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Dentistry
ing fun ctio n and esthetics in a patient w ith
, PhDVFsun zer, DDS, PhD^
Am elogene sis Imperfecta is a hereditary disorder that affects enam el on primary and permanent teeth.
It IS a rare dental disease but represents a majcr restorative chalienge fcr the dentist. A 14-year-old bey
presented with se nsitive, discclored . and m uti lated teeth and a decreased vertical dimension of occlusion.
The aim of treatment was to reduce dental sensitivity, to restore esthetics, and to correct the vertical
dimens ion of occlusion. To modify the occlusion, and to protect the denfin from chemical and fhermal
attacks, nickel-chrome onlays were placed on the molars. To improve the esthetics of the incisors and
prem olars, resin comp osite restorations w ere applie d. The patient was regularly recalled during the
postoperative period- Radiographic and clinical examinations 10 months posttreatment revealed no
evidence of disorders associated w ith the restored teeth or their supporting structures Quintessence Int
2002:33:199-204)
Key words amelogenssis imperfecta, metal onlay, resin composite restoration, tooth discoloration,
tooth sensitivity, X-linke d recessive hype matu ration
he term melogenesis imperfect (AI) has been
defined to include a variety of genetically deter-
These anomal ies can be c lass i f ied as
hypocalcified, hypoplastic, or hypomature based on
clinical and radiographie appearance, histologie
appea rance, and mode of inheritance (Table
l). * ^
Their essential gross features dist inguish the
hypoplastic and hypocalcified types: In hypoplastic
forms, the enamel does not develop to its normal
thickness; in the hypocalcified forms, enamel thick-
ness on newly erupted teeth closely approaches that of
normal teeth, but the enamel is soft, friable, and can
easily be removed from the denfin. In contrast to the
hypoplasfic types, the hypomaturation types develop
enamel of normal thickness. The hypcmaturation
forms differ from hypocalcification in that the enamel
is harder, with a mottled opaque white to yellow-
brown or red-brown color, and tends to chip from the
underlying dentin rather than to wear away.'' '
stant Pro fess or Dep artme nt of Ope rative D entist iy, University of
essor, Deparlme rit of Operativ e Den tistry. University of Selu k, Fa culty
y. K onya, Turkey.
CASE REPORT
xamination and diagnosis
A 14-year-old boy was referred for treatment of gross
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TA BL E Clinical atid radiograp hie appe aranc e of am eloge nesis i tnperfecta^
Inheritance
Hypoplastic
utosomat
dominant
utosomai
recessive
linked
dominant
Subtype
Pitted
Local
Smooth
Rough
Rough
Smooth
male
Smooth
female
Hypomaturation
iinked
recessive
uotsomai
recessive
Hypocalcif iei:
utosomal
dominant
Male
Female
Pigmented
Color
Ye How-white
Ye How-white
Yellow
Ye How-white
to white
Yellow
Yellow-brown
Yellow
White;
darkens
with age
Yellow
Brown;
stains deep
White to
honey
Enamel
thickness
Normal
Normal
One quarter to
one eighth of normal
One Quarter of
normal
Nearly absent
Thin
Normal
and thin
Near normal
Normal
Normal
Normal
Enamel
hardness
Normal
Normal
Normal but
may abrade
Chips from
dentin
Abrades easily
Abrades easily
Soft; abrades
Soft; abrades
Chips easily
Soft; cheesy
Clinical
appearance
Pin-points in random.
multiple teeth
Pits or depress ions.
usually bucea Hy.
linear horizontal
Thin glossy general;
teeth do not contact
Rough
granular surface;
teeth do not contact
Rough granular surface;
occasionally missing teeth
Smooth shiny thin;
teeth do not contact
Vertical bands ot
normal enamel
between hypoplastic
fVlottied enamel which
darkens; posterior
cervical less affected
Vertical bands of
normal enamel between
abnormal; posterior
cervical less affected
Shiny smooth.
dark enamel
Soft cheesy enamel;
can be removed with
a prophylaxis
Radiographie
appearance
fvlild lucen cy in dee
Mild lucency in dee
Thin
opaque enam
normal contrast to
Thin opaque enam
normal contrast to
Enamei not eviden
Thin opaque enam
outline; normal con
to dentin
Vertical radiolucent
Enamel same
radiodensity as de
Enamel same
radiodensjty as de
Enamel same
radio density as de
Enamel same as o
radiodensity than d
detailed medical, dental, and social history was
obtained. The patient was examined dentally and
medically. Photographs and dentai and skull radio-
graphs were obtained.
Tissue loss affected all teeth. The enamel layer was
very thin and yellow-brown, and the cuspal structure
was com pletely absent (Figs 1 and 2 ). The molars w ere
most severely affected. However, the clinical appear-
Periapical and panoramic radiographs revea
loss of enamel, especially on the occlusal surf
posterior teeth. The pulp chambers and root
were abnormally large. The approximal enamel
teeth appeared to have the same radioden
dentin (Fig 3).
The p atient s occlusal vertical dimension a
vertical dimension were assessed. The intero
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ig uc c ijs al views. Molar teeth are highly affected
2 Lateral view. Enamel is most affecte d in the
ig
3 Pahoramic radio grapti. The radiod en sities ot enamel and dentin aresimi
erior and posterior o cclu sal third. iar.
ifion the molars were in a Class I relationship but
anterior teeth w ere in an edge-to-edge relationship.
hygiene was no t satisfactory and there was
ence of gingivifis. Moreover because of the poor
ance of the teeth and their sensitivity the young
e of similar abnorm alities in his family includ ing
possible to examine other family members. He said
that his mother and grandfather had partial prostheses
and crowns hecause of the loss of many teeth at early
ages. Thus it was concluded that this patient probably
suffered from a type of X-lirdied recessive hypomatu-
ration AI.
re tment
treatment plan was drawn up with the following
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Fig 4 Niciei-clirome oniays at ceme nlation
Fig 5 Anterior view of he teeth in occiusion 10 montlis after
treatment.
vertical dimension of occlusion, improving the esthet-
ics,
and restoring the masticatory function. The
poorly calcified enamel, abnormal size of the pulp
chambers, short clinical crowns, tight interproximal
contact, and small posterior embrasure areas were
considered to be complicating factors. The rehabilita-
tion of the dentition in a young teenager with AI
should be considered from the point view of the
development of the teeth, the health of the periodon-
tal tissues, and the mandibular and maxillary growth
potential.
The patient was informed of the diagnosis and all
the treatment modalities were discussed with him. He
the molar region and direct resin composite r
tions in incisor and premolar teeth. These ma
were chosen hecause they cost less than ce
restorations and with the hope that they would
esthetic and functional rehabilitation until the
could cover the cost of porcelain restorations.
After the molar teeth were prepared for
restorations, impressions were made witb pol
siloxane impression material in stock trays. An
occlusal record was also taken. The occlusal v
dimension was increased 2 mm at the incisors.
After casting was completed, the onlays wer
on tbe dies, and the accuracy of the fit and occ
was assessed. Following polishing, the inner su
of onlay were sand blasted with 50-iim alum
oxide heads to maximize surface area and to
oughly clean and degrease them prior to cemen
The teeth were isolated with cotton rolls and
volume suction and cleaned witb a siurry of p
and water. After the teeth were rinsed and thor
dried, alloy primer and ED primer of Pana
Panavia F, Kuraray) were applied to the too
inner surfaces of onlays. The onlays were then
with a layer of a dual-cure dental adhesive
Panavia F) and seated firmly with finger pr
Excess material was removed with a small br
curing light was applied from all directions
achieve optimal hardening at the cavosurface m
and the margins of the restorations were isolate
Oxyguard {Kuraray). Figure 4 shows the seat
finished onlays after luting procedu res.
The defects of maxillary and mandibular in
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eating habits were established.
e slightly inflamed, because of insufficient brush-
. Radiographic exam ination revealed no evidence
DISCUSSION
tible to staining from coffee,
and ha s a radioden sity similar to that of den tin.
Historically, patients with AI have been treated
Because of tremendous advances in the field of
y it is today possible to restore function and
lete crowns for the m anagem ent of AI.
The first stage of provisional treatment should be
old enough to coop era t e during t rea tm en t .
Esthetics and function in patients with AI are
in their patients with AI. To reestablish full occlusion
in their patients, they used overdenture prostheses and
then crowns.
The use of supraoccluding cast restorations has
been shown to be a successful alternative method of
managing the developing dentition in patients with AI.
Hu nte r and Stone,- managing Al in a 9-year-old boy,
used supraoccluding cobalt-chrome onlays. The place-
ment of supraoccluding restorations increases tbe ver-
tical dimension of occlusion. Tbese restorations are
used before the teeth are fully erupted. The use of the
cast restorations hoth controls sensitivity and protects
and preserves tooth structure.^
The same advantages were obtained in tbe present
case. Cast nickel-chrome onlays were placed on the
seven permanent molars to stahihze the occlusion, to
halt attrition, and to decrease sensitivity. Because the
sensitivity was confined to the occlusai surfaces, par-
tial coverage was considered adequate, and little
reduction of the occlusal and axial surfaces was neces-
sary. Because of changes in the occlusal vertical
dimension, occlusion was damaged in the anterior
region. Ten months after treatment, however, anterior
occlusion had reached its normal position.
In some types of AI, the pafient's enamel not only
is thin but also may display abnormal mineral content
Tbe axial surfaces may be chalky, weak, and highly
susceptible to carious breakdown. Sucb teeth require
complete coverage with preformed crowns until preci-
sion cast crowns can be provided in the patient's late
teens or early adulthood.
Tulga^ applied resin composite restorations for
anterior teeth and stainless steel prefabricated crowns
for premolars to 10-year-old children with Al as provi-
sional treatment. In the present case, the treatment
was completed with resin composite together with
metal onlays. Venezie et aP' reported that difficulty in
honding to hypomineralized enamel can significantly
l imi t the restora t ive and or thodont ic t rea tment
options for patients with AI. In their study, they found
that pretreating the tooth surface with 5 sodium
hypochlorite improved bonding of an orthodontic
bracket to enamel affected hy AI.
CONCLUSION
The treatment plan for cases of amelogenesis Imper-
fecta is related to m any factors: the age of the patient,
the socioeconomic status of the patient, the type and
severity of the disorder, and the intraoral situation at
the time the treatment is planned. Early initiation of
treatment is important before severe tissue destruc-
tion can occur. In the present case, a 14-year-old boy
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dentition was treated with nickel-chrome onlays and
resin composite restorations to alleviate sensitivity,
improve estbefics, and restore function.
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