Open Journal of Stomatology, 2013, 3, 397-401 OJST
http://dx.doi.org/10.4236/ojst.2013.38067 Published Online November 2013 (http://www.scirp.org/journal/ojst/)
Multidisciplinary treatment approach of a patient with
amelogenesis imperfecta: A case report
Jihan M. Turkistani1, Abdullah S. Almushayt1, Sami A. Farsi2, Jihan M. Turkistani3*
1Faculty of Dentistry, King Abdulaziz University, Jeddah, Saudi Arabia
2King Fahad General Hospital, Jeddah, Saudi Arabia
3King Abdulaziz Medical City-Jeddah, Jeddah, Saudi Arabia
Email: *dr_jihant@hotmail.com
Received 3 July 2013; revised 4 August 2013; accepted 1 September 2013
Copyright © 2013 Jihan M. Turkistani et al. This is an open access article distributed under the Creative Commons Attribution Li-
cense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.
ABSTRACT
Introduction: Amelogenesisimperfecta (AI) is a de-
velopmental disturbance, genomic in origin, which
interferes with normal enamel formation of both pri-
mary and permanent dentitions in the absence of a
systemic disorder. Three major categories can be rec-
ognized clinically, namely hypoplastic, hypomatura-
tion and hypocalcified. Timely and comprehensive
intervention is critical to spare the patient from psy-
chological consequences of these disfiguring condi-
tions. Clinical Report: A 10-year-old boy was pre-
sented with dissatisfaction with appearance of his
teeth sensitivity and poor masticatory efficiency. His
medical history was noncontributory. Composite re-
storative material was selected as a suitable replace-
ment of the defective structures because of its esthet-
ics and high sustainability. Treatment Objectives:
The multidisciplinary approach was oriented toward
achieving functional and esthetic rehabilitation of
these teeth with minimal chair-side time. Treatment
Outcomes: The use of composite restorative material
resulted in successful and satisfactory enhancement
of the patient’s esthetic appearance, as well as protec-
tion against further wear and sensitivity thereby im-
proving his functional demands. The longevity of the
treatment outcome required meticulous maintenance
of oral hygiene and patient compliance. Conclusion:
Management of a patient with AI is a challenge for
the clinician. Treatment options vary considerably
depending on several factors such as age of the pa-
tient, socioeconomic status, severity of the disorder,
and most importantly, the patient’s cooperation.
Composite restorative material is considered an ex-
cellent conservative transitional treatment for protec-
tion of AI weakened teeth.
Keywords: Amelogenesi s I mperfecta; Hypocalcified
Enamel; Management; Multidisciplinary; Composite
Restorative Material
1. CASE PRESENTATION
A healthy, 10-year-old Saudi boy was referred to the
pediatric dentistry specialty clinic at King Abdulaziz
University by a general dental practitioner for treatment
of his disfigured teeth. His chief complaints were sensi-
tivity to hot and co ld, dissatisfaction with the appearance
of his teeth, and a compromised masticatory function.
The medical history indicated no contraindications for
dental treatment.
A thorough extra and intraoral clinical evaluation re-
vealed poor oral hygiene with plaque accumulation,
which had resulted in generalized gingivitis. The patient
presented short clinical crowns with generalized areas of
thin, discolored, hypoplastic enamel and areas of frac-
tured enamel and exposed dentin (Figur e 1).
A premature loss of vertical dimension, as well as an
overbite of 30% and an over jet of 6 mm, was evident. A
Class II Angle molar relationship, division I malocclu-
sion, was evidenced on both right and left sides. There
are multiple spaces between the teeth. At the time of
evaluation, existing restorations on teeth #16 and 46
were defective resin composites, and teeth #26 and 36
had large carious lesion s.
The initial panoramic, bitewing and periapical radio-
graphic examination (Figures 2 and 3), showed an early
permanent dentition stage, with all permanent teeth pre-
sent and covered by thin layer of enamel. The under-
lying dentin-pulp complex appeared normal. The dental
ag of this patient is earlier than his chronologic age. e
*Corresponding a uthor.
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J. M. Turkistani et al. / Open Journal of Stomatology 3 (2013) 397-401
398
Figure 1. Preoperative clinical pictures of affected teeth.
Figure 2. Panoramic view.
Figure 3. Periapical and bitewing view.
Tooth germs of all four third molars were absent.
2. WHAT IS YOUR DIAGNOSIS
Discussion
The clinical and radiographic findings suggested a he-
reditary enamel defect i.e. Amelogenesis Imperfecta (AI),
and this diagnosis was strengthened by the positive fam-
ily history of the same clinical presentation from the fa-
ther side, his 8-year-old brother had suffered from the
same condition, in addition, given history of disfigured
primary dentition in the same manner. Consequently,
based on the Witkop’s classification [1,2], diagnosis of
hypoplastic AI was made. Caries risk was assessed by
interviewing the patient regarding diet, oral hygiene, and
general lifestyle habits. The patient was placed by the
assessment in a “high risk” category, given the presence
of several predisposing factors, with AI carrying the
most weight of all.
Amelogenesis imperfecta is a developmental inherited
disturbance that is associated with malfunction of the
enamel-forming proteins in the absence of a systemic
disorder. In general, it affects all or nearly all of the teeth
in both the primary and permanent dentitions [3,4]. The
AI trait can be transmitted by either autosomal dominant,
autosomal recessive, or X-linked modes of inheritance
[5]. The estimated frequency of AI in the US population
is 1:7000 [5]. The most widely accepted classification of
AI includes three types: hypocalcified, hypoplastic and
hypomaturation [1,2,6]. In the hypoplastic type, there is a
deficiency in the quantity of enamel. The enamel is cor-
rectly mineralized and appears hard and shiny but is mal-
formed. In the hypocalcified type, the enamel is formed
in relatively normal amounts but is poorly mineralized,
soft, and friable and can be easily removed from the den-
tin. In the hypomaturation type, abnormalities in the
maturation stage of enamel formation result in a mottled
appearance, opaque white to red-brown coloration, and
enamel that is softer than normal and tends to chip from
the underlying dentin [6 ]. To date, at least 14 forms of AI
have been described based on the specific dental abnor-
malities and pattern of inheritance [1].
Children with AI can exhibit variable manifestations,
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J. M. Turkistani et al. / Open Journal of Stomatology 3 (2013) 397-401 399
with discoloration (yellow, brown, or gray), generalized
areas of dentin exposed, pitted enamel with an increased
susceptibility to plaque accumulation, caries, anterior
open bite, and hyp ersensitivity to temperature change s as
some of the most commonly occurring signs [7,8].
The differential diagnosis of AI includes other forms
of enamel dysmineralization which will exhibit a pattern
based upon the time of insult, thus affecting the enamel
forming at the time. In contrast, AI will affect all teeth
similarly and can have a familial history. Fluorosis can
mimic AI, but usually the teeth are not affected uni-
formly, often sparing the premolars and second perma-
nent molars. A history of fluoride intake can aid in the
diagnosis [8].
3. THERAPY
The complexity of the condition requires an interdisci-
plenary approach for optimal treatment outcomes. Dif-
ferent treatment options have been proposed in the dental
literature. Options ranged from simple microabrasion [9-
11], to composite restorations [12-15], gold or stainless
steel crowns [13], all-ceramic crowns [7,16], metal-ce-
ramic crowns [7,12,17], and veneers [18,19]. Many case
reports have focused on the early management of chil-
dren and adolescents, and the use of bonded restorations
has gained popularity because of the many benefits asso-
ciated with these materials; excellent esthetics, conserva-
tive approach, and improved wear make their use advan-
tageous [12,13].
Pediatric Dentistry and Prosthodontics participated in
treatment planning. Regarding orthodontic therapy and
achievement of a Class I occlusion, although needed,
was not deemed a priority on the treatment sequence.
The main goal of treatment was to provide transitional
restorations for the protection of remaining tooth struc-
ture against further wear and sensitivity. Fu ll coverage of
all teeth with fixed prosthesis was identified as the ideal
treatment; however, this approach requires the removal
of a considerable amount of tooth structure. In addition,
the patient’s incomplete skeletal growth excluded this as
an immediate option. Therefore, resin composites, par-
ticularly the indirect type, were chosen to provide an
excellent prosthetic alternative until co mpletion of skele-
tal growth. Indirect resin composites (also referred to as
prosthetic composites) were introduced to dental practice
to improve the clinical performance and provide optimal
esthetics by ensuring precise marginal integrity, ideal
proximal contacts and excellent anatomic morphology
[20].
The following treatment sequence took place: Maxil-
lary and mandibular arch impressions were made. Regis-
tration of skeletal relations was made with facebow and
appropriate bite registration materials, and the case was
mounted in a semi-adjustable articulator (Figure 4).
Evaluation of the articulated study models revealed
that there is no need to increase the occlusal vertical di-
mension, because enough space is available for the
thickness of restorative material of the posterior teeth. A
diagnostic wax-up of all premolars, canines and incisors
was made (Figure 5), followed by impression of each
arch for construction of clear template material which
has been used later on for temporization ( Figure 6).
Stainless steel crowns were planned for coverage of
defective teeth # 16 , 26, 36, and 46 in order to provide a
posterior bite support, which will maintain the vertical
dimension of occlusion. After cementation of the stain-
less steel crowns, all remaining permanent teeth were
prepared for receiving the prosthetic composites. Tooth
preparation involv ed axial and occlusal reduction limited
to the removal of areas of thin hypoplastic enamel and
undercut s ( Figure 7).
Final maxillary and mandibular full arch impressions
were made with a silicone impression material along
with bite registration, wh ich were sent to the dental labo-
ratory for construction of prosthetic composite crowns.
For temporization, the clear matrix template was filled
with injectable temporization material (Protemp™ Plus,
3 M ESPE). The matrix was carefully seated in place and
kept in position through slight digital pressure for 1 - 3
minutes. Upon removal of the matrix, any areas of cer-
vical overextension were trimmed, and any unsealed
Figure 4. Registration of skeletal relations with face bow and
bite registration.
Figure 5. Diagnostic wax-up.
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J. M. Turkistani et al. / Open Journal of Stomatology 3 (2013) 397-401
400
Figure 6. Clear template material for upper and lower arches.
Figure 7. Teeth preparation.
areas along the margin were filled with a flowable resin.
The temporary material was cemented with temporary
cement (Figure 8).
Final composite crowns were delivered from the den-
tal laboratory (Figure 9), and these crowns were tried-in
the patient’s mouth after cementing them with temporary
cement. Occlusal and proximal contact, marginal integ-
rity and anatomic morphology were checked. The final
restorations were sent back to the dental laboratory for
final polishing and glazing. Lastly, they were cemented
with permanent resin cement (Figure 10). The final res-
torations exhibited not only good esthetics but also a
return to an optimal masticatory function as well as pro-
viding complete coverage of all affected areas of the ex-
posed dentin. These restorations not only addressed the
patient’s hypersensitivity issue but also contributed to the
prevention of plaque accumulation.
4. TREATMENT OUTCOME
The use of composite restorative material resulted in
successful and satisfactory enhancement of the patient’s
esthetic appearance, as well as protection against further
wear and sensitivity thereby improving his functional
demands. The longevity of the comprehensive treatment
of this patient required strong emphasis on meticulous
oral home-care regimen. Equally important to the suc-
cess of this treatment are the periodic recall visits for
monitoring of the restorations placed. The patient was
monitored at 3-month intervals for 6 months. The resto-
rations exhibited no signs of deterioration. The patient’s
oral hygiene was satisfactory. The restorations remained
intact, with no discoloration, crazing, or carious lesions
(Figure 11).
5. CONCLUSIONS
1) The importance of an interdisciplinary approach to
the successful treatment of a patient with hypoplastic AI.
has been conc luded.
2) The conservativeness and suitability of full cover-
age resin composite restorations for the transitional
Figure 8. Temporization.
Figure 9. Final composite crowns.
Figure 10. Permanent cementation with resin cement.
Figure 11. 6-month follow-up visit.
treatment of affected teeth on an adolescent patient who
has not yet completed skeletal growth has also been con-
cluded.
3) Treatment options vary considerably depending on
several factors such as patient’s age, socioeconomic
status, severity of the disorder, and most importantly, th e
patient’s cooperation. For optimal results, the treatment
must be determined on an individual basis and with con-
sultation of different disciplines.
4) This case emphasizes the importance of recognizing
unusual pathology within the oral cavity, early diagnosis
and prompt referral for subsequent management.
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