Prosthodontic Management of a Child With Ectodermal Dysplasia

7/28/2019 Prosthodontic Management of a Child With Ectodermal Dysplasia

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ProsthodonticManagementofaChildwithEctodermalDysplasia:

ACaseReport

AkshayBhargavaArunSharmaSachetPopliRenuBhargava

JournalofIndianProsthodonticSociety2010


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Introduction

Ectodermal dysplasia is hereditary disorder associated with

dysplasia of tissues ofectodermalorigin primarily nail, teeth,

hairandskinandoccasionallydysplasiaofmesodermderived

tissues.

Types

Hidrotic

Hypohidrotic


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Inboth types teethandhair aresimilarlyaffectedbut

manifestations in nails and sweat glands and

hereditarypatterntendtodiffer.


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The X linked hypohidrotic form or Christ Siemens

syndromeischaracterizedbyclinicaltriadof

Hypohidrosis

Hypotrichosis

Hypodontia.

Hidrotic form is inherited as an autosomaldominant trait

and affects teeth, hair and nails but usually spares the

sweatglands.


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Prosthodonticrehabilitationisofgreatimportancetopatient

with Ectodermal dysplasia for functional, physiologic and

psychosocialreasons


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Case Report

A5-year-oldgirl child reported todental

clinic, with reference from her

pediatricianforhavingprobleminmixing

with friends andmoving around socially

(with suggestive signs of clinical

depression),alsodifficultyineatingfood

duetoabsenceofteeth.


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Diagnosed with Anhidrotic Ectodermal

Dysplasiaattheageoftwo.

Familyhistorywasnegative.

Child was moderately built with

hypotrichosis,scarceeyebrowsandscarce

eyelashes, dry anhidrotic skin, depressed

nasalbridge,thicklips,darkpigmentedskin

around periorbital area and nose, facial

heightwasreduced.


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Childwasveryshyanduncooperative.

Parents reported that child doesnt sweat and

herlipsandtongueremaindryinallclimates.

Therewashowevernoeffectonthenails


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Intraoral examination revealed presence of partially

eruptedtwopegshapedteethinregionof53and83

Edentulousridgewasatrophicwithdecreasedheight.

Palatewasshallow, oralmucosawasnormal anddry

duetolesssaliva,tonguewasrelativelylarge.


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OPG showed presence of an impacted tooth in

relationto12regionanderupted53,83


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Prosthodontic Management Preliminaryimpressionwasmade

Denture(maxillaryandmandibular)wasdecidedtobemade

initiallywithoutanymodificationofexistingtwoteethinorder

togainthechildsconfidence.

Special tray with uniform 2 mm full arch wax spacer

coveringnaturalteethwasprepared.


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Peripheral border seal was established and secondary

impressionsweremadewithnonEugenolZincOxideimpression

paste.

Master casts were made and occlusal rims with temporary

denturebasewerefabricated.

Jawrelationsweredonebymanuallyguidingmandibleinto

centric.

Lowerteethsetofsizelargesetwastrimmedtoshapelike

maxillaryanteriors.


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loweranteriorsweretrimmedsimilarlyfromsmallsize

lowerarchteethset.

Trial was done and dentures were fabricated using

heatpolymerizingacrylic.

Itwasnotpossibletogivelongclinicalsittings,aschild

wasnotverycooperativealltime.


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Impressiontechniqueandjawrelationweredecidedtobe

improved subsequent to fabrication of denture by relining

withsoftpermanentrelinerratherthancontinuewithclinical

sittingsandlosecooperationofchild


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Therefore to improve fit of dentures tissue surface,

maxillary and mandibular dentures were relined with

tissueconditionermaterial.

Patientwasrecalledafter2daysandthedentureswere

relined


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There was improved speech and a marked

improvement in social activities of patient with

provisionaldenture.

Childwasrecalledafterevery3months.


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After 6 months dentures especially mandibular

denturewasfoundtobebecomingillfitting.

In9thmonthpatientwasunabletowearlowerdenture

andhadstoppedwearingdentureduringeating.

Clinical evaluation showed that lower tooth (83) had

erupted and therefore lower denture was not being

able toseaton ridge.Therewasalsoan increase in

sizeofbothjaws.


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Itwasdecidedtofabricatenewremovablepartialdenturewith

clasp in relation to 83 for lower arch, and maxillary

overdenture(asmadepreviously)forupperarch.

upper and lower prosthesis were fabricated in same

conventional manner as discussed earlier, except medium

phase.

Polyether impression material was used to make final

impressions.


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Discussion

Treatment for a patient with ectodermal dysplasia varies

and generally depends on childs age, dental agenesis,

degreeofmalformationofteeth,growthanddevelopmentof

stomatognathic system of the patient and patients

motivation


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Prosthodontictreatmentforchildrenwithectodermal

dysplasia includes removable partial denture or

completedenture,overdentureandimplants.These

approaches may be used either individually or in

combinationtoprovideoptimalresults.


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Complete denture prosthesis given to patient alters alveolar

height, provides a better musculocutaneous profile and brings

aboutsignificantimprovementinmastication,esthetics,phonetic

functionandpsychologicalsupport.

usual treatment for ectodermal dysplasia focuses on series of

complete or partial denture during years when growth of

dentofacial region is taking place and definite rehabilitation

followingcompletionofjawgrowth.


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Earlyprosthetictreatmentisgenerallyrecommendedfromageof

5yearsanddenturescanbefabricatedasearlyas34yearsof

ageforcooperativechildren.

TillandMarquesrecommendedthataninitialprosthesisshouldbe

delivered before child begins school so that child has normal

appearanceandtimetoadapttoprosthesis.

Thisearlyrestorationoffacialappearanceisessentialfornormal

psychologicaldevelopment.


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problemsassociatedwithearlyplacementofcompletedenture

aremainlyassociatedwithperiodicadjustmentduetogrowth

changes and difficulties in achieving good retention and

stability.

Difficulties in achieving adequate resistance to lateral and

anteroposterior displacement of the denture in hypohidrotic

ectodermal dysplasia patient are due to dryness of oral

mucosa and underdevelopmentofmaxillary tuberosities and

alveolarridges.


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Whenteetharepresentinmouth,overdenturesaremost

desirabletreatmentoption.

Overdenturehasanaddedadvantage,thattheypreserve

alveolarbone.

Asresultofcontinuinggrowthanddevelopment,periodic

prosthesismodificationorreplacementisneeded.


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FixedProsthodontictreatmentisseldomusedbecauseof

decreasednumber ofabutment, andmoreoverpatient is

tooyoung.

UseofFPDswithrigidconnectorsshouldbeavoidedasit

mayinterferewithjawgrowth.

Inyoungpatients, individualcrownrestorationanddirect

composite restorations have been used in combination

withremovablepartialdenture


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For adult patients with ectodermal dysplasia, dental

implants are treatment of choice because growth has

stabilizedandimplantscanbeusedtosupport,retain

andstabilizeprosthesis.

For using implants in young patients, timing of

treatment is of utmost importance to avoid possible

complicationsthatmayresultfromjawgrowth.


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In this case as child was 5 years old when first reported to

clinic,implantscouldnotbeconsideredastreatmentofchoice.

Itwasdecidedtofabricateanoverlaydenture.After6monthsit

wasobservedthateruptionof83andtheincreaseinjawsize

leadtolossofretentioninmandibulardenture,whileonlythere

was increase in jaw size in maxilla, and marginal loss in

retention and stability of the upper denture,child had no

complaintinmaxillarydenture.


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Treatment not only improvedpatients functional and

estheticstatusbutalsoimprovedpsychologicalhealth

andsociallifesoimportantforcompletedevelopment

ofchild.


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Conclusion

Basisphilosophyistorestoreestheticandfunctionofchildat

present time, till complete growth of jaw has occurred. The

finaltreatmentwillbeimplantsupportedprosthesiswithbone

augmentationasandwhennecessary.


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Documents

Prosthodontic Management of a Child With Ectodermal Dysplasia