Dental Rehabilitation of Amelogenesis Imperfecta in The Mixed Dentition

Cas e R e po r t
Dental Rehabilitation of Amelogenesis Imperfecta

in the Mixed Dentition
Ruby Kharkwal Shah
MDS-Pediatric and Preventive Dentistry, Private Practioner

Corresponding Address: Dr. Ruby Kharkwal Shah, 74 B wing, Shivam Apts. 96 J P Road,
Andheri West, Mumbai - 400058, Maharashtra, India. E-mail: drrubykharkwal@gmail.com
Abstract
Amelogenesis Imperfecta represents a group of hereditary defects of enamel unassociated with any other generalised defects.
It is entirely an ectodermal disturbance, since the mesodermal components of the teeth are basically normal. This clinical report
describes the management of a 10 year-old boy with a X-linked hypocalcified type of Al. The first phase of the treatment was
preventive measures to improve dental and periodontal health. On the second phase the root stumps were extracted and the
molars were endodontic treated and covered with stainless steel crowns. Polycarbonate crowns on maxillary permanent incisors
and direct composite veneers on mandibular permanent incisors were placed.
Keywords: Amelogenesis imperfecta, Composite, Esthetics, Polycarbonate crowns, Stainless steel crowns
INTRODUCTION
Amelogenesis imperfect comprises of group of
developmental anomalies affecting the morphology and
appearance of enamel of a few teeth or all teeth. This may
also be assocaied with other biochemical changes in the
body.1 There are four main types of AI based on phenotype
namely hypoplastic, hypomaturation, hypocalcified and
hypomaturationhypoplastic with taurodontism.2 AI has
genetic origin and the mode of inheritance may vary. It can
affect both primary and permanent dentition.3
Type
Forms of AI
Clinical features
Type I
Hypoplastic
Enamel
Hypomatured
Enamel
Hypocalcified
Enamel
Hypomaturedhypoplastic Enamel
Well-mineralized enamel but

in reduced amount
Mottled, opaque white-brown
or yellow discoloured enamel
Pigmented, softened and
easily scrapable enamel
Combined features of
hypomatured and hypoplastic
Type II
Type III
Type IV
According to the literature, AI patients, regardless of subtype,

have similar oral complications: tooth sensitivity, poor dental
esthetics, and decreased occlusal vertical dimension. Other
dental anomalies associated with AI include, but are not
limited to multiple impacted teeth, congenitally missing teeth,
open occlusal relationship, taurodontism, pulpal calcification,
root malformations, progressive root and crown resorption
and anterior open bite occlusion.5
International Journal of Scientific Study |March 2014 | Vol 1 | Issue 6
The treatment of patients with AI should be planned

taking both the clinical and the emotional demands into
consideration. Historically, patients used to cover their
teeth with either pieces of paper or chewing gum in order
to have an ordinary appearance.
The psychological impact on an individual who has teeth
affected by amelogenesis imperfecta cannot be underestimated.
The individual may be too embarrassed to smile because they
are unhappy with the appearance of their teeth.6 Therefore,
the planning of esthetic restorations on the anterior teeth may
not only preserve the tooth structure and reduce sensitivity,
but also eliminate the adverse social impact of their teeth.
Treatment is as ever based on the principles of prevention
before intervention. However, since AI has genetic origin
so preventive care cannot be possible and intervention is
to be done early in a more radical manner.
This report describes the clinical management of a case
of amelogenesis imperfecta in the mixed dentition, with
special emphasis on the esthetic demands of the patient
and the longevity of the restorations.
CASE PRESENTATION
A 10 year old male attended the clinic because of discolored
primary and permanent teeth which manifested some thermal
56
Kharkwal: Dental Rehabilitation of Amelogenesis Imperfecta in the Mixed Dentition
sensitivity. A detailed medical, dental, and social history was

obtained. The patient was examined dentally and medically.
Clinically, the childs oral hygiene was unsatisfactory and
he exhibited moderate chronic marginal gingivitis. On
examination it was found that enamel thickness of all teeth
was reduced and in some teeth dentin was exposed. The
teeth had orange brown discoloration, with diffuse pitting
present on all surfaces of the teeth. (Figure 1a-e).
Radiographic investigation included an Orthopantomogram
(OPG). OPG showed retained 54 root stumps, 65 with
The treatment objectives were to improve the esthetics,

eliminate the tooth sensitivity, prevent further loss of tooth
structure, modify the childs attitude and behavior towards
dental treatment and improve his periodontal health.
The parents were informed of the diagnosis and all the
treatment modalities were discussed with them. As part of
the treatment plan, the treatment alternatives were explained
to the child and his parents. This included the amount of
tooth structure that would need to be removed, the expected
clinical longevity of the restorations and the length of the
treatment period. After considering all of the treatment
options, it was decided to place direct composite veneers
(3M ESPE Filtek Z250 Universal Restorative) on the
permanent lower incisors, Polycarbonate preformed Crowns
(3MTM ESPETM) on the permanent upper incisors and
stainless steel crowns (3MTM ESPETM) on the primary and
permanent molars; the parents understood that this could
temporarily compromise the esthetics in the molar regions.
Initial periodontal therapy consisted of oral prophylaxis and
oral hygiene instructions, scaling, and root planning. This was
followed by extractions of retained 54 root stumps and 65.
A Universal restorative composite was chosen to restore
the defective tooth structure of the permanent incisors.
No preparations were preformed on the incisors, other
than cleaning with a rotary bristle brush with pumice prior
to acid-etching with 35% phosphoric acid for 30 seconds
followed by rinsing with water spray. The labial surfaces
of the mandibular incisors were then directly restored
with resin composite. Conservative tooth preparation was
e
Figure 1: (a-e) Preoperative clinical picture showing irregular
dark yellow to brown discolored labial surface of maxillary and
mandibular teeth
Figure 2: The radiographic appearance of the dentition

panoramic radiograph showing diminished enamel on the
erupted teeth and all of the developing teeth consistent with the
patients chronological age
57
completely resorbed roots and pulpally involved 85 and 75.

Crowns of all teeth showed reduced enamel thickness with
normal dentin. Root morphology of all teeth was completely
normal (Figure 2). The clinical and radiographic features
and the family history were consistent with a diagnosis of
X-linked hypocalcified Amelogenesis Imperfecta.
Figure 3: Postoperative clinical picture showing esthetic

composite veneering in mandibular teeth and Polycarbonate
crowns on maxillary teeth
International Journal of Scientific Study | March 2014 | Vol 1 | Issue 6
performed on the maxillary permanent incisors prior to

placement of the polycarbonate crowns (Figure 3).
Endodontic procedures were performed on 85, 75 and 74.
Furthermore, preformed stainless steel crowns (3M) were
placed following minimal slice preparations of the teeth
bilaterally at each visit so as to balance the occlusion and
alter the vertical dimension (Figure 4a, 4b). The adaptation
and quality of the margins of the preformed stainless steel
crowns were evaluated using a panoramic radiograph.
Maxillary primary canines were left un-restored as they
were about to exfoliate.
The stainless steel crowns on the first permanent molars
are considered to be only temporary restorations. Once
the second permanent molars and the premolars have
established the level of the occlusal plane, the stainless steel
crowns on the first permanent molars will be replaced by
cast full-coverage restorations (Figure 5).
Six monthly follow up was done till 18 months. Six months
after the completion of treatment, no deterioration
was visible in the restorations. The Gingiva was slightly
inflamed, because of insufficient brushing.
Figure 4: (a) Postoperative clinical picture showing composite

veneering and stainless steel crowns in posterior teeth in
mandibular arch. (b) Postoperative clinical picture showing
polycarbonate crowns and stainless steel crowns in posterior
teeth in maxillary arch
DISCUSSION
Treatment of the different amelogenesis imperfecta types
depends on the specific AI type and the character of the
affected enamel. Treatments range from preventive care
using oral prophylaxis, sealants and bonding for esthetics to
extensive removable and fixed prosthetic reconstruction. The
treatment approach should ideally be developed considering
the specific AI type and underlying defect. Nowadays, there
is a range of materials used to restore the teeth that includes
the use of composite resin, polycarbonate crowns, stainless
steel crowns (SSCs), glass ionomer cement and functional
maintenance dispositives to restore a mutilated dentition.7
In most cases, full coverage restorations are preferable for
posterior primary teeth due to the extensive loss of enamel
and also to prevent further loss of tooth structure.8 In
primary and the early mixed dentition, stainless steel crowns
prove to be the most effective type of restoration.7,8
The successful management of amelogenesis imperfecta
during childhood requires the cooperation and motivation
of both the patient and parents need to be fully assessed
before a definitive treatment plan is formulated.9 Usually,
the treatment will extend over many years and long term
success will depend on regular attendances for restorative
procedures and the maintenance of a high level of oral care.
Frequent topical fluoride applications and dietary control
are strongly recommended to prevent caries. Plaque
retention and calculus formation resulting from the rough
enamel surfaces necessitate high levels of oral health
care.10 The exposed dentin can be sensitive to such stimuli
as sweet, hot and cold; topical fluoride applications can
control this until definitive restorations can be placed.
The newly available Tooth Mousse (GC) which contains
Recaldent CPP-ACP may prove useful in this regard.
Scott H Rosenblum treated a 13-year-old with full coverage
stainless steel crowns on the molars with an increase in
vertical dimension and stainless steel crowns with veneer
phasing on the anterior teeth.11 In adolescents, porcelain
veneers are also likely to be useful; however their use with
amelogenesisimperfecta has not been extensively reported.
Porcelain jacket crowns which provide esthetic permanent
restorations, have reportedly been successful in affected
adults, but their use in young patients is contraindicated
because of the presence of large pulp chamber and
the likely need for frequent replacement due to passive
eruption.12
CONCLUSION
Figure 5: The post-operative panoramic radiograph after
restorations of all the erupted teeth and extraction of the
retained root stumps
The psychosocial effects of amelogenesis imperfecta

on affected individuals are significant.6 Although there
58
are technical difficulties associated with performing

extensive restorative care in the mixed dentition, they are
outweighed by the psychosocial benefits to the affected
child. Nevertheless, preserving as much tooth structure
as possible is highly desirable because the restorations
will need to be replaced several times during adulthood.12
Alderd
MJ,
Crawford
PJ,
Savarirayan
R.
Amelogenesis
imperfecta - classification and catalogue for the 21st century. Oral Dis.
2003;9:19-23.
Witkop CJ Jr. Amelogenesis imperfect, dentinogenesis imperfect and dentin
dysplasia revisited: Problem in classification. J Oral Pathol. 1988;17:547-53.
Bckman B. Amelogenesis imperfecta - clinical manifestations in 51
families in a northern Swedish county. Scand J Dent Res. 1988;96:505-16.
BW Neville, DD Douglass, CM Allen, JE Bouquot. Abnormalities of teeth.
In: Oral and Maxillofacial Pathology. Elsevier, Philadelphia, Pa, US; 2004.
p. 89-94.
2.
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Markovic D, Petrovic B, Peric T. Case series: Clinical findings and oral

rehabilitation of patients with amelogenesis imperfecta. European Archives
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Coffield KD, Philips C, Brady M, Roberts MW, Strauss RP, Wright T.
The psychosocial impact of developmental dental defects in people with
hereditary amelogenesis imperfecta. J Am Dent Assoc.2005;136: 620-630.
Bouvier D, Duprez JP, Bois D. Rehabilitation of young patients with
amelogenesis imperfect: a report of two cases. ASDC J Dent Child.
1996;63:443-7.
Randall RC. Literature review for AAPD: preformed metal crowns for
primary and permanent molar teeth. Consensus Conference on Pediatric
Restorative Dentistry. San Antonio, Texas, Apr 15-16; 2002.
Bedi R. The management of children with amelogenesis imperfecta. Rest
Dent 1989;5: 28-34.
Seow KW. Clinical diagnosis and management strategies of amelogenesis
imperfecta variants. Pediatr Dent 1993;15: 384-393.
Scott H. Rosenblum. Restorative and orthodontic treatment of an
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Law Kwok-Tung, Nigel M. King.The restorative management of
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How to cite this article: Ruby Kharkwal. "Dental Rehabilitation of Amelogenesis Imperfecta in the Mixed Dentition". International Journal
of Scientific Study. 2014;1(6):56-59.
Source of Support: Nil, Conflict of Interest: None declared.
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Cas e R e po r t

Dental Rehabilitation of Amelogenesis Imperfecta

MDS-Pediatric and Preventive Dentistry, Private Practioner

Well-mineralized enamel but

According to the literature, AI patients, regardless of subtype,

The treatment of patients with AI should be planned

Kharkwal: Dental Rehabilitation of Amelogenesis Imperfecta in the Mixed Dentition

sensitivity. A detailed medical, dental, and social history was

The treatment objectives were to improve the esthetics,

Figure 2: The radiographic appearance of the dentition

completely resorbed roots and pulpally involved 85 and 75.

Figure 3: Postoperative clinical picture showing esthetic

International Journal of Scientific Study | March 2014 | Vol 1 | Issue 6

Kharkwal: Dental Rehabilitation of Amelogenesis Imperfecta in the Mixed Dentition

performed on the maxillary permanent incisors prior to

Figure 4: (a) Postoperative clinical picture showing composite

International Journal of Scientific Study | March 2014 | Vol 1 | Issue 6

The psychosocial effects of amelogenesis imperfecta

Kharkwal: Dental Rehabilitation of Amelogenesis Imperfecta in the Mixed Dentition

are technical difficulties associated with performing

Markovic D, Petrovic B, Peric T. Case series: Clinical findings and oral

International Journal of Scientific Study | March 2014 | Vol 1 | Issue 6

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