Management of Amelogenesis Imperfecta in Adolescent

Patients: Clinical Report

Liliana Ortiz, DDS,1 Ann Marie Pereira, DDS,2 Leila Jahangiri, BDS, DMD, MMSc,1 & Mijin Choi, DDS, MS,
FACP, MBA1
1
Department of Prosthodontics, New York University College of Dentistry, New York, NY
2
Department of Prosthodontics, Nova Southeastern University College of Dental Medicine, Fort Lauderdale, FL

Keywords
Adolescent patients; amelogenesis
imperfecta; enamel defects; interdisciplinary
treatment; mixed dentition.
Correspondence
Mijin Choi, DDS, MS, FACP, MBA, Clinical
Associate Professor, Dept. of Prosthodontics,
NYU College of Dentistry, 345 E 24th St.,
Clinic 4W, New York, NY 10010.
E-mail: mc185@nyu.edu.
The authors deny any conflicts of interest
regarding this study.
Accepted April 27, 2019
doi: 10.1111/jopr.13069
Abstract
The oral rehabilitation of adolescent patients with amelogenesis imperfecta (AI) is
complex due to the presence of mixed dentition with altered eruption sequence. In
this article, the interdisciplinary treatment approach for adolescent patients with AI
is discussed. The types and timing of treatments at various stages of growth are
described through a literature review on this topic. AI is an inherited condition that
disturbs the development of the enamel structure. Because of the presence of mixed
dentition, definitive treatment options often have to be delayed until eruption of
permanent dentition is complete, requiring careful treatment coordination and proper
sequencing between different dental disciplines starting at a young age. Adolescent
patients require prosthodontic treatment design that can be adapted to the changes
in arch shapes, sizes, interarch relationship, and esthetic needs. AI patients are often
challenged with both excessive and limited restorative spaces within the same arch
due to the abnormal growth patterns, enamel structure, tooth size, and tooth shape.
Therefore, careful determination of the required restorative space is critical to ensure
optimal prognosis. This clinical report discusses treatment recommendations, timing
of various treatment modalities, and involvement of appropriate interdisciplinary
teams for managing adolescent patients.

Amelogenesis imperfecta (AI), also known as congenital
enamel hypoplasia, affects the development of enamel struc-
ture. The majority of AI cases are due to mutations of genes that
encode enamel matrix proteins, which govern critical function
during enamel mineral initiation, elongation, and organization.1
Researchers have described many forms of AI, which can be
categorized in at least 13 classification systems based solely on
phenotypes (hypoplastic, hypomaturation, hypocalcified and
hypomature-hypoplastic enamel with taurodontism), mode of
inheritance, molecular defects and biochemical analysis. AI can
be inherited as an autosomal dominant, autosomal recessive,
X-linked recessive; or as a result of a spontaneous mutation in
patients who have no family history of AI. This anomaly affects
both the primary and permanent dentitions.2-4
By strict definition, AI conditions exist independent of other
related systemic disorders; however, inherited enamel defect
can be a component of a syndrome that involves other organ
systems. An example is the enamel renal syndrome.5,6
The prevalence of AI varies. For example, in the United
States, the prevalence is estimated at 0.06 to 0.07:1000 individ-
uals. In a northern Swedish county, its prevalence is estimated
as high as 1.4:1000 individuals. These differences are mainly
due to diagnostic and demographic criteria along with related
mutant genes in the studied population.7,8
Intraoral clinical features of hypomature AI include abnor-
mally rough and pitted tooth surfaces and discoloration of teeth
with hypersensitivity. These abnormal tooth surfaces attract
plaque and calculus, causing teeth to be at a high risk of caries
and attrition at a young age. Defective enamel or lack of enamel
creates tooth hypersensitivity. Therefore, full-coverage restora-
tions are often needed for function, comfort, and esthetics.
Additional clinical characteristics of AI include: short clinical
crown heights, malformed teeth, congenitally missing teeth, su-
pernumerary teeth, pulp calcifications, taurodontism, root mal-
formations, anterior open bite, and abnormal growth pattern of
the maxilla and the mandible.2,11,12
In females, the growth of the maxilla and mandible is usually
completed by the age of 14-15 years old and in males, it can
continue until 18 years old of age.9,10
These abnormal growth patterns in maxilla and mandible typ-
ically present with inadequate posterior restorative space with
minimal to no loss of occlusal vertical dimension (OVD), but
with an excessive anterior restorative space due to the anterior
open bite caused by the abnormal growth.13 The combination

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C 2019 by the American College of Prosthodontists 607
AI in Adolescent Patients Ortiz et al

of limited posterior restorative space and the excessive anterior

restorative space poses a clinical challenge because short poste-
rior clinical crowns can lack adequate retention and resistance
form. The optimal treatment often includes both the increase
in OVD and a crown-lengthening procedure. The amount of
increase in OVD should be carefully assessed based on the
need to correct short posterior teeth, but this can result in ex-
cessive anterior crown height with a compromised crown-root
ratio. Therefore, a balanced anterior and posterior restorative
space is imperative for favorable long-term prognosis of the
treatment.
The purpose of this clinical report is to focus on the manage-
ment of an adolescent patient with AI. The goal is to provide the
treatment management steps, describe appropriate timing, and
define the involvement of a multidisciplinary team to enhance
overall treatment outcomes. Figure 1 lntraoral frontal view in maximum intercuspation.

Clinical report
A 16-year-old male patient presented with chief complaints that
included extreme tooth sensitivity, dissatisfaction with tooth
size, shape, and shade, along with poor chewing function. He
indicated that he did not take any medications, and reported no
drug allergies. He also denied use of alcohol, tobacco, or any
recreational drugs. Before presenting to the clinic, the patient
received pediatric dental care for 11 years that included oral
hygiene maintenance under nitrous oxide inhalation sedation
because of his extreme tooth sensitivity to cold. Socially, the
patient was reclusive, never smiled, and did not integrate well
at school because of his oral condition. His mother was very Figure 2 Panoramic radiograph.
supportive and had hope that the dental treatment could "fix"
her son’s social issues.
PDI Class IV for dentate patient (ACP Prosthodontic Diagnos-
The initial clinical examination showed poor oral hygiene
tic Index) and AI: Type – Hypomature.
due to hypersensitive teeth, which also contributed to difficulty
The treatment goals were divided into 3 treatment phases.
in chewing and eating. The patient was observed to have a
The first phase (from age 1 to 15) was to: (1) improve the
tongue-thrusting habit.
patient’s oral hygiene; (2) build an interdisciplinary care team;
The extraoral examination showed unequal distribution of
(3) provide full-coverage provisional restorations to reduce
facial thirds. The lower third of the face appeared to be longer.
hypersensitivity; and (4) provide adequate chewing function.
The patient stated that he refused to smile because of the ap-
The second phase (15 to 21 years old) was to provide final
pearance of his teeth and the extreme sensitivity.
restorations, and the third phase (22 years old and older) was
The individual tooth examination showed generalized defec-
for periodontal maintenance care.
tive enamel with yellow-brown, rough surfaces and exposed
dentin. The patient was hypersensitive to air and water. There
Phase 1: Diagnostic and provisional phase (1 to
was no loss of OVD, and an anterior open bite of 3 mm was
15 years old), pediatric dentistry and
present with short posterior teeth and limited posterior restora-
orthodontics
tive space (Fig 1).
A pretreatment panoramic radiograph (Fig 2) was taken, The patient was referred to orthodontics to correct misaligned
and normal trabecular pattern with no evidence of gross bony teeth, periodontics to treat the existing chronic gingivitis, and
pathologies was noted. All permanent teeth were present, ex- oral surgery to determine the timing of extraction of the im-
cept the impacted teeth: maxillary and mandibular third molars, pacted teeth. The different treatment options with and without
maxillary and mandibular canines. Maxillary and mandibular orthodontic treatments were presented to the patient and the
first and second molars were partially erupted. According to the parent; however, the orthodontic treatment option was rejected
cephalometric analysis, the patient was determined to be Skele- because of the time required and the inability to bond orthodon-
tal Class III. Based on these findings, the following clinical tic brackets to the defective enamel surfaces. During this phase,
diagnoses were given: generalized chronic plaque-induced gin- the patient received crown lengthening on all posterior teeth to
givitis; impacted maxillary and mandibular canines and third improve retention and resistance forms.
molars; partially erupted maxillary and mandibular first and The articulated casts were scanned using 3Shape D1000
second molars; occlusal plane discrepancies; anterior open bite; scanner, and 3Shape Dental Design Software (Copenhagen,

608 Journal of Prosthodontics 28 (2019) 607–612 

C 2019 by the American College of Prosthodontists
Ortiz et al
Demark) was used to complete a diagnostic virtual wax-up at
the existing OVD as there was no loss of OVD. The appropriate
occlusal plane and incisal edge position were established to op-
timize occlusion and esthetics. A mock-up was created from
maxillary right first premolar to maxillary left first premo-
lar with autopolymerizing polymethylmethacrylate (PMMA)
(Alike acrylic; GC America, Alsip, IL) to evaluate esthetics
and phonetics (Fig 3).
To fabricate long-term provisional restorations, a decision
was made to use CAD/CAM provisional crowns after the
patient’s approval of the intraoral mock-up restorations. The
CAD/CAM provisional material was multilayered compos-
ite PMMA blocks (Harvest Dental, Ephrata, PA). The mate-
rial is highly esthetic with good color stability and strength14
(Fig 4). The provisional restorations were fabricated as single
units for optimal periodontal health and were cemented with
noneugenol temporary cement (Temp-bond NE; Kerr, Bioggio,
Switzerland).
During this first phase, the provisional restorations were re-
moved every 2 months for oral hygiene and fluoride treatment.
Because the patient’s growth was not yet complete, at 6-month
intervals he was monitored for tooth movement and changes in
tooth position by comparing the provisional models to his clin-
ical presentation. Throughout this phase, the patient reported
improvement to sensitivity, masticatory efficiency, and self-
confidence.
After the provisional phase was completed, impacted canines
(teeth 6 and 11) and third molars were extracted. The extraction
sites healed without any complications.
Phase 2: Restorative phase (15 to 21 years old),
prosthodontics, orthodontics, endodontics, and
periodontics
When the patient reached the age of 18, the OVD and esthet-
ics were reevaluated. There was no loss of OVD. The planned
incisal edge position and esthetic plane remained the same.
The conditions of the abutment teeth did not require any ad-
ditional periodontal, endodontic, orthodontic, or surgical treat-
ments. The definitive restoration design was finalized to in-
clude: single-unit lithium disilicate all-ceramic crowns (e.max;
Ivoclar Vivadent, Schaan, Liechtenstein) for maxillary central
incisors, maxillary left lateral incisor, maxillary left canine,
maxillary premolars, mandibular central and lateral incisors,
mandibular canines, and mandibular premolars (teeth #4, 8 to
13, 20 to 25, 27 to 29); zirconia fixed dental prosthesis (FDP)
with layered feldspathic porcelain on the facial and buccal sur-
faces (Zirkonzahn, Gais, Switzerland) for maxillary right first
premolar, maxillary right canine (pontic) and maxillary right
lateral incisor (teeth #5 to 7), and full-coverage metal-ceramic
crowns with metal occlusal surface (Noble alloys-semiprecious
metal) were planned for maxillary first and second molars and
mandibular first molars (teeth #2, 3, 14, 15, 19, and 30).
Final impressions were made with polyether impression
materials (Permadyne and Impregum medium viscosity, 3M
ESPE, St. Paul, MN) using the double retraction cord tech-
nique. The completed all-ceramic and posterior metal-ceramic
crowns were verified for fit, occlusion, esthetics, and phonet-
ics. The restorations were cemented with a resin luting agent
Journal of Prosthodontics 28 (2019) 607–612 
C 2019 by the American College of Prosthodontists
AI in Adolescent Patients
for all-ceramic crowns (Multilink; Ivoclar Vivadent) and glass
ionomer cement for posterior metal-ceramic crowns (Fuji; GC
America). The patient was very satisfied with the treatment
results.
Phase 3: Maintenance (>22 years old),
prosthodontics and periodontics
Oral hygiene instructions were given, and the patient was pre-
scribed with recall evaluations recommended at 3-month inter-
vals. Figures 5 to 7 show the inserted definitive restorations and
gingiva health of the patient via frontal and occlusal views of
the maxilla and the mandible.
Discussion
Early recognition of AI with appropriate care is essential in
preventing the progressive damage of dentition while avoid-
ing the negative psychological impact on the patient due
to the unique restorative challenges posed by the abnormal
growth and eruption patterns. The treatment goals should be
divided into multiple phases based on the patient’s needs and
age.
The key treatment considerations for managing adolescent
patients with AI include the determination of appropriate inter-
disciplinary treatment team, the types of treatments to be given
during early (11 to 14 years old) and adolescent phases (15
to 21 years old), and the timing of definitive therapies includ-
ing fixed restorations and implant therapy when needed. The
timing of various interdisciplinary involvement is very impor-
tant for adolescent patients, because the growth of the maxilla
and mandible can change the position of the alveolar bone and
the maxillomandibular relationship. The pubertal growth spurt
begins between the age of 10 to 12 in girls and 15 to 16 in
boys; however, age ranges cannot be used as the sole indicator
for initiation of definitive dental treatment, because the rate of
growth during puberty varies widely. If implant therapy is se-
lected, the stabilization of the growth spurt should be verified
with hand and wrist radiographic analyses for the fusion of the
epiphysis and metaphysis and the third distal phalanx, as there
are large differences in the pubertal growth spurt with ages and
sexes.15
In the early adolescence phase, pediatric dentistry and or-
thodontics play the dominant role in the diagnosis and man-
agement of primary and permanent dentition. Prosthodontic
treatment planning typically begins around 15 years of age.
Figure 8 describes the recommended specialty care during the
various growth phases. The primary purpose of treatments dur-
ing this early adolescence phase should be focused on eliminat-
ing tooth sensitivity, maintaining oral hygiene, and providing
esthetics with provisional restorations.
One of the typical clinical features of AI is the anterior open
bite. The prevalence of anterior open bite among AI patients is
reported to be between 24% and 60%.11-16 However, the loss
of OVD is typically not reported in the literature. The patient
in this report presented with anterior open bite without loss of
OVD, and short posterior clinical crown height. This clinical
presentation resulted in excessive anterior restorative space and
limited posterior restorative space. The conflicting restorative
609
AI in Adolescent Patients
Figure 3 Maxillary mock-up, frontal view.
Figure 4 Milled provisionals in composite PMMA, frontal view.
Figure 5 Definitive restorations, frontal view.
space issues within the same arch require a careful decision-
making process that should include crown-lengthening proce-
dures. Crown lengthening is often needed to improve the reten-
tion and resistance forms of the teeth in the area with limited
restorative space.
The tooth surface characteristics associated with AI include
the degeneration of the reduced enamel epithelium with some
610 Journal of Prosthodontics 28 (2019) 607–612 
Ortiz et al
Figure 6 Maxillary definitive restorations, occlusal view.
Figure 7 Mandibular definitive restorations, occlusal view.
areas of mineralized pellicle.17 The defective enamel surface
is known to be due to the defective amelogenin proteins. In an
animal study of the adhesive bond strength to enamel surface
with AI condition,18 the AI enamel showed that the lower bond
strength was statistically insignificant; however, the long-term
bond strengths of AI enamel and dentin surfaces were not
known.
A retrospective study of AI with extensive restorations
evaluated various complications, and debonding was listed
as the number one problem associated with AI. Porcelain
fractures, caries, and the need for endodontic therapy were
frequently observed complications.19 Therefore, the selection
C 2019 by the American College of Prosthodontists
Ortiz et al AI in Adolescent Patients

Figure 8 Timeline of interdisciplinary team management.

Table 1 Specialty care involvement and treatment considerations

Specialty involved Treatment considerations and decisions

Infancy to early Pedodontics r AI can be diagnosed by 1 to 2 years old

adolescence (1 to 15 r Frequent oral hygiene regimen due to the tendency of high plaque accumulation
years old) r Space maintenance to allow eruption of permanent teeth
Orthodontics r Severe malposition of the teeth
r Often complex ortho treatment is indicated (max 4 to 5 mm with conventional method
& max 8 mm with anchorage device)
r Bonding of ortho brackets may be an issue due to abnormal enamel surface
Middle and late Endodontics r Possible false positive endodontic diagnosis due to inherent hypersensitivity associated
adolescence (15 to 21 with defective enamel
years old) Oral surgery r Timing of extraction of impacted teeth to reduce premature alveolar bone loss and loss
of OVD
Periodontics r Increase plaque and calculus accumulation
r Crown lengthening due to short clinical crown
r Frequent oral hygiene maintenance regiment
Prosthodontics r Management of inadequate posterior restorative space w/o loss of OVD
r Definitive treatment plan & implant therapy after 15 y/o for girls and 18 y/o for boys
r Management of hypersensitivity
r Esthetics and proper chewing function

of appropriate restorative materials, adhesive cement, and the Another advantage of lithium disilicate is the ability to etch
cementation technique is crucial. the restorative surface to improve bond strength, thus reducing
All-ceramic materials such as lithium disilicate, alumina, adhesive cement failure. On the other hand, zirconia offers
and zirconia are known to have more favorable tissue response.

Journal of Prosthodontics 28 (2019) 607–612 

C 2019 by the American College of Prosthodontists 611
AI in Adolescent Patients
superior flexural strength and, therefore, is suitable for FDP
design.20-22
In the treatment of this patient, all-metal ceramic crowns,
lithium disilicate crowns (e.max), and zirconia FDP were used.
Metal-ceramic crowns on the distal-most teeth were used to
maintain the OVD within the limited posterior restorative space.
The lithium disilicate crowns for single restoration and zirconia
FDP in the maxillary anterior region were selected for esthetics
and better tissue response.
Table 1 outlines the treatment management approach and
treatment modalities for AI from infancy to early adolescence
period and middle to late adolescence period. The early ado-
lescence period includes pediatric dentistry (1 to 15 years old)
and early orthodontic assessment around 6 years of age. The
middle-to-adolescence period requires the involvement of pe-
riodontics, orthodontics (2nd evaluation), prosthodontics (by
15 years old), endodontics, and oral surgery teams. Patients in
these phases often need periodic orthodontic evaluation to as-
sess changes in skeletal relationship and to determine the need
for additional orthodontic and surgical intervention for final
restorations and prostheses. Finally, in the restorative phase,
between the ages of 15 to 21 years, definitive prosthodontic
treatment is rendered along with long-term maintenance treat-
ment. Patients with AI typically receive restorations at an early
stage of their lives and require a long-term care plan. The selec-
tion of restorative materials for defective tooth surface should
include careful consideration for long-term bond strength and
favorable soft tissue response.
Summary
The oral rehabilitation of adolescent patients with AI is com-
plex, since teeth have different eruption sequences, and defini-
tive treatment cannot be rendered until the complete eruption
of the permanent dentition. During the adolescent phase, the
prosthetic treatment options should not include any irreversible
splinted fixed prosthesis. The restorative treatments should fo-
cus on preserving anatomical structures that will be used in
the definitive treatment phase, and providing functional and
esthetic reversible treatments to improve self-confidence and
development of the maxilla and mandible.
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C 2019 by the American College of Prosthodontists