International Journal of

Environmental Research
and Public Health

Case Report
Management of Amelogenesis Imperfecta in Childhood:
Two Case Reports
Mirja Möhn 1, *, Julia Camilla Bulski 1 , Norbert Krämer 1 , Alexander Rahman 2 and Nelly Schulz-Weidner 1

1 Dental Clinic, Department of Pediatric Dentistry, Justus Liebig University, Schlangenzahl 14,
35392 Giessen, Germany; julia.c.bulski@dentist.med.uni-giessen.de (J.C.B.);
norbert.kraemer@dentist.med.uni-giessen.de (N.K.);
nelly.schulz-weidner@dentist.med.uni-giessen.de (N.S.-W.)
2 Department of Conservative Dentistry, Periodontology and Preventive Dentistry, Hannover Medical School,
Carl-Neuberg-Strasse 1, 30625 Hannover, Germany; rahman.alexander@mh-hannover.de
* Correspondence: mirja.moehn@dentist.med.uni-giessen.de; Tel.: +49-641-9946241

Abstract: Amelogenesis imperfecta (AI) is defined as an interruption of enamel formation due to






Citation: Möhn, M.; Bulski, J.C.;
Krämer, N.; Rahman, A.;
Schulz-Weidner, N. Management of
Amelogenesis Imperfecta in
Childhood: Two Case Reports. Int. J.
Environ. Res. Public Health 2021, 18,
7204. https://doi.org/10.3390/
ijerph18137204
genetic inheritance. To prevent malfunction of the masticatory system and an unaesthetic appearance,
various treatment options are described. While restoration with a compomer in the anterior region
and stainless steel crowns in the posterior region is recommended for deciduous dentition, the
challenges when treating such structural defects in mixed or permanent dentition are changing
teeth and growing jaw, allowing only temporary restoration. The purpose of this case report is to
demonstrate oral rehabilitation from mixed to permanent dentition. The dentition of a 7-year-old
patient with AI type I and a 12-year-old patient with AI type II was restored under general anesthesia
to improve their poor aesthetics and increase vertical dimension, which are related to problems with
self-confidence and reduced oral health quality of life. These two cases show the complexity of dental
care for structural anomalies of genetic origin and the challenges in rehabilitating the different phases
of dentition.
Keywords: amelogenesis imperfecta; pediatric dentistry; dental care; therapy concept

Academic Editors: Lauren Bohner, 1. Introduction

Marcel Hanisch and Fawad Javed Amelogenesis imperfecta (AI) is described as generalized defects in enamel formation
in primary and permanent dentition because of a genetic disorder. The inherited malfor-
Received: 22 May 2021
mation of teeth can be x-linked, autosomal dominant, autosomal recessive, or sporadic. In
Accepted: 29 June 2021
particular, mutation or altered expression of the enamelin (ENAM), amelogenin (AMEL),
Published: 5 July 2021
matrixmetalloproteinaise-20 (MMP20), kallikrein-4 (KLK4), and FAM83H genes is associ-
ated with the malfunction of enamel-forming proteins [1]. An association with a general or
Publisher’s Note: MDPI stays neutral
systematic disorder has not been reported.
with regard to jurisdictional claims in
The clinical manifestation includes four types of AI [2]. The most common phenotype
published maps and institutional affil-
is type I, characterized by a hypoplastic structure with a decreased quantity of enamel. The
iations.
teeth show reduced enamel thickness, rough surface, and various extensions of defects
(Figure 1) [3]. Type II, called hypomaturation, shows mottled and softer enamel due to
defective protein maturation within the enamel matrix. Additionally, chipping of the
enamel from the dentin can be found (Figure 2) [1]. In AI type II, the enamel thickness is
Copyright: © 2021 by the authors.
normal. The secretion phase of the ameloblasts proceeds as usual, but in the maturation
Licensee MDPI, Basel, Switzerland.
phase, normal reabsorption of the secreted enamel matrix proteins does not take place.
This article is an open access article
Subsequently, a very high proportion of organic matter remains in the enamel [4].
distributed under the terms and
Type III (hypocalcification) is associated with defects in calcification and appears in
conditions of the Creative Commons
Attribution (CC BY) license (https://
enamel with normal thickness at the time of eruption. Because of the poor mineralization,
creativecommons.org/licenses/by/
the enamel rapidly wears down and X-rays show less opacity. Type IV manifests as a mixed
4.0/). appearance of hypoplasticity–hypomaturation combined with taurodontism [1,5].

Int. J. Environ. Res. Public Health 2021, 18, 7204. https://doi.org/10.3390/ijerph18137204 https://www.mdpi.com/journal/ijerph
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Int. J. Environ. Res. Public Health 2021, 18, x FOR PEER REVIEW 2 of 10

Int. J. Environ. Res. Public Health 2021, 18, 7204 2 of 9

the enamel rapidly wears down and X-rays show less opacity. Type IV manifests as a
the enamel rapidly wears down and X-rays show less opacity. Type IV manifests as a
mixed appearance of hypoplasticity–hypomaturation combined with taurodontism [1,5].
mixed appearance of hypoplasticity–hypomaturation combined with taurodontism [1,5].

(a) (b)
(a) (b)
Figure 1. A 55 1½-year-old
1. A patientwith
withAIAItype
typeI:I:(a)
(a)upper
upperteeth;
teeth;(b)
(b)lower
lowerteeth.
teeth. Clinical
Clinical exanimation
exanimation revealed
revealed pain
pain and
and
Figure
Figure 2 -year-old patient
1. A 5 ½-year-old patient withloss
AI of
type I: (a)structure
upper teeth; (b) lower teeth. Clinical exanimation revealed pain and
hypersensitivity in yellow teeth and dental (pits).
hypersensitivity in yellow teeth and loss of dental structure (pits).
hypersensitivity in yellow teeth and loss of dental structure (pits).

(a) (b)
(a) (b)
Figure 2. A 14-year-old patient with hypomaturation of AI: (a) upper teeth; (b) lower teeth. Clinical exanimation revealed
Figure
yellow 2. A 14-year-old
teeth patient
affecting oral with
health hypomaturation
related of AI: (a) upper teeth; (b) lower teeth. Clinical exanimation revealed
quality of life.
yellow teeth affecting oral health related quality of life.
yellow teeth affecting oral health related quality of life.
Due to the structure of enamel hypersensitivity, plaque accumulation and poor
Due
Due to thestructure
the structure ofenamelenamel hypersensitivity, plaque accumulation and poor
aesthetics are reported [6].ofTo prevent hypersensitivity, plaque
dental caries, gingival accumulation
inflammation,and open poor
bite,aes-
or
aesthetics
thetics are are reported
reported [6].ToToprevent
[6]. preventdentaldentalcaries,
caries,gingival
gingivalinflammation,
inflammation, open open bite, or
loss of vertical dimension, interdisciplinary patient care is recommended. In particular,
loss
loss of verticalordimension,
conservative prostheticinterdisciplinary
dimension, and orthodonticpatient
interdisciplinary patient care is recommended.
care are
treatment recommended. In
In particular,
crucial for successful particular,
oral
conservative or
conservative or prosthetic
prostheticand andorthodontic
orthodontic treatment
treatment areare
crucial for successful
crucial for successfuloral reha-
oral
rehabilitation. Various treatment options have been described depending on the patient’s
bilitation. Various
rehabilitation. treatment
Various treatmentoptions havehave
options beenbeen
described depending
described depending on the
on patient’s
the patient’sage
age and socioeconomic conditions and the severity of malformation [7]. While stainless
and and
age socioeconomic
socioeconomic conditions
conditionsand andthe severity of malformation
the severity of malformation [7]. While stainless
[7]. While steel
stainless
steel crowns, strip crowns, and compomer restorations are common in primary dentition,
crowns,
steel strip strip
crowns, crowns, and compomer
crowns, and compomer restorations are common
restorations are common in primary dentition,
in primary the
dentition,
the challenge in mixed and permanent dentition in adolescents is care of the dentition
challenge in mixed and permanent dentition in adolescents is care
the challenge in mixed and permanent dentition in adolescents is care of the dentition of the dentition during
during growth [8]. Whereas ceramic crowns and veneers are preferred for adults,
growth growth
during [8]. Whereas ceramic crowns
[8]. Whereas ceramic and veneers
crowns areveneers
preferred for adults, CAD/CAM
CAD/CAM composites offer an opportunity forand
high-quality are preferred
restorations in for adults,
adolescent
composites offer an opportunity for high-quality restorations
CAD/CAM composites offer an opportunity for high-quality restorations in in adolescent children. The
adolescent
children. The advantages of this approach are less chair time and the possibility of
advantages
children. Theof advantages
this approach ofare
thisless chair time
approach areand
lessthechair
possibility
time and of intraoral repairs in
the possibility of
intraoral repairs in cases of material fractures. Besides the oral complications due to the
cases of material
intraoral repairs infractures. Besides the
cases of material oral complications
fractures. Besides the oralduecomplications
to the genetic due defects of
to the
genetic defects of the enamel, poor aesthetics can also be associated with problems with
the enamel,
genetic defectspoorofaesthetics
the enamel, canpoor
also aesthetics
be associated canwith
also problems
be associated withwith
self-confidence
problems with and
self-confidence
reduced oral and reducedquality
health-related oral health-related
of life [9,10]. quality of life [9,10].
self-confidence
The aim
aim of ofand
thisreduced
paper was oral
was health-related
to report quality of life [9,10].
report the management
management of AI
AI patients from from mixed
The
The aim of this
this paper
paper was to to report thethe management of of AI patients
patients from mixedmixed
dentition
dentition in in childhood
in childhood
childhood to to permanent
to permanent
permanent dentitiondentition
dentition in in early
in early adulthood
early adulthood
adulthood by by presenting
by presenting two
dentition presenting two two
patients, aged
patients, aged 7
aged 77 and and 12 years. For this purpose, the differences between direct filling
patients, and 1212 years. For this
years. For this purpose,
purpose, thethe differences
differences between
between direct
direct filling
filling
therapies
therapies combined
combined with
with prefabricated
prefabricated crowns
crowns in
in mixed
mixed dentition
dentition and
and indirect
indirect restoration
restoration
therapies combined with prefabricated crowns in mixed dentition and indirect restoration
in the permanent dentition were compared, and the patients were followed up at 3 and
6 months.
 inthe
in thepermanent
permanentdentition
dentitionwere
werecompared,
compared,and
andthe
thepatients
patientswere
werefollowed
followedup
upatat33and
and66
months.
months.
Int. J. Environ. Res. Public Health 2021, 18, 7204 3 of 9
2.2.Case
CaseReports
Reports
2.1.Case
2.1. CaseReport
Report1:1:Mixed
MixedDentition
Dentition
AA7-year-old
2. Case 7-year-old
Reports girl girlwas
wasreferred
referredto toour
ourpediatric
pediatricpolyclinic
polyclinicdue dueto toaesthetic
aestheticproblems
problems
and
and sensitive
sensitive
2.1. Case teeth
teeth
Report with pain.
with pain.
1: Mixed The mother felt extremely affected in her
The mother felt extremely affected in her social life by
Dentition social life byher
her
daughter’sstructural
daughter’s structural problem,as as thechild
childwas
wasbeing
beingteased
teased byotherotherchildren
childrenatatschool
school
A 7-year-old girlproblem,was referredthe to our pediatric polyclinicby due to aesthetic problems
due to
due to herher teeth.
teeth. TheThe family
family history
history showed
showed no no abnormalities;
abnormalities; neither
neither parent
parent had had
and sensitive teeth with pain. The mother felt extremely affected in her social life by her
phenotypic dental
phenotypic dental structure
structure anomalies.
anomalies. Clinical
Clinical examination
examination revealed
revealed AI AI type
type II
daughter’s structural problem, as the child was being teased by other children at school due
hypomineralized teeth.
hypomineralized teeth. Oral
Oral examination
examination presented
presented easily
easily chipping
chipping enamel
enamel combined
combined
to her teeth. The family history showed no abnormalities; neither parent had phenotypic
with
with reduced
reduced enamel
enamel thickness,
thickness, rough surface,
roughexamination and various
surface, and revealed extensions
various extensions of structural loss.
dental structure anomalies. Clinical AI type I of structural loss.
hypomineralized
Defects
Defects
teeth. in
Oralinenamel
enamel
examinationmatrix
matrix formation
formation
presented showed
showed
easily pitted
pitted
chipping andgrooved
and
enamel grooved
combined enamel,
enamel, especially
with especially
reduced ininthe
enamel the
maxillary
maxillary front
front and
and upper
upper and
and lower
lower first
first permanent
permanent molars.
molars. InIn addition,
addition,
thickness, rough surface, and various extensions of structural loss. Defects in enamel matrix the
the maxillary
maxillary
and mandibular
and mandibular
formation showedfrontsfronts and
pitted were
were clearlyenamel,
clearly
grooved spacedespecially
spaced apart. The
apart. The patient’s
patient’s
in the maxillary oral
oral hygiene
hygiene
front and upper was
was
inadequate
inadequate
and lower first due to
duepermanent hypersensitivity
to hypersensitivity and the
and thethe
molars. In addition, tooth
tooth surface
surface
maxillary structure.
andstructure.
mandibular A panoramic
A fronts
panoramicwere
radiograph
radiograph
clearly spaced revealed
revealed loss
apart.loss
Theofof enamel(Figure
enamel
patient’s (Figure
oral 3).Tooth
3).
hygiene Tooth wearcould
waswear couldbe
inadequate be
due detected
detected (Figure4).
(Figure
to hypersensitivity 4).AA
caries
caries
and the lesion
lesion
toothinin region
region
surface 6464could
couldA
structure. bepanoramic
be diagnosedradiograph
diagnosed (Figure5a–d).
(Figure 5a–d).
revealed loss of enamel (Figure 3).
Due
Due to
to the
the extensive
extensive scope
scope of
of care,
care, the
the patient
patient
Tooth wear could be detected (Figure 4). A caries lesion in region underwent
underwent comprehensive
comprehensive rehabilita-
rehabilita-
64 could be diagnosed
tion,
tion, which
which
(Figure 5a–d). was
was performed
performed under
under general
general anesthesia
anesthesia because
because of
of her
her age
age and
and anxiety.
anxiety. Ad-
Ad-
hesive build-ups and stainless steel crowns stabilized the
hesive build-ups and stainless steel crowns stabilized the vertical dimension. vertical dimension.

Figure3.3.
Figure
Figure PanoramicX-ray
3.Panoramic
Panoramic X-rayofof
X-ray of7-year-old
7-year-oldpatient.
7-year-old patient.
patient.

(a)
(a) (b)
(b)
Figure 4. Plaster models of upper and lower jaws showing loss of tooth wear because of attrition and abrasion (physical
tooth wear): (a) right side; (b) left side.
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Int. J. Environ. Res. Public Health 2021, 18, 7204 4 of 9

Figure 4. Plaster models of upper and lower jaws showing loss of tooth wear because of attrition and abrasion (physical
tooth wear): (a) right side; (b) left side.

a b c

d e f
g h

i j

Figure
Figure 5.5.AA7-year-old
7-year-oldpatient
patientwith
withAIAItype I. (a–f)
type Preoperative
I. (a–f) Preoperativesituation with
situation multiple
with substance
multiple defects
substance on all
defects onteeth; (c)
all teeth;
after fluoride varnish application. (g–j) Postoperative result. Oral surgery was performed under general anesthesia, with
(c) after fluoride varnish application. (g–j) Postoperative result. Oral surgery was performed under general anesthesia, with
stainless steel crowns applied to second primary molars and adhesive filling materials in first primary molars, first molars
stainless steel crowns applied to second primary molars and adhesive filling materials in first primary molars, first molars
and anterior teeth.
and anterior teeth.

Stainless
Due to the steel crownsscope
extensive (3M™of , Neuss,
care, the Germany) were applied
patient underwent to second primary
comprehensive rehabili-
molars; after tangential preparation, adaptation, and control
tation, which was performed under general anesthesia because of her age of the “snap effect”, metal
and anxiety.
crowns were cemented with glass ionomer cement (Ketac ™ Cem Aplicap™, 3M™, Neuss,
Adhesive build-ups and stainless steel crowns stabilized the vertical dimension.
Germany). First
Stainless primary
steel crownsmolars
(3M™ ,were
Neuss, reconstructed
Germany) were using an all-in-one
applied to secondadhesive
primarysystem
molars;
(Scotchbond ™ Universal, 3M™, Neuss, Germany) and compomer (Dyract®, Dentsply
after tangential preparation, adaptation, and control of the “snap effect”, metal crowns were
Sirona,
cemented Bensheim,
with glassGermany),
ionomerexcept
cement tooth 64,™which
(Ketac Cem was severely
Aplicap ™ , 3M destroyed
™ , Neuss,and had to
Germany).
be extracted.
First primary Permanent
molars weremolars and anterior
reconstructed using teeth were covered
an all-in-one adhesive with direct
system composite™
(Scotchbond
filling material
Universal, 3M™(Figure
, Neuss,5e–h). Without
Germany) and removing
compomermalformed enamel, etching
(Dyract® , Dentsply with 34%
Sirona, Bensheim,
phosphoric acid (Scotchbond ™ Etchant, 3M™, Neuss, Germany) for 60 s was carried out,
Germany), except tooth 64, which was severely destroyed and had to be extracted. Per-
followed by rinsing
manent molars and and drying.
anterior teethAfter
werebonding
covered(Scotchbond Universal,filling
with direct ™composite 3M™,material
Neuss,
Germany),
(Figure 5e–h).a flow composite
Without (Venus
removing ® Diamondenamel,
malformed Flow A2, Kulzer,
etching Hanau,
with Germany) was
34% phosphoric acid
directly
(Scotchbond ™ Etchant,
applied, 3M™composite
and then, (Venus® Diamond
, Neuss, Germany) A2,carried
for 60 s was Kulzer, Hanau,
out, Germany)
followed by rins-
was drying. After bonding (Scotchbond™ Universal, 3M™ , Neuss, Germany), a flow
applied.
ing and
composite (Venus® Diamond Flow A2, Kulzer, Hanau, Germany) was directly applied, and
then, composite (Venus® Diamond A2, Kulzer, Hanau, Germany) was applied.
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After rehabilitation, the patient was reevaluated after 3 months. Follow-up 6 months
After
later
later rehabilitation,
showed the patient
a satisfactory was reevaluated
aesthetic afterresult.
and functional
functional 3 months.
result. Follow-up
Since
Since 6 months
there was
there no space
later showed in
narrowing a region
satisfactory
74, weaesthetic and to
did not need functional
fabricate aresult.
space Since there (Figure
maintainer was no space
6).
(Figure 6).
narrowing in region 74, we did not need to fabricate a space maintainer (Figure 6).

a
a

b
b

b c
b c
Figure 6. Follow-up 6 months after restoration under general anesthesia: (a) upper teeth; (b) lower teeth; (c) front teeth.
Figure
Figure 6. Follow-up
6. Follow-up 6 months afterrestoration
restorationunder
undergeneral
generalanesthesia:
anesthesia: (a)
(a) upper
upper teeth;
teeth; (b)
(b) lower
lowerteeth;
teeth;(c)
(c)front
frontteeth.
teeth.All
All restorations in 6situ,
months after
no abnormalities.
restorations in situ, no abnormalities.
All restorations in situ, no abnormalities.
2.2. Case Report: Permanent Dentition
2.2.2.2.
CaseCase Report:
Report: Permanent
Permanent Dentition
Dentition
A 12-year-old patient presented to our polyclinic because of the unattractive aesthet-
Ahis
12-year-old
icsAof12-year-old
teeth due patient
patient presented
to AIpresented
type II. Intoto ourpolyclinic
our
addition polyclinic because
because
to the change ofthe
inof theunattractive
color unattractive
of aesthetics
aesthet-
all permanent teeth,
ofofhis
icsthe his teeth
teeth due
due to
toAI
AI type
typeII.
II.In
In addition
addition to
to the
the change
change in
in color
colorof all
of all
loss of vertical dimension and the gaps in the dentition were impressive (Figure permanent
permanent teeth,
teeth,the
7).
loss
theThe ofofvertical
loss12-year
verticaldimension
dimension and the thegaps
gapsininthe dentition were impressive (Figure 7).7).The
molars had not andyet (completely) the dentition
erupted into were impressive
the oral cavity. In(Figure
addition to
The12-year molars had notnot
yetyet
(completely) erupted intointo
the oral cavity. In addition to AI,tothe
AI,12-year molars
the patient had
presented with(completely)
cardiac disease erupted the oral
and immunodeficiency. cavity.AsInheaddition
was a foster
patient
AI,child, presented
the patient with
presented cardiac diseasedisease and immunodeficiency. As he was achild,
and immunodeficiency. As he was a foster fosterhis
his family historywith
wascardiac
not available.
family history was not available.
child, his family history was not available.

(a) (b)
(a) (b)
Figure 7. Cont.
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(c)(c)
Figure
Figure
Figure7.7.
7. A AA 12-year-old
12-year-old
12-year-oldpatient
patient showing
showing
patient clinical
clinical
showing signs
signs
clinical ofof
of AI
signs AI type
type
AI II: II:
type (a,b);
(a,b);
II: upper
upper
(a,b); teeth
teeth
upper (c) (c)
teeth front
front
(c) teeth.
teeth.
front teeth.
Besides
Besides the yellow
the yellow
Besides color,
color,
the yellow gaps
gapsgaps
color, between
between teeth
teethteeth
between are predominant.
are predominant.
are predominant.

DueDue to to
Due tothe
the the strong
strong need
strong needforfor
need aesthetic
aesthetic
for aesthetic improvement
improvement
improvement and and thethe
and associated
associated
the general
general
associated disease,
disease,
general disease,
it it
wasitwas
was decided
decided
decided to to toperform
perform
perform all-composite
all-composite
all-composite rehabilitation.
rehabilitation.
rehabilitation. In In Inthis
this direct
direct
this approach,
approach,
direct approach, resin
resinresin
composite
composite
composite restoration
restoration
restoration waswas
was used
used forfor
used forthe
the thetransitional
transitional
transitional treatment
treatment
treatment of of ofhypomature
hypomature
hypomature AI.AI.
AI.
First,
First, anan
First, animpression
impression
impression of of ofthe
the the maxilla
maxillamaxillaand and
and mandible
mandible
mandible with
with with A-silicone
A-silicone
A-silicone material
material
material (Panasil ® ®®
(Panasil
(Panasil
Putty
Putty Putty Fast,
Fast, Fast, Kettenbach,
Kettenbach,
Kettenbach, Germany)
Germany)
Germany) was was
was taken
taken taken through
through
through advanced
advanced
advanced chairside.
chairside.
chairside. Due Due to to
Due tothe
the the
sufficient
sufficient
sufficient space
spacespace of
of the the
of the upper
upperupper and
andand lower
lower lower jaws,
jaws, jaws, there
there there was
waswas no
no no
need need
need for
for for preparation.
preparation.
preparation. Full
Full Full
composite
composite
composite crowns
crownscrowns werewere
were built
built upup
built upon
on onplaster
plasterplaster models
models models after
after after articulation
articulation
articulation according
according
according to totoaverage
average
average
values
values byby
values bythe
the thedental
dental dental technician.
technician.
technician. AAjaw
A jaw jaw relation
relation
relation determination
determination
determination could
could notnot
could bebe
not beperformed
performed
performed duedue
due
to tothe
thepatient’s
patient’s insufficient
insufficient compliance.
compliance. Due
Due totothis
thisparticular
particular
to the patient’s insufficient compliance. Due to this particular feature, it should be pointed feature,
feature, it should
it should bebepointed
pointed
out,
out, in in
out, inthis
this thiscase,
case, case,
thatthat
that adaptation
adaptation
adaptation to to
thetothe
the
new newnew mandibular
mandibular
mandibular posture
posture
posture would
would bebe
would berequired
required
required after
afterafter
insertion.
insertion.
insertion. The The
Theparentsparents
parents were were
were informed
informed
informed about
about
about this this
this
in in in detail.
detail.
detail.
UnderUnder
Under general
general
general anesthesia,
anesthesia,
anesthesia, thethe therestorations
restorations
restorations were were
were tried
tried
tried onon onand
and and cemented
cemented
cemented with with
with dual-
dual-
dual-
curing
curing resin
resin cement
cement (Variolink
(Variolink ®® Esthetic,
Esthetic, Ivoclar
Ivoclar ™™ Vivadent
Vivadent ™, ™
™ , Ellwangen, Germany) using
, Ellwangen, Germany) using
curing resin cement (Variolink ® Esthetic, Ivoclar ™ Vivadent Ellwangen, Germany) using
Monobond
Monobond ® ® PlusPlus and
and Adhese
Adhese ® ®Universal
Universal VivaPen
VivaPen ® ®(Ivoclar
(Ivoclar
™ ™
™ Vivadent
Vivadent ,™Ellwangen,
, Ellwangen,
™, ™Ellwangen,
Monobond ® Plus and Adhese ® Universal VivaPen ® (Ivoclar Vivadent
Germany).
Germany). The The occlusion
occlusion was
was slightly
slightly raised
raised to to provide
provide
Germany). The occlusion was slightly raised to provide sufficient space for this restorative sufficient
sufficient space
space for
for this
this restorative
restorative
reconstruction. Fissure sealing (Helioseal ® , Ivoclar ™ Vivadent ™ , Ellwangen, Germany)
reconstruction.
reconstruction. Fissure
Fissure sealing
sealing (Helioseal
(Helioseal ® ® , IvoclarVivadent
, Ivoclar ™ ™ Vivadent, Ellwangen,
™ ™ , Ellwangen, Germany)
Germany)
was performed onthe
thesecond
second permanent molars, and stainless steel crowns (3M ™ , Neuss,
waswas performed
performed onon the second permanent
permanent molars,
molars, and and stainless
stainless steel
steel crowns
crowns (3M(3M™, ™ , Neuss,
Neuss,
Germany)
Germany)
Germany) were were
were placed placed
placed on ononall
all allfirst
first first permanent
permanent
permanent molars
molars
molars and and
and cemented
cemented
cemented with
with
with glass
glass
glass ionomer
ionomer
ionomer
cement (Ketac ™ Cem Aplicap ™ , ,3M ™ , Neuss, Germany) (Figure
cement
cement (Ketac
(Ketac ™
™ Cem Cem Aplicap
Aplicap ™
™, 3M , 3M
™ ™ , Neuss,
Neuss, Germany)
Germany) (Figure
(Figure 8).8). 8).

(a)(a)
Figure 8. Cont.
 Int.J. J.Environ.
Int. Environ.Res.
Res. Public
Public Health
Health 18,18,
2021,
2021, 7204 PEER REVIEW
x FOR 7 of7 10
of 9

(b)
Figure
Figure8.8.AA12-year-old
12-year-oldpatient
patientwith
withAIAItype
typeII:II:(a)
(a)upper
upperteeth;
teeth;(b)
(b)lower
lowerteeth.
teeth.Oral
Oralsurgery
surgerywaswas
performed
performedunder general anesthesia
under general anesthesiausing
using indirect
indirect composite
composite restorations
restorations and stainless
and stainless steel
steel crowns.
crowns.
Three-month follow-ups were recommended to identify and repair possible defects at
Three-month
an early stage. Infollow-ups
our case, the were recommended
patient adapted to to fullidentify and repairvery
crown treatment possible
well defects
and had
atnoansymptoms
early stage. in In
theour case, the patient adapted
temporomandibular joint afterto follow-up
full crownwithin treatment very well and
6 months.
had no symptoms in the temporomandibular joint after follow-up within 6 months.
3. Discussion
3. Discussion
Rehabilitating a patient with AI is challenging from both the functional and aesthetic
point of view. The
Rehabilitating complexity
a patient with of AIthe disease requires
is challenging from bothan interdisciplinary
the functional and approach
aestheticto
achieve
point optimal
of view. The treatment
complexity results. Several
of the diseasetreatment
requiresoptions have been proposed.
an interdisciplinary Recently,
approach to
the use optimal
achieve of bondedtreatment
restorations has gained
results. Several popularity
treatment dueoptions
to the many haveadvantages
been proposed.of these
materials,the
Recently, including
use ofexcellentbondedaesthetics,
restorations conservative
has gained approach,
popularity and improved
due to the wearmanymake.
advantages of these materials, including excellent aesthetics, conservative approach, andof
Dental rehabilitation is one important part of improving oral health-related quality
life for children
improved with generalized structure defects. The main objective of dental treatment
wear make.
of patients with hereditary
Dental rehabilitation is structural
one important anomalies
part ofis improving
to prevent nearby caries damage
oral health-related [6]. In
quality
ofaddition,
life for the dentistwith
children should counteract
generalized the abrasion
structure of the
defects. Theclinical
main crown
objectiveby performing
of dental
treatment of patients with hereditary structural anomalies is to prevent nearby the
early treatment in order to prevent dimension loss and tooth loss [3]. In every case, age
caries
of the patient must be considered in treatment planning. In
damage [6]. In addition, the dentist should counteract the abrasion of the clinical crown our case, aesthetic rehabilitation
bywas very important
performing for both patients.
early treatment in order to prevent dimension loss and tooth loss [3]. In
According to Toupenay
every case, the age of the patient et al., therebeisconsidered
must no agreement regarding the
in treatment protocol
planning. Infor
ourtherapy
case,
except the timing: treatment should begin
aesthetic rehabilitation was very important for both patients. as early as possible to prevent tooth sensitivity
and enamel loss [3].
According to Toupenay et al., there is no agreement regarding the protocol for
While stainless steel crowns, strip crowns, and compomer restorations are common in
therapy except the timing: treatment should begin as early as possible to prevent tooth
primary dentition, the growing jaw and the changing of teeth present challenges in terms
sensitivity and enamel loss [3].
of treatment options in mixed and permanent dentition. Therefore, the full spectrum of
While stainless steel crowns, strip crowns, and compomer restorations are common
dental materials should be exhausted during the development of a young adolescents. In
in primary dentition, the growing jaw and the changing of teeth present challenges in
particular, primary and mixed dentition only allow temporary therapy: conventional/resin-
terms of treatment options in mixed and permanent dentition. Therefore, the full
based glass ionomer cements, compomers/composites, strip crowns, preformed metal, or
spectrum of dental materials should be exhausted during the development of a young
tooth-colored crowns [11].
adolescents. In particular, primary and mixed dentition only allow temporary therapy:
In our first case, we mainly chose direct adhesive filling materials for tooth build-up
conventional/resin-based glass ionomer cements, compomers/composites, strip crowns,
because of the small loss of substance. It is typical for AI type I that the existing malformed
preformed
enamel hasmetal, similar oror tooth-colored crowns [11].to enamel that is formed regularly [1]. For ad-
identical characteristics
In our first case, we mainly
hesive therapy, this means that a normal chose direct adhesive
etching patternfilling
can materials
be expected forand
tooththebuild-up
adhesive
because of the small loss of substance. It is typical for
system used will act identically to physiologically formed enamel. Therefore, AI type I that the existing malformed
it was not
enamel
necessary has tosimilar
remove or theidentical
enamel characteristics to enamel in
partially or completely that
thisis case.
formed regularly
Especially in [1]. For
younger
adhesive
patients with teeth that have just erupted, it can be beneficial for the practitioner andthe
therapy, this means that a normal etching pattern can be expected and the
adhesive
patient to system
be ableused will act
to restore themidentically
noninvasivelyto physiologically formed enamel.
but still functionally Therefore,
and aesthetically. it
Con-
was not necessary to remove the enamel partially or completely
ventional or resin-based glass ionomer cements would not have been suitable due to their in this case. Especially in
younger patients
lower flexural with teeth
strength andthatwearhave just erupted,
resistance. These it can be beneficial
properties allow thefor the practitioner
material to be used
and the patient to be able to restore them noninvasively
temporarily chairside, but it should not be applied under optimal conditions when using but still functionally and
aesthetically. Conventional or resin-based glass ionomer
general anesthesia [12]. The development of compomers (polyacrylic/polycarboxylic acid cements would not have been
Int. J. Environ. Res. Public Health 2021, 18, 7204 8 of 9

modified composites) combines the advantages of glass ionomer cements (easy application)
and composites (aesthetics). Nowadays, compomers (e.g., Dyract® ) are the first choice
for restoring primary teeth. In contrast, composites should be used as a long-term filling
material in permanent dentition because of their higher wear resistance and compressive,
flexural, and tensile strength [13]. Only the second primary molars were restored with
stainless steel crowns to repair circular defects in this case. The advantages of metal crowns
are easy adaptation, gentle preparation, and time-saving handling [14]. Preformed ceramic
crowns for molars and incisors require strict preparation and involve high abrasion of
antagonists [15]. In follow-up, we could not find any loss of filling, which was also to be
expected regarding the etching pattern, which did not differ from the healthy enamel.
For affected permanent teeth, various full crowns are indicated. Depending on the
patient’s age, metal, composite, and ceramic crowns are common. While individual ceramic
crowns are contraindicated in adolescents due to jaw growth, composites can provide a
temporary restoration. In particular, newly introduced high-performance CAD/CAM com-
posites enable aesthetic restoration of malformed permanent teeth in young patients [16].
The patient in our second case showed severe loss of enamel in permanent dentition with
excess space in the upper and lower jaws. Therefore, indirect composite crowns were
used to rebuild the vertical dimension. Previous impressions and cooperation with the
dental technician made rehabilitation of the chewing system easier. The interdental spaces
could be used to avoid grinding of the teeth. The process of preparing the teeth could
protect the hard dental tissue. Other authors have described direct composite restoration in
combination with a wax-up to restore complex cases [17]. However, this treatment is very
time-consuming and demanding. In the case of adhesive restorations, it should be noted
that normal conditioning (etching pattern, effectiveness of the adhesive system) is not pos-
sible in affected enamel, so very early failure and loss of restorations are often recorded [18].
In addition, the remaining enamel can repeatedly flake off, so the corresponding tooth will
need a new restoration or the existing one will need to be expanded [4].
Generally, a successful therapy concept is based on a close recall program with oral
hygiene instruction, remotivation, and fluoride application. In this way, carious lesions or
restorative defects and gingivitis can be prevented. A limitation of our study is that up to
now there has only been a follow-up of 6 months after intervention. However, since the
parents’ compliance with the follow-up appointments is considered reliable, we expect a
good prognosis with regard to the aspects mentioned above. In addition, the use of electric
toothbrushes should also be considered regarding the practical implementation of home
oral hygiene. A study by Preda et al. showed that electric toothbrushes were superior to
manual toothbrushes in plaque removal. Therefore, rotating–oscillating or sonic-action
heads should be recommended for patients with difficult hygienic conditions to avoid
bacterial infiltration [19].
Due to the extensive treatment needs, complex treatment measures, and often age-
related insufficient cooperation, comprehensive rehabilitation under general anesthesia
cannot be avoided. Overall, the patients report less sensitivity, better oral hygiene ability,
and better quality of life.

4. Conclusions
The cases described in this paper show the complexity of the dental care of structural
anomalies of genetic origin. Patients with hereditary structural anomalies require close
lifelong dental care to maintain the therapeutic results.

Author Contributions: Conceptualization, M.M. and N.S.-W.; methodology, M.M.; software, J.C.B.;
validation, M.M., N.S.-W. and N.K.; formal analysis, M.M.; investigation, M.M., N.S.-W. and J.C.B.;
resources, N.K.; data curation, A.R.; writing—original draft preparation, M.M. and N.S.-W.; writing—
review and editing, M.M., J.C.B., N.K., A.R. and N.S.-W.; visualization, J.C.B.; supervision, N.K.;
project administration, N.K. All authors have read and agreed to the published version of the
manuscript.
Int. J. Environ. Res. Public Health 2021, 18, 7204 9 of 9

Funding: This research received no external funding.

Institutional Review Board Statement: Ethical review and approval were waived for this study, due
to individual parents’ consent.
Informed Consent Statement: Informed consent was obtained from all subjects involved in the
study. Written informed consent was obtained from the patients’ parents to publish this paper.
Data Availability Statement: Not applicable.
Conflicts of Interest: The authors declare no conflict of interest.

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