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Sans Maaysana 42(1)(2013): 8588

Cnca Manfstatn f Bparcdntc (BCD) Syndrm: A Cas R(Manfstas Knka Sndrm Bfarkadntk (BCD): Sat Tnjaan Ks)

R.NAvASheillA ReTNA* & S.N.M.P. SoCKAliNgAM

ABSTRACT

BCD syndrome is a rare birth syndrome characterised by cleft lip and palate, euryblepharon with/without limb anomalies,

ectropion of lower eyelids and hypertelorism. The dental anomalies include cone shaped microdontic teeth and oligodontia.

Different combinations of these signs have been found sporadically in patients. This syndrome is also known as Elschnigs

syndrome. By at large, patients with this syndrome have no potentially fatal anomalies. Their growth and development

were usually normal. This case review described the clinical manifestation and oral ndings of a patient with BCD

syndrome. The dental need of the patient was outlined.

Keywords: Ectropion; Elsching syndrome; euryblepharon; lagophthalmia; oligodontia

ABSTRAK

Sindrom BCD adalah satu sindrom kelahiran yang jarang dijumpai dan mempunyai ciri-ciri seperti rekahan bibir dan

lelangit, euryblepharon dengan/tanpa anomali pada lengan tangan dan kaki, ektropion kelopak mata bawah dan

hipertelorisme. Anomali pergigian termasuk kehadiran gigi-gigi mikrodontik yang berbentuk kon dan oligodontia.

Akan tetapi, kombinasi berlainan daripada ciri-ciri yang dinyatakan boleh juga dijumpai pada segelintir pesakit secara

sporadik. Sindrom ini juga dikenali sebagai sindrom Elschnig. Kebiasaannya, pesakit sindrom ini jarang memiliki anomali

serius yang boleh menyebabkan kematian. Tumbesaran dan perkembangan mereka juga adalah normal. Tinjauan ini

melaporkan manifestasi klinikal serta penemuan oral pada seorang pesakit sindrom BCD. Keperluan pergigian untuk

pesakit ini juga dibincangkan.

Kata kunci: Ectropion; euryblepharon; lagophthalmia; oligodontia; sindrom Elschnig

iNTRoDuCTioN

Bparcdntc (BCD) syndrm s a rar brt

syndrm caractrzd by cft p and paat (mst ftn

batra), cn sapd mcrdntc tt, ypdnta r

dnta, ctrpn f t r yd, aptama,

yprtrsm, dstcass and rybparn. lmb

anmas sc as cndactyy and syndactyy may as

b sn. in 1912, escn dscrbd cmpcatns

t cft p and paat, ctrpn f r yd

and rybparn bt t tk sm tm fr ts t b

rcnzd as a syndrm. grn t a.(1996) identiedts syndrm as an atsma dmnant dsrdr. eyd

anmas dstnss bparcdntc syndrm

frm tr syndrms tat as manfsts t fatrs

k cft p and paat t dnta r mcrdnta.

The clinical manifestation and oral ndings of a patient

t BCD syndrm b dscssd n ts r. T

dnta nds f ts patnt ar as tnd.

CASe Review

A 5 yar d r t mtp mssn tt and dansd

t BCD syndrm as rfrrd t s by t Padatrc

Dpartmnt f t Natna unrsty f Maaysa Mdca

Cntr (uKMMC). hr matrna randmtr cmpand f

dssatsfactn rardn r dnta apparanc. T patnt

as n a dprssd stat and as ry sy f attndn

sc d t r faca and dnta apparanc. T

patnt as ad a ry sf-stm. S s f Cns

dscnt s parnts ar nt ratd. hr mtr ad an

nntf prnancy and dry. hr tr sbns and

famy mmbrs ar nrma. Tr s n stry f faca/

dnta anmas/syndrm rnnn n t famy. S

as brn t batra cft p and paat. extraray,

t patnt prsntd t batra y anmas tat

ncd ctrpn f t r yds, aptams,yprtrsm, dstcass and rybparn (Fr 1).

hr scap ar as spars and strat. Ntr syndactyy

nr yppastc na as dtctd. Rpard batra cft

p as bsrd. S as n a Cass iii skta ratnsp

with a hypoplastic maxilla. Intraorally, there is a stula

asscatd t t prmary paat d t ntf an

f t srca rpar (Fr 2). As, t patnt prsntd

t mcrstma and as dnta and mcrdnta t

cnca sapd tt (Frs 3 and 4). Radrapcay,

an rtpantmram f ts patnt rad many

mssn prmannt tt bds t a rdcd aar

bn and a yppastc maxa (Fr5). T nta

manamnt nd prnt masrs ncdn

uoride application and oral hygiene instructions. We


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dcdd t cnstrct a par f parta dntrs fr ts

patnt snc ts as r man cncrn as as n t

prspct f mprn r asttcs, spc and fnctn

so that she could build up her condence level. Our patient

as xtrmy pasd t r n apparanc snc t

new set of partial dentures had boosted her self condence

apart frm mprn r asttcs, spc and fnctn

(Fr 6). ln trm manamnt fr ts patnt d

prbaby dmand fr bn raftn and mpants.

DiSCuSSioN

As f 2006, ny 32 cass fBCD syndrm a bn

rprtd rdd (ida t a. 2006). it s as knn as

cftn-ctrpn-cnca tt dsas snc t dscrbs

t man cnca fatrs f ts dsas. grt and

dpmnt ar nrma n a t patnts tat a bn

stdd.

Frm t tratr r, t as fnd tat tr as

n ndr prdctn n trms f ts syndrm (ida t

a. 2006). hr, tr as a pattrn f fama cass,

sstn an atsma dmnant nrtanc (Aansn

& Mcgary 1985). lps t a. (2003) ad prpsdt pssbty f nmnt f p63 and intrfrn

FiguRe 1. Apparanc f t patnt

FiguRe2. Intraoral view of the palate showing the stula

FiguRe 3. intrara f t ppr arc

FiguRe 4. intrara f t r arc

FiguRe 5. ortpantmram f t patnt


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Ratry Factr 6 (iRF6) ns n ts cndtn. it as

as bn sn tat patnts t ts syndrm d nta any fata anmas. or patnt xbtd nrma

rt and dpmnt fr r a. Tr s n stry

f t syndrm nrtanc n r famy. T pssb

nmnt f a partcar n cannt b rd t.

hr, tr as n dnc f ts rprtn n r

mdca rcrds. Prbaby t ccrrnc f ts cas may

b f spradc n natr.

Bascay, patnts t ts syndrm manfst

many y anmas, dnta anmas and mb

anmas. ey anmas ncd rybparn,

ctrpn, dstcass, yprtrsm and aptama.

Dnta anmas ar cft p and paat (mst ftnbatra), cnca tt, ypdnta r dnta and

mcrdnta. odnta as bn fnd n tr qartr

f t cass rprtd ras cnca tt a bn

ntd n ss tan af f ts patnts. ida t a. (2006)

rd dtad rprts abt dnta amn BCD

patnts n t tratr and fnd tat t ard frm a

md frm t andnta. T sts f mssn tt n md

dnta cass r say fnd adjacnt t t cft

f t as (gn-Amda t a. 1998). ida t a.

(1996) as ntd tat 2 cass ad sr dnta, and

a tt xcpt prmannt mars r symmtrcay

mssn (gn Amda t a. 1998; Martnz t a. 1987).

lmb anmas ncdn cndactyy and syndactyy

ar nt aays sn nk y anmas and dnta

anmas c s a mst t dans ts cndtn.

otr spradc symptms tat a bn rprtd n

patnts t ts dsrdr ar yptyrdsm and

mprfrat ans (lps t a. 2003). Stds a as

sn tat patnts t ts syndrm may prsnt t

spars ar (ida t a. 2006). or patnt manfstd bt

dnta and cnca tt. F tt r ntd n

ac arc by cnca and radrapc assssmnts. S

as batra cft p and paat c cncrs t

majrty f t cass rprtd n t tratr. hr ar

as as spar and strat. hr, mb anmasr nt fnd n ts patnt. Antr fatr fnd n

r patnt as mcrstma t tnnn f bt ps.

wtr ts as a tr prsntatn f ts syndrm r

an effect due to the surgical repair of cleft lip was difcult

t ascrtan.

Bsds t cnca prsntatn, r patnt prsntd

t a cmpx asttc prbm tat ndd an rnt

attntn. Snc patnt s a sc n cd, s s qt

aar f r asttcay npasn apparanc many

d t r y anmas, yppastc maxa and mtp

mssn tt. As a rst, s as tasd and sn n a

ss tan frndy by r frnds at sc. S as

startd t cmpar r apparanc t trs arnd r.

Ts as d r t bn dprssd and ftn ss aay

frm sc. hr sf-stm frtrmr affctd

r stds as s s nab t cncntrat cmpty at

sc apart frm bn absnt frm sc. Nt ny tat,

bt patnt as prbm cn fd and s nab t at

arts f fd d t t mtp mssn tt. Mst f

t tm, s s n a sft dt. in trms f spc, s asprbm prnncn crtan apabts. it as trfr

dcdd t cnstrct dntrs fr ts patnt n f r

psycca and fnctna stats. Dntr cnstrctn

b an ntrmdat tratmnt fr ts patnt snc bn

raftn and mpant pacmnt may b carrd t as a n

trm manamnt n t ftr. Drn cnstrctn f t

dntr fr ts patnt, ad t prfrm a f carf

manipulations such as using the uoride tray during the

prmary mprssn takn and as cnstrctn t r

denture rst in order to obtain the bite registration for the

cnstrctn f t ppr dntr. Ts as dn d t t

nabty f t patnt t pn t mt dy bcasf mcrstma.

CoNCluSioN

As n many syndrmc cass, t rdnta prbms

r ftn rsadd by t majr syndrmc

manfstatns. As dntsts, sdnt b aarmd t

t mr apparanc f ts patnts and sy aay frm

tm. w mst b ab t apprcat tr spca dnta

nds and prd t apprprat rmdy t tr prbm.

in t crrnt cas, nt ny mprd r apparanc,

but also psychologically had made her more condent

t f. hr, s st nd t cntn

mntrn and r appntmnts nt adtd.

ACKNowleDgeMeNT

T atrs d k t tank t patnt and r

ardan fr tr cpratn n an s t s t

cnca pctrs fr ts r.

ReFeReNCeS

Aansn, J.e. & Mcgary, B.C. 1985. Fama cftn

syndrm t ctrpn and dnta anmay-tt mb

anmas. Clin. Genet. 27: 426-429.

FiguRe6. Cmptd parta dntrs


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grn, R.J., Zr, h., Crts, M.w., wdmann, h.R.,

warbr, M., Majsk, F., gssn-Kasbac, g., Pra-

Andrsn, B. & Zacka, e. 1996. Bpar-c-dntc

(BCD) syndrm. American Journal of Medical Genetics

65: 109-112.

gn-Amda, M.l., Rdn, e.S.o., Kkts-Nakata, N.M. &

Bna-Amantn, D. 1998. Bpar-c-dntc (BCD)syndrm: Rprt n fr n patnts. American Journal

of Medical Genetics 76: 133-136.

ida, Akk., Nara, Sta., Taka, R., on, K. & ikda, N.

2006. Bpar-c-dntc (BCD) syndrm: Cas rprt.

Cleft Palate-Craniofacial Journal 43: 237-243.

lps, v.l.g.d.S., gn-Amda, M.l. & Rdn, e.S.d.o.

2003. Bpar-c-dntc (BCD) syndrm: expandn

t pntyp?American Journal of Medical Genetics 121:

266-270.

Martnz, R.B., Mnastr, A.l., Pnr, M., Frr-Maa,

N., optz, J.M. & Rynds, J.F. 1987. Cft p/paat-

dnta- syndactyy-ar atratns, a n syndrm:

R f t cndtns cmbnn cdrma dyspasa

and cft p/paat.Am. J. Med. Genet. 27: 23-31.

Dpartmnt f oprat Dntstry

Facty f Dntstry

unrst Kbansaan MaaysaJaan Raja Mda Abd Azz

50300 Kaa lmpr

Maaysa

*Crrspndn atr; ma: [email protected]

Rcd: 19 Dcmbr 2011

Accptd: 18 May 2012