair normal absorp-
tion and circulating serum levels of calcium and phospho-
rus. Vitamin D–resistant rickets, hypoparathyroidism, and
pseudohypoparathyroidism are all conditions demonstrating
characteristic dentinal abnormalities that are summarized in
Box 3-4.1
■ BOX 3-4

REGIONAL ODONTODYSPLASIA SYSTEMIC ABNORMALITIES IMPAIRING

NORMAL ABSORPTION AND CIRCULATING
Odontodysplasia is a condition representing a localized SERUM LEVELS OF CALCIUM
arrest in tooth development thought to result from a regional AND PHOSPHORUS
vascular developmental anomaly. Affected teeth have thin
layers of poorly calcified enamel and dentin with large, dif- Vitamin D–Resistant Rickets
fusely calcified pulp chambers and shortened, poorly defined • Hypomineralized dentin
• Increased width to predentin
• Odontoblastic disorganization
• Decreased alkaline phosphatase activity in tooth germ
• Enlarged pulp and pulp horns
• No enamel defect

Hypoparathyroidism
• Tooth defects are more severe in males
• Permanent teeth are predominantly affected
• Short, wedge-shaped roots with delayed apical closure
• Interglobular calcification in dentin, especially at apices
• Enamel hypoplasia

Pseudohypoparathyroidism
• Enlarged pulp chambers
• Irregular dentinal tubules
• Small crowns and short, blunted roots
■ FIGURE 3-12 Dentinal dysplasia type 1. Note rootless primary • Pitted enamel surfaces
teeth.

■ FIGURE 3-13 Dentinal dysplasia type 2. Note thistle-tube shape to permanent pulp chambers.
 CHAPTER 3 Anomalies of the Developing Dentition 63

■ FIGURE 3-14 Odontodysplasia in the patient’s upper left maxillary area.

CEMENTUM
Developmental defects involving cementum as an exclusive
entity apart from other dental structures are uncommon. It
is especially difficult to identify problems in cementogenesis
from diseases involving the periodontal ligament. An inter-
esting finding in Turner hypoplasia is that in addition to
coronal enamel defects of the affected permanent teeth,
cementum is formed in the coronal areas denuded of
enamel.1 This underscores the protective effect that the
reduced enamel epithelium has on the unerupted tooth
crown. Furthermore, it represents the reciprocal inductive
effect of dentin that, when in direct contact with the dental
follicle, directs mesenchymal cell differentiation into cemento-
blasts. Areas denuded of enamel allow this phenomenon to
occur.
Histologically defective cementum occurs in three note-
■ FIGURE 3-15 Hypophosphatasia. Note premature exfoliation
worthy conditions. Epidermolysis bullosa dystrophica, an of primary anterior teeth in the upper and lower anterior areas.
inherited vesicular and bullous disease of the skin and
mucous membranes, involves formation of fibrous, poorly
calcified acellular cementum and overproduction of cellular Anomalies of Color
cementum. Cleidocranial dysplasia also displays histologic
alterations in cementum formation. Lukinmaa and associ- Both the primary and permanent dentitions can manifest
ates noted that permanent teeth were devoid of cellular significant color changes from extrinsic or intrinsic stains.
cementum and had partially hyperplastic acellular Because of their developmental significance, only the int-
cementum.24 rinsic stains are addressed here. In 1975, Eisenberg and
Hypophosphatasia is a complex condition involving the Bernick provided a detailed classification of the causes
failure of bone to mineralize properly, which is associated of tooth discolorations.25 Causes of intrinsic stains can be
with low serum alkaline phosphatase levels. Osteoporosis, due to blood-borne pigments, drug administration, and
bone fragility, and premature loss of primary incisors are hypoplastic-hypocalcified disease states. Congenital por-
classic clinical features (Figure 3-15). The latter finding is phyria, bile duct defects, anemias, and transfusion-reaction
ascribed to the failure of cementum formation on the pre- hemolysis are examples of blood-borne pigments.
maturely exfoliated incisors and to a decrease in cementum A classic example of drug-induced intrinsic staining
formation in the retained primary teeth. The condition occurs from the tetracycline group of antibiotics. Both denti-
exerts its greatest effect prenatally and during the first year tions can have severe discoloration from this antibiotic
of life. Bone and dentin are affected along with cementum, when given in concentrations of 21 to 26 mg/kg or greater
so the entity is not exclusively a cementum defect. over as brief a period as 3 days (Figure 3-16).26 Tetracycline
64 PART 1 Fundamentals of Pediatric Dentistry
■ FIGURE 3-16 Tetracycline staining of primary and permanent
dentitions. Note darker hues to the primary teeth with more diffuse
yellow stain to permanent incisors.
hydrochloride has the greatest potential for staining among
the tetracyclines. The agent forms an orthocalcium phos-
phate complex with dentin and enamel, which is then oxi-
dized by ultraviolet light. The oxidation process results in
pigments that stain the hard tissues. The critical period for
initiation of primary and permanent tooth staining is that
of intrauterine development through 8 years of age. Tetracy-
cline administration must be especially avoided during this
time. The International Academy of Dental Traumatology
(IADT) does recommend systemic doxycycline twice a day
for 7 days for avulsion injuries in which there is a closed
apex. Doxycycline is present in significant levels within gin-
gival crevicular fluid. A caution is clearly stated in dealing
with patients where tetracycline administration may result
discoloration.27
Systemic and localized enamel hypoplasia can result in
tooth discoloration. Many of the enamel and dentin dyspla-
sias also result in tooth color changes similar to those seen
with the heritable anomalies of AI and dentinogenesis
imperfecta. Excess fluoride overlaps both the drug-induced
and hypoplastic categories of agents responsible for tooth
discoloration. The more severe form of dental fluorosis can
produce a range of discoloration, from opaque white spots
with diffuse striations to a brown mottling.
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