37. Cleft Lip, Anomalies of Face, Palate Jaw, Teeth

8/13/2019 37. Cleft Lip, Anomalies of Face, Palate Jaw, Teeth

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37 Cleft lip & palate. Developmental anomalies of the face,

palate, jaws & teeth

RICHARD P JUNIPER and WILLIAM P SMITH

Cleft lip and palate

Clefts of the lip, alveols, ha!d and soft palate a!e the "ost #o""on #on$enital

a%no!"alities of the o!ofa#ial st!#t!es& The' f!e(entl' o##! as isolated defo!"ities

%t #an %e asso#iated )ith othe! "edi#al #onditions pa!ti#la!l' #on$enital hea!t

disease& The' a!e also an asso#iated feat!e in ove! *++ !e#o$nied s'nd!o"es&

All #hild!en %o!n )ith a #left lip and palate need tho!o$h paediat!i# assess"ent to

e-#lde othe! #on$enital a%no!"alities& In #e!tain #i!#"stan#es $eneti# #onsellin$

"st %e so$ht if a s'nd!o"e is sspe#ted&

In#iden#eThe in#iden#e of #left lip and palate is one in .++ /01.++2 live %i!ths, and 010+++ live

%i!ths fo! isolated #left palate& The in#iden#e in#!eases in 3!iental $!ops /014++2 and

de#!eases in the %la#5 poplation /016+++2& The hi$hest in#iden#e !epo!ted fo! #left lip

and palate o##!s in the Native A"e!i#an t!i%es of Montana, USA /0167.2&

Altho$h #left lip and palate is an e-t!e"el' dive!se and va!ia%le #on$enital

a%no!"alit', seve!al distin#t s%$!ops e-ist, na"el' #left lip )ith8)ithot #left

palate /CL8P2, #left palate alone /CP2 and s%"#os #left palate/SMCP2&

The t'pi#al dist!i%tion of #left t'pes is1

9#left lip alone : 04 pe! #ent;

9#left lip and palate


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Classifi#ation

An' #lassifi#ation fo! s#h a dive!se and va!ied #ondition as #left lip and palate needs

to %e si"ple, #on#ise, fle-i%le, e-a#t %t $!aphi#& It "st %e sita%le fo!

#o"pte!isation %t des#!iptive and "o!pholo$i#al& An e-a"ple of s#h a

#lassifi#ation is the LAHSHAL s'ste" )hi#h is a%le to des#!i%e site, sie, e-tent as

)ell as t'pe of #left /@i$& *7&02&Co"plete #lefts of the lip, alveols, and ha!d and soft palate a!e desi$nated #apital L,

A, H and 4, !espe#tivel', )he!eas in#o"plete #lefts a!e !e#o!ded in s"all lette!s;

"i#!ofo!" #lefts a!e do#"ented )ith aste!is5s& Hen#e, LAHSHAL is the anato"i#al

pa!aph!ase of a #o"plete %ilate!al #left lip and palate& Anothe! e-a"ple, lahSh,

!ep!esents an in#o"plete !i$ht nilate!al #left lip and alveols )ith a #o"plete #left of

soft palate e-tendin$ pa!tl' on to the ha!d palate&

P!i"a!' "ana$e"ent

Antenatal dia$nosis

An antenatal dia$nosis of #left lip, )hethe! nilate!al o! %ilate!al, is possi%le %'lt!asond s#an afte! 0 )ee5s of $estation& Isolated #left palate #annot %e dia$nosed

on an antenatal s#an& When an antenatal dia$nosis is #onfi!"ed, !efe!!al to a #left

s!$eon is app!op!iate fo! #onsellin$ to alla' fea!s& Photo$!aphs of #left lip sho)n to

pa!ents B%efo!e and afte!? s!$e!' a!e invala%le& Int!od#tion to a pa!ent sppo!t

$!op and "eetin$ pa!ents of a #hild )ith a si"ila! #left )ho has nde!$one s!$e!'

"a' also %e e-t!e"el' helpfl&

@eedin$

Most %a%ies %o!n )ith #left lip and palate feed )ell and th!ive p!ovided app!op!iate

advi#e is $iven and sppo!t availa%le& So"e "othe!s a!e s##essfl in %!eastfeedin$,

pa!ti#la!l' )hen the #left is in#o"plete and #onfined to the lip& >ood feedin$

patte!ns #an %e esta%lished )ith soft %ottles /e&$& Mead Johnson2 and "odified teats

/o!thodonti#, N'52& Si"ple "eas!es, s#h as enla!$in$ the hole in the teat, often

sffi#e& @eedin$ plates, #onst!#ted f!o" a dental i"p!ession of the ppe! a), a!e

!a!el' ne#essa!' to i"p!ove feedin$& So"e %a%ies a!e p!ovided )ith an a#tive plate

)hi#h ai"s not onl' to i"p!ove feedin$ %t also !ed#e the )idth of the #left lip and

palate p!io! to s!$e!'& The lon$


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o! nea!


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S!$i#al te#hni(es

The!e have %een "an' diffe!ent s!$i#al te#hni(es and se(en#es advo#ated in #left

lip and palate "ana$e"ent& Cleft lip !epai! is #o""onl' pe!fo!"ed %et)een * and .

"onths of a$e, )he!eas #left palate !epai! is f!e(entl' pe!fo!"ed %et)een . and 0

"onths&

The Delai!e te#hni(e and se(en#e /Ta%le *7&02 is one of "an' !e$i"es #!!entl'p!a#tised&

Cleft lip s!$e!'

S5in in#isions /@i$ *7&7 and @i$& *7&2 a!e developed to !esto!e displa#ed tisses

in#ldin$ s5in and #a!tila$e to thei! no!"al position, )hilst $ainin$ a##ess to the

fa#ial, nasal and lip "s#lat!e&

Ms#la! #ontinit' is a#hieved %' s%pe!iosteal nde!"inin$ ove! the ante!io!

"a-illa& Nasola%ial "s#les a!e an#ho!ed to the p!e"a-illa )ith non

st!es& 3%li(e "s#les of o!%i#la!is o!is a!e st!ed to the %ase of the ante!io!

nasal spine and #a!tila$inos nasal sept"& Clos!e of the #left lip is #o"pleted %'

st!in$ the ho!iontal fi%!es of o!%i#la!is o!is to a#hieve a fn#tionin$ o!al sphin#te!

/@i$ *7& and @i$ *7&0+2&Cleft palate s!$e!'

Cleft palate #los!e #an %e a#hieved %' one< o! t)o


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Co""on spee#h p!o%le"s asso#iated )ith #left lip and palate a!e1

9 Felopha!'n$eal in#o"peten#e : this is asso#iated )ith in#!eased nasal ai!flo)

and !esonan#e p!od#in$ a nasal o! Bh'pe!nasal? (alit' to spee#h& It f!e(entl'

!efle#ts poo! fn#tion of soft palate asso#iated )ith inade(ate "s#le !epai!&

9 A!ti#lation p!o%le"s : these eithe! a!ise as a #o"pensato!' "e#hanis" to

ove!#o"e velopha!'n$eal in#o"peten#e o!, less #o""onl', a!e de to a)8dental ando##lsal a%no!"alities&

Investi$ation %'1

9 videoflo!os#op'

9nasal ai!flo) stdies /ae!ophonos#op'2

9nasendos#op'

is helpfl in definin$ the e-a#t "e#hanis" of the p!o%le" )hi#h aids "ana$e"ent&

Spee#h p!o%le"s a!e "ana$ed %'1

9spee#h and lan$a$e the!ap';

9se#onda!' palatal s!$e!'1

: int!avela! veloplast' /"s#la! !e#onst!#tion of soft palate2;

: pha!'n$oplast';9spee#h


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and spee#h !e#o!din$ a!e indispensa%le, and pe!"it !e$la! adit and i"p!oved

ot#o"es&

Developmental abnormalities of the face, palate, jaws and teeth

Treacher Collins syndrome/@i$& *7&0=2

T!ea#he! Collins? s'nd!o"e o! "andi%la! fa#ial d'sostosis has a va!ia%le

p!esentation& The #a!dinal feat!es a!e of an anti


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An$le, an A"e!i#an o!thodontist, pt the o##lsion of the teeth into th!ee #lasses1

9#lass I : no!"al !elationship of the lo)e! to the ppe! teeth in the "ola! a!ea;

9#lass II : the lo)e! "ola! teeth a!e pla#ed poste!io! to the no!"al position;

9#lass III : the poste!io! "ola! teeth a!e pla#ed ante!io! to the no!"al position&

This #lassifi#ation has %een e-tended to in#lde the fa#ial s5eleton s#h that1

9s5eletal II : s"all "andi%le is pla#ed poste!io! to the no!"al position in !elation tothe "a-illa;

9s5eletal III : "andi%le is pla#ed ante!io! to the no!"s position !elative to the

"a-illa&

It is a helpfl des#!iption of a fa#ial s5eletal defo!"it', %t it onl' des#!i%es the

"andi%le in !elation to the "a-illa )hi#h itself "a' %e h'pe!plasti# o! h'poplasti#&

Ths, the& BHaps%!$ a)? )hi#h des#!i%ed the fa"ilial feat!es of the !o'al hoses of

E!ope in the nineteenth #ent!' des#!i%es la!$e lo)e! a), a h'pe!plasti# "andi%le,

)hi#h is f!e(entl' asso#iated )ith a h'poplasti# "a-illa )hi#h a##entates the

defo!"it' in the "andi%le&

The "ost #o""on defo!"it' of the fa#ial s5eleton in the Ca#asian is the h'poplasti#

"andi%le : s5eletal #lass II


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p!in#iples of t!eat"ent a!e to #o!!e#t defo!"ities f!o" the #!ani" do)n)a!ds& Ths,

in C!oon?s disease /see a%ove2 the #!aniofa#ial s!$eon )ith the ne!os!$eon )ill

#o!!e#t the #!anial defo!"it' in the fi!st 6:* 'ea!s of life, and )ill !et!n to deal )ith

the "idfa#e %' the a$e of . o! 7 'ea!s, often in #o


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epitheli" in#lded in l'"phoid a$$!e$ates d!in$ e"%!'o$enesis& The #!!ent

no"en#lat!e is l'"phoepithelial #'st&

9 3!al to!i /@i$ *7&0 and @i$ *7&6+2 : it is n#e!tain as to )hethe! these a!e t!e

develop"ental a%no!"alities& The' o##! at t)o "ao! sites, the "ost #o""on lies on

the "edial side of the "andi%la! alveols in the !e$ion of the p!e"ola! teeth& Less

f!e(entl' "idline palatal to!i o##!, a$ain in line )ith the p!e"ola! teeth& These a!edense, %on' s)ellin$s that #ase no p!o%le" apa!t f!o" o##asional l#e!ation,

pa!ti#la!l' if dent!es a!e to %e )o!n& The' a!e onl' !e"oved if the' #ase diffi#lt'

in the )ea!in$ of dent!es&

9 C'sts in the th'!o$lossal t!a#t : the th'!oid $land develops as an eva$ination of the

epitheli" at the n#tion of the ante!io! t)oene!all' the' a!e s"alle!


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than the no!"al teeth in the sa"e a!ea and seldo" develop little "o!e than a #!o)n

and a vesti$ial !oot& The' "a' %e "ltiple& In the in#iso! !e$ion the' "a' %e

!esponsi%le fo! the fail!e of e!ption of ada#ent pe!"anent teeth, pa!ti#la!l' the

#ent!al and late!al in#iso!s and #anine& The' $ene!all' lie palatal to thei! no!"al

#onte!pa!ts& The' have to %e !e"oved to allo) e!ption of the pe!"anent teeth to

o##!& Ra!el', in the thi!d "ola! !e$ion, denti$e!os #'sts "a' develop a!ond the#!o)n& These a!e #ha!a#te!ised %' a !adio$!aphi# appea!an#e of a #'st envelopin$ the

#!o)n of the tooth that is displa#ed&

3donto"es a!e a$$!e$ations of tooth


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!iens, +& /0+2 Do#"entation of #left lip, alveols and palate& In Mana$e"ent of

Cleft Lip and Palate /eds J& a!da#h and H&L& Mo!!is2, W& Sande!s, Philadelphia,

PA, p& 067&

Ma!5s, A&@& and Delai!e, J& /0*2 @n#tional p!i"a!' #los!e of #left lip& !itish

Jo!nal of 3!al Ma-illo fa#ial S!$e!', *0,60&

Ma!5s, A&@&, S"ith, WP and Delai!e, J& /0*2 P!i"a!' #los!e of #left palate1 afn#tional app!oa#h& !itish Jo!nal of 3!al Ma-illofa#ial S!$e!', *0, 70&

Ni#ola, P& /0*2 The o!%i#la!is otis "s#le1 a fn#tional app!oa#h to its !epai! in

the #left lip& !itish Jo!nal of Plasti# S!$e!', *., 0=&

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37. Cleft Lip, Anomalies of Face, Palate Jaw, Teeth