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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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