Australian Dental Journal

The official journal of the Australian Dental Association

Australian Dental Journal 2013; 58: 133140

doi: 10.1111/adj.12039

Developmental enamel defects in the primary dentition:

aetiology and clinical management
S Salanitri,* WK Seow*
*Centre for Paediatric Dentistry, School of Dentistry, The University of Queensland, Brisbane, Queensland.
LoganBeaudesert Division, Metro South Health District, Queensland Health.

ABSTRACT
Developmental enamel defects, presenting as enamel hypoplasia or opacities are caused by damage or disruption to the
developing enamel organ as a result of inherited and acquired systemic conditions. The high prevalence of these defects
in the primary dentition demonstrates the vulnerability of the teeth to changes in the pre- and postnatal environment.
The presence of enamel hypoplasia increases the risk of primary teeth to early childhood caries and tooth wear as the
defective enamel is thinner, more plaque retentive and less resistant to dissolution in acid compared to normal enamel.
The purpose of this paper was to critically review the aetiology and clinical complications of developmental enamel
defects in the primary dentition and propose recommendations for the clinical management of affected teeth.
Keywords: Enamel hypoplasia, enamel opacities, early childhood caries, primary dentition.
Abbreviations and acronyms: CMV = cytomegalovirus; CPP-ACP = casein phosphopeptide amorphous calcium phosphate; DDE =
developmental defects of enamel; EEC = early childhood caries; HAS-ECC = hypoplasia-associated ECC; MIH = molar-incisor hypo-
plasia; SEM = scanning electron microscopic.
(Accepted for publication 16 January 2013.)

Index, often used in a simplified form, is one of the

INTRODUCTION
Developmental defects of enamel (DDE) are com-
monly encountered in clinical practice, and may be
defined as aberrations in the quality and quantity of
dental enamel which are caused by disruption and/or
damage to the enamel organ.1,2 The presentation and
severity of the defect are usually dependent on the
stage of development during which the insult occurs
as well as the extent and duration of the insult.3
Enamel hypoplasia is a quantitative defect and
presents as a deficiency of enamel while enamel hypo-
mineralization is a qualitative enamel deficiency pre-
senting as alterations in the translucency or opacity of
the enamel which may be diffuse or demarcated and
coloured white, yellow or brown.1,4 Diffuse opacities
are thought to be associated with the group of teeth
which are undergoing enamel maturation at the time
when a systemic insult occurs. In contrast, demarcated
opacities and hypoplasia are more commonly noted in
teeth which had sustained a localized and transient
injury. Although there are many indices which have
been proposed for specific types of DDE, such as the
Dean and Thylstrup and Fejeskov indices of fluoro-
sis,5,6 the Developmental Defects of Enamel (DDE)
2013 Australian Dental Association
most popular.7
Compared to the permanent dentition, the preva-
lence of DDE in the primary dentition has not been
well reported. As noted in Table 1, which shows
investigations of DDE in the primary dentition pub-
lished since 1996, DDE in the primary dentition has
been reported to occur at rates ranging between 10%
to 49%. In the study of Montero and co-workers on
517 children in the USA, an overall prevalence of
49% was reported,8 and in the study of Slayton and
co-workers, 6% and 27% of healthy children in the
USA had at least one tooth with enamel hypoplasia
and enamel opacities respectively, giving a total
prevalence of DDE of 33%. In contrast, Casanova-
Rosado and co-workers found that only 10% of the
Mexican children studied exhibited DDE.9 In Austra-
lia, Seow and co-workers reported a prevalence of
DDE of 25% in the primary dentition with enamel
opacities three times more prevalent than enamel
hypoplasia.10
Although DDE are now increasingly recognized as
important risk factors for dental caries and
tooth wear, the condition is not well diagnosed and
aetiology is still unclear. Furthermore, the clinical
133
S Salanitri and WK Seow

Table 1. Selected studies on the prevalence of associated with the Treacher-Collins syndrome,17 oto-
developmental defects of enamel (DDE) in the dental syndrome,18 22q11 deletion syndrome (also
primary dentition known as velocardiofacial syndrome)19,20 and Heimler
syndrome.21
Authors Year Country of Prevalence
study

Li et al.72 1996 China 22% Acquired conditions

Slayton et al.102 2001 USA 33%
Montero et al.8 2003 USA 49%
Chaves et al.103 2007 Brazil 44%
Hong et al.57 2009 USA 4% (molars only)
Casanova-Rosado 2011 Mexico 10%
et al.9
Seow et al.10 2011 Australia 25%
implications, preventive and restorative management
of DDE are currently not well recognized by many
practitioners. Therefore, the aim of this paper was to
present a critical review on the aetiology, clinical
complications and management of DDE in the pri-
mary dentition.
Aetiology of developmental defects of enamel
There are numerous hereditary, acquired, systemic
and local aetiological factors which are associated
with enamel defects. Since enamel does not remodel,
the defects theoretically present a record of the insults
suffered by the enamel organ during development of
the enamel. However, determining the specific timing
of insults to the developing enamel is often difficult
due to the current lack of knowledge regarding the
chronology of the different stages of amelogenesis as
well as individual variation in rates of enamel forma-
tion.2,11
Hereditary conditions
Enamel defects may be a presenting feature in heredi-
tary conditions that involve only dental enamel or they
may be a component of a generalized systemic syn-
drome. The inherited conditions which involve enamel
only are known as amelogenesis imperfecta and the
defects may present as enamel hypoplasia, hypominer-
alization or hypomaturation. Abnormalities of the
genes involved in amelogenesis are primarily responsi-
ble for these defects.12,13 In children with amelogene-
sis imperfecta, characteristically, the teeth in both
permanent and primary dentitions show DDE.
There are also many hereditary medical syndromes
which may feature enamel hypoplasia. Enamel defects
are often seen in Usher syndrome which is character-
ized by sensorineural hearing loss, retinitis pigmentosa
and enamel hypoplasia,14 as well as in Seckel syn-
drome which features intellectual disability and multi-
ple skeletal defects.15 Ellis Van Creveld syndrome also
show enamel hypoplasia together with defects of the
skeletal and cardiac system.16 DDE have also been
134
Numerous systemic or local acquired conditions that
occur during the antenatal, perinatal or postnatal peri-
ods of development can also cause damage to devel-
oping enamel and result in DDE in the primary
dentition. Prenatal conditions usually result in DDE
which are located in the parts of the enamel formed
before birth, while defects caused by postnatal disrup-
tions are found in the parts formed after birth. A
common area for DDE in the primary dentition is the
parts of enamel which mark the transition from
intra-uterine to extra-uterine life, and separate the
prenatally formed enamel from that which develops
postnatally.22 This region, also known as the neonatal
line, is present on the crowns of all primary teeth
which commence mineralization prenatally.23 The pre-
sentation of the neonatal line varies from a mild
change in direction of the enamel rods to a severe
macroscopic defect extending into the dentine,
depending on the degree of disruption experienced by
the infant during the perinatal and neonatal periods.22
Scanning electron microscopy studies of the neonatal
line have demonstrated that it usually shows a less
well organized enamel prism alignment, and has more
organic material and less mineral content compared
to the surrounding normal enamel.24,25 Thus, systemic
disruptions occurring around the time of birth often
result in exaggerated neonatal lines that are clinically
visible as enamel hypoplasia in the primary denti-
tion.26 Such conditions may be associated with trau-
matic or preterm births, as well as metabolic or
infectious conditions or environmental exposure to
toxic chemicals.
Prenatal conditions which may be associated with
enamel hypoplasia in the child include maternal vita-
min D deficiency during pregnancy and neonatal tet-
any.27 Other antenatal factors which have been
shown to contribute to enamel hypoplasia include
maternal smoking during pregnancy, increased mater-
nal weight gain during pregnancy and failure to access
antenatal care.28,29 Multiple births are also a risk
factor for DDE due to the higher rate of neonatal
complications experienced by these children.30
Although not commonly encountered in developed
countries, nutritional deficiencies in the infant, partic-
ularly those associated with insufficient supply and
absorption of vitamins A, C and D and calcium are
well known to be risk factors for enamel hypoplasia
in preterm31 and Indigenous communities.32 In addi-
tion, suboptimal nutrition stemming from extended
2013 Australian Dental Association

breastfeeding without solid supplementation has also
been suggested as a cause of DDE in primary teeth.28
Children born prematurely and those with low or
very low birth weight have a higher prevalence of
enamel hypoplasia compared to children born at full
term with normal birth weights.22,26,3337 DDE found
in low birth weight and preterm children can be
attributed to many adverse systemic conditions
associated with premature birth, including respiratory
disease, cardiovascular defects, gastrointestinal distur-
bances, haematological problems, immune deficien-
cies, intracranial haemorrhage, anaemia and renal
defects.37 Abnormalities associated with the minerali-
zation pathways such as hypocalcaemia, osteopaenia,
rickets and hyperbilirubinaemia have been directly
implicated in the aetiology of DDE in the primary
dentition.26,33,38 Deficient vitamin D metabolism,
inadequate mineral levels and the inability of the gas-
trointestinal tract to absorb mineral are important
contributors to the defective formation of dental
enamel in preterm children due to immaturity of met-
abolic and mineralization systems.33,37 Furthermore,
the risks of enamel hypoplasia from systemic condi-
tions are often further compounded by local trauma
from laryngoscopy and endotracheal intubation which
are often necessary in preterm children.39
Besides prematurity of birth, coeliac disease is
another condition where malabsorption and mineral
deficiencies resulting from the gut enteropathy caused
by gluten intolerance can cause DDE. Enamel defects
are commonly encountered in coeliac disease to the
extent that they have been proposed as a possible
diagnostic sign of silent coeliac disease in chil-
dren.40,41 Scanning electron microscopic analysis of
hypoplastic teeth from children with coeliac disease
has provided evidence that the enamel is less mineral-
ized as well as more irregular in its organization.42
Renal and liver disease is also often associated with
enamel hypoplasia, probably as a result of the miner-
alization pathways being disrupted in many types of
renal and liver disease in children.4345
Infectious diseases caused by bacteria and viruses
such as infections of the urinary tract, otitis and upper
respiratory disease have been associated with DDE.29
Although uncommon in developed countries, congeni-
tal syphilis acquired from maternal Treponema
pallidum infections can cause enamel hypoplasia or
notching of the incisor teeth,46 and viral infections
such as chicken pox, rubella, measles, mumps, and
influenza have been associated with DDE in both
primary and permanent dentitions,3 while cytomegalo-
virus (CMV) has been reported to cause enamel hypo-
plasia or hypocalcification in approximately one-third
of affected infants.47
Enamel hypoplasia is also commonly present in
children with cerebral palsy caused by maternal or
2013 Australian Dental Association
Developmental enamel defects in the primary dentition
foetal infection, foetal anoxia and hyperbilirubinaemia
which are also risk factors for enamel hypoplasia.48,49
Thus, in many children with cerebral palsy, it is likely
that the DDE commonly observed in the affected chil-
dren are caused by the systemic disturbances which
have damaged both the neurological and the enamel
forming cells.50,51
Many chemicals and drugs have the potential to
damage ameloblasts and cause DDE. Although there
are few reports regarding the primary dentition, levels
of fluoride greater than 1 ppm in the water have cor-
related with higher levels of DDE in the permanent
dentition compared to fluoride levels of less than
1 ppm.52 Children with high lead levels from environ-
mental exposure to lead paint, or accidental or pica
ingestion have been reported to show enamel hypopla-
sia of the pitting variety.3 Tetracyclines have been
well known to cause dental discolourations and
enamel hypoplasia.53 More recently, amoxycillins
have been implicated as a cause of enamel hypoplasia
although it is often difficult to discount the effects of
the fevers and infections which had required the use
of the antibiotics.54
Local factors such as trauma and infections have
also been associated with enamel hypoplasia of the
teeth in the immediate vicinity of the damage, in
contrast to systemic factors which usually affect all
developing teeth in the jaws. For example, trauma to
the developing tooth germ, such as exerted through
the laryngoscope or endotracheal intubation is known
to cause damage to the ameloblasts, and result in
opacities or hypoplasia in preterm children.37 In addi-
tion, demarcated markings which are commonly
found on the labial surfaces of primary canines are
also thought to result from local trauma exerted
through the thin buccal cortical bone overlying the
canines.55
Clinical complications of developmental enamel
defects
As incisor teeth affected by DDE can result in com-
promised aesthetics due to staining and morphological
alterations, children with DDE teeth may experience
feelings of anxiety and social embarrassment
regarding their dental appearance.56 Furthermore, in
many affected children there is increased dental sensi-
tivity due to enamel hypomineralization and exposed
dentine.3,57,58
As shown in Table 2, several studies have reported
a higher prevalence of early childhood caries (ECC) in
children with enamel hypoplasia. The correlation
between DDE and ECC may be underestimated due
to the presence of caries masking the underlying undi-
agnosed enamel defects.59 In previous investigations
which aimed to study the association of DDE and
135
S Salanitri and WK Seow

Table 2. Selected studies which reported on the association of developmental defects of enamel with early child-
hood caries
Authors Year Country N Age range of ECC Prevalence (%)
subjects (yrs)
Enamel hypoplasia Enamel hypoplasia
(EH) present (EH) absent

Matee et al.67 1994 Tanzania 2192 14 29 4.6

Montero8 2003 USA 517 2.74.9 50 26
Chaves et al.103 2007 Brazil 228 03 16.9 0.9
Hong et al.57 2009 USA 491 59 52.6 34.5
Elfrink et al.104 2010 Netherlands 242 5 14 5
Targino et al.105 2011 Brazil 274 14.5 48.4 26.2
Zhou et al.106 2011 China 394 02 34.3 27
Seow et al.10 2009 Australia 617 04 16.9%55.3% of children 7.4% of ECC free
with ECC had EH children had EH
Nelson et al.107 2010 USA 246 14 6.9% increase in caries
when EH present
Zhou et al.108 2012 China 155 832 months EH 15 times more likely
to experience caries
60
Schroth et al. 2005 Canada 98 35 High levels of EH associated
with high levels of ECC
Law et al.65 2006 Australia 71 1.86 Significant association of
EH and caries

ECC, authors have reported that examining children
at the age of four years may be too late as primary
teeth with defective enamel are likely to be cavitated,
carious, restored or extracted by that age.60
Observations of high levels of enamel hypoplasia
and ECC presenting simultaneously in Australian
Indigenous communities in both remote61 and urban
areas62 have led Seow to propose that enamel hypopla-
sia is one of the main caries risk factors in children
from disadvantaged backgrounds.63 Further evidence
for the caries risk posed by enamel defects is found in
case-control studies of children with ECC and preterm
children.6466 Schroth et al. reported that the high
prevalence rate of enamel hypoplasia of approximately
50% was the key risk factor for early childhood caries
in the native communities in the Canadian province of
Manitoba.60 These results supported the study of Mat-
tee and co-workers which found that Tanzanian
infants with enamel hypoplasia experienced more
severe ECC in the absence of other risk factors such as
inappropriate bottle feeding, use of sweetened pacifiers
and cariogenic diets, suggesting that enamel defects is
a significant risk factor for ECC.67 Mounting evidence
for the increased caries risk from enamel hypoplasia in
young children has led Caufield and co-workers to
recently propose a new name, Severe, hypoplasia-
associated ECC or HAS-ECC for a type of severe
ECC which is strongly associated with enamel
hypoplasia.68 The children who are most likely to have
HAS-ECC are therefore those who are well-known
to be at high risk for enamel hypoplasia, such as Indig-
enous and preterm children.69,70
The increased susceptibility of DDE affected teeth
to caries is most likely due to the enamel defects
presenting retentive and irregular sites for plaque and
136
cariogenic bacteria.71,72 Even on relatively intact,
non-carious surfaces, penetration of bacteria into por-
ous enamel to sites close to the dentino-enamel junc-
tion has been documented from SEM studies.73 The
significance of this observation is that it is likely that
cariogenic bacteria can colonize the porous subclinical
DDE lesions with relative ease to initiate caries.22 Pla-
que removal is thus difficult at the DDE sites, and
toothbrushing in these areas is often made more diffi-
cult due to increased sensitivity of the teeth. Further-
more, as the defective enamel is less resistant to acid
dissolution, demineralization from bacterial acids will
progress more rapidly compared to normal enamel.
In addition to caries, the thinner and weaker hypo-
plastic or hypomineralized enamel predisposes the
DDE teeth to tooth wear. Kazoullis and co-workers
reported a strong association of dental erosion with
enamel hypoplasia58 and Seow and Taji proposed that
this is likely to be due to easy dissolution of hypomin-
eralized teeth to ingested acids.74
Management of primary teeth affected by
developmental defects of enamel
Management of enamel hypoplasia in the primary
dentition should focus on early diagnosis and preven-
tive care. Screening and early detection of DDE and
caries is one of the benefits of having a childs first
oral examination by 12 months of age.75 As few par-
ents and other health professions are aware of this
recommendation, community education is required.76
It should also be recognized that young children with
extensive involvement of DDE such as amelogenesis
imperfecta usually require referral to a specialist pae-
diatric dentist and full mouth rehabilitation under
2013 Australian Dental Association

general anaesthesia. Also, as enamel formation of the
permanent molars and incisors occur at the same time
as the primary molars, the presence of DDE in the
primary molars should also indicate a risk for molar-
incisor hypoplasia (MIH) in the permanent denti-
tion.77 If the entire primary dentition is affected, the
possibility of a genetic cause should be considered.
Thus, children with DDE in primary molars should be
followed up at regular recalls to monitor for presence
of DDE when the permanent teeth erupt (between the
ages of 6 to 12 years). If the permanent teeth show
DDE, a preventive programme to manage tooth sensi-
tivity, caries and toothwear should be instituted as
soon as possible after diagnosis. In cases of extensive
enamel hypoplasia in the permanent dentition,
interdisciplinary team management involving general
practitioners, specialist paediatric dentists and orth-
odontists may be necessary.
As DDE predisposes the teeth to increased caries
risk and toothwear, parents need to be informed that
teeth with enamel defects are highly susceptible to
decay and erosion from acids in foods and drinks.58
Preventive advice given to parents should include
replacing cariogenic snacks with healthy foods, twice
daily toothbrushing, and topical fluoride application.
To reduce sensitivity from toothbrushing, a very soft
toothbrush and lukewarm water for mouthrinsing
may be suggested.
Topical fluoride is one of the most effective anti-
caries agent for teeth affected by DDE, and may be
given as neutral sodium fluoride gels or fluoride var-
nishes applied professionally 3 or 6 monthly or used
as daily or weekly rinses in children who are able to
expectorate after rinsing.78,79 According to current
ADA guidelines,80 for children younger than six years
of age, mouthrinses are not recommended due to risks
of swallowing the rinses. For toddlers, 36 monthly
applications of fluoride varnish on teeth affected by
DDE will help to decrease the risk for ECC as demon-
strated in randomized controlled trials.81
In addition, treatment with other remineralizing
agents such as casein phosphopeptide amorphous cal-
cium phosphate (CPP-ACP) can provide a reservoir of
calcium and phosphate which will help remineralize the
hypomineralized areas and early carious lesions on the
tooth surface.8284 In addition, as CPP-ACP has been
shown to have the ability to inhibit the adherence of
cariogenic bacteria mutans streptococci on enamel
surfaces,85 there are possible benefits of CPP-ACP appli-
cation for reducing cariogenic bacterial colonization in
children. Preliminary evidence for this can be found in a
recent study which demonstrated that there are fewer
children colonized with mutans streptocci in the group
who applied a 10% CPP-ACP gel daily compared to the
group who used a 0.12% chlorhexidine paste daily or
control children who used only toothpaste.86
2013 Australian Dental Association
Developmental enamel defects in the primary dentition
As with permanent teeth, in hypoplastic primary
molars, non-chemically bonded restorative materials
such as amalgams usually have suboptimal perfor-
mance due to breakdown of the structurally weak
enamel that often results in marginal leakage, recur-
rent caries and pulp involvement.8789 In permanent
teeth with DDE, marginal breakdown has been
known to occur frequently when cavity margins are
positioned close to the affected enamel, probably due
to an inherent weakness in the enamel prism sheaths
and loss of enamel strength.90 Therefore, restorations
that can bond to dentine as well as enamel such as
resin modified glass-ionomer cements and polyacid
modified composite resins are likely to be suitable for
sealing and restoring small lesions in primary teeth
with DDE.9193
Stainless steel crowns are the restorations of choice
for both primary and permanent molar teeth affected
by enamel hypoplasia, as they offer the highest dura-
bility and best protection against further break-
down.94 For many hypoplastic primary teeth, the
stainless steel crowns are best inserted using a conser-
vative technique with minimal removal of tooth struc-
ture and employing the use of separators to create
interproximal spacing.9597 This technique, first advo-
cated by Seow for protecting teeth with large pulps
associated with developmental abnormalities,95 has
been applied to place stainless steel crowns on hypo-
plastic primary and permanent molars. This method
reduces the sensitivity that is frequently encountered
in hypoplastic teeth, and helps preserve tooth struc-
ture and maintain space and crown height. The con-
servative method of crown placement is also applied
in the Hall technique for restoration of carious teeth
where stainless steel crowns are placed on decayed
primary teeth with minimal or no caries removal or
tooth preparation.98,99 Although the Hall technique
has been advocated by some authors,100 its clinical
efficacy for restoration of primary molars in toddler-
age children with ECC will need to be established.101
The authors recommendation is that the Hall tech-
nique may be attempted for stainless steel crown res-
toration of hypoplastic primary molars in children
where sedation or general anaesthesia is not available.
CONCLUSIONS
DDE in the primary dentition may occur as a result
of a wide range of hereditary and acquired aetiologi-
cal factors, and may result in dental hypersensitivity
and compromised aesthetics. As the complications
of enamel hypoplasia include an increased risk for
caries and tooth wear, protection of the enamel and
effective preventive care and monitoring are required.
Therefore, management of enamel defects in the pri-
mary dentition includes early detection and preventive
137
S Salanitri and WK Seow
management such as sealants and remineralizing
agents and durable restorations such as stainless steel
crowns for the molars and composite resins for the
anterior teeth.
ACKNOWLEDGEMENTS
This project was supported by the Dental Board of
Queensland.
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Address for correspondence:
Professor W Kim Seow
Director
Centre for Paediatric Dentistry
School of Dentistry
200 Turbot Street
Brisbane QLD 4000
Email: k.seow@uq.edu.au
2013 Australian Dental Association