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

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

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Published by sabina_grapini
37. Cleft Lip, Anomalies of Face, Palate Jaw, Teeth
37. Cleft Lip, Anomalies of Face, Palate Jaw, Teeth

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Published by: sabina_grapini on Nov 30, 2013
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37Cleft lip & palate. Developmental anomalies of the face, palate, jaws & teeth
RICHARD P JUNIPER and WILLIAM P SMITHCleft lip and palateClefts of the lip, alveols, 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$nied s'nd!o"es&All #hild!en %o!n )ith a #left lip and palate need tho!o$h paediat!i# assess"ent to e-#lde othe! #on$enital a%no!"alities& In #e!tain #i!#"stan#es $eneti# #onsellin$ "st %e so$ht if a s'nd!o"e is sspe#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 $!ops /014++2 and de#!eases in the %la#5 poplation /016+++2& The hi$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%$!ops e-ist, na"el' #left lip )ith8)ithot #left  palate /CL8P2, #left palate alone /CP2 and s%"#os #left palate/SMCP2&The t'pi#al dist!i%tion of #left t'pes is19#left lip alone : 04 pe! #ent;9#left lip and palate <<<<<<=4 pe! #ent;9isolated #left palate : =+ pe! #ent&Cleft lip8palate p!edo"inates in "ales )he!eas #left palate alone appea!s "o!e #o""on in fe"ales& In nilate!al #left lip, the defo!"it' affe#ts the left side in .+ pe! #ent of #ases&Aetiolo$'Conte"po!a!' opinion on the aetiolo$' of #left lip and palate is that #left lip and  palate /CL8P2 and isolated #left palate have a $eneti# p!edisposition and a #ont!i%to!' envi!on"ental #o"ponent& A fa"il' histo!' of #left lip and palate in )hi#h the fi!st<de$!ee !elative is affe#ted in#!eases the !is5 to one in 64 live %i!ths& >eneti# inflen#e is "o!e si$nifi#ant in #left lip8palate than #left palate alone )he!e envi!on"ental fa#to!s e-e!t a $!eate! inflen#e&Envi!on"ental fa#to!s i"pli#ated in #leftin$ in#lde "ate!nal epileps' and d!$s /e&$& ste!oids, diaepa", phen'toin2, altho$h the %enefit of antenatal foli# a#id spple"ent to p!event #left lip and palate !e"ains e(ivo#al&Altho$h "ost #lefts of the lip and palate o##! as an isolated defo!"it', the Pie!!e Ro%in se(en#e !e"ains the "ost #o""on s'nd!o"e& This s'nd!o"e #o"p!ises isolated #left palate, !et!o$nathia and a poste!io!l' displa#ed ton$e /$lossoptosis2, )hi#h is asso#iated )ith ea!l' !espi!ato!' and feedin$ diffi#lties&Isolated #left palate is "o!e #o""onl' asso#iated )ith a s'nd!o"e than #left lip8palate and #left lip alone& 3ve! 04+ s'nd!o"es a!e asso#iated )ith #left lip and  palate, altho$h Sti#5le! /ophthal"i# and "s#los5eletal a%no!"alities2, Shp!inten /#a!dia# ano"alies2, Do)n?s, Ape!t?s and T!ea#he! Collins? a!e "ost f!e(entl' en#onte!ed&
 
Classifi#ationAn' #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 $!aphi#& It "st %e sita%le fo! #o"pte!isation %t des#!iptive and "o!pholo$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, sie, e-tent as )ell as t'pe of #left /@i$& *7&02&Co"plete #lefts of the lip, alveols, and ha!d and soft palate a!e desi$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$ht nilate!al #left lip and alveols )ith a #o"plete #left of soft palate e-tendin$ pa!tl' on to the ha!d palate& P!i"a!' "ana$e"entAntenatal dia$nosisAn antenatal dia$nosis of #left lip, )hethe! nilate!al o! %ilate!al, is possi%le %' lt!asond 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! #onsellin$ to alla' fea!s& Photo$!aphs of #left lip sho)n to  pa!ents B%efo!e and afte!? s!$e!' a!e invala%le& Int!od#tion to a pa!ent sppo!t $!op and "eetin$ pa!ents of a #hild )ith a si"ila! #left )ho has nde!$one s!$e!' "a' also %e e-t!e"el' helpfl&@eedin$Most %a%ies %o!n )ith #left lip and palate feed )ell and th!ive p!ovided app!op!iate advi#e is $iven and sppo!t availa%le& So"e "othe!s a!e s##essfl in %!eastfeedin$,  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 sffi#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!$e!'& The lon$<te!" %enefit of s#h a !e$i"e !e"ains np!oven&Ai!)a'Mao! !espi!ato!' o%st!#tion is n#o""on and o##!s e-#lsivel' in %a%ies )ith Pie!!e Ro%in se(en#e& H'po-i# episodes d!in$ sleep and feedin$ #an %e life th!eatenin$& Inte!"ittent ai!)a' o%st!#tion is "o!e f!e(ent and "ana$ed %' n!sin$ the %a%' p!one& Mo!e seve!e and pe!sistent ai!)a' #o"p!o"ise #an %e "ana$ed %' B!etained nasopha!'n$eal int%ation? to "aintain the ai!)a'& S!$i#al adhesion of the ton$e to the lo)e! lip /la%io$lossope-'2 in the fi!st fe) da's afte! %i!th is an alte!native %t less #o""onl' p!a#tised "ethod of "ana$e"ent&P!in#iples of #left s!$e!'The lti"ate $oal in #left lip and palate "ana$e"ent is a patient )ith no!"al appea!an#e of lip, nose and fa#e, )hose spee#h is no!"al and )hose dentition and fa#ial $!o)th fall )ithin the !an$e of no!"al develop"ent&S!$i#al te#hni(es a!e ai"ed to !esto!e no!"al anato"'& With the e-#eption of !a!e #onditions s#h as holop!osen#ephal', the!e is no t!e h'poplasia of the tisses involved eithe! side of the #left& The!e is, ho)eve!, displa#e"ent, defo!"ation and nde!develop"ent of the "s#les and fa#ial s5eleton& E"phasis is pla#ed on "s#la! !e#onst!#tion of the lip, nose and fa#e, as )ell as "s#les of the soft palate& No!"al
 
o! nea!<no!"al anato"' p!o"otes no!"al fn#tion, the!e%' en#o!a$in$ no!"al $!o)th and develop"ent of lip, nose, palate and fa#ial s5eleton& An in<depth nde!standin$ of the anato"' of the #left is invala%le if the s!$eon is to a#hieve no!"al, o! nea!<no!"al, anato"i#al !e#onst!#tion&Anato"' of #left lip and palateCleft lipThe a%no!"alities in #left lip a!e the di!e#t #onse(en#e of dis!ption of the "s#les of the ppe! lip and nasola%ial !e$ion& The fa#ial "s#les /@i$& *7&62 #an %e divided into the th!ee "s#la! !in$s of Delai!e&Unilate!al #left lip& In the nilate!al #left lip, the nasola%ial and %ila%ial "s#le !in$s a!e dis!pted on one side !esltin$ in an as'""et!i#al defo!"it' involvin$ the e-te!nal nasal #a!tila$es, nasal sept" and ante!io! "a-illa /p!e"a-illa2 /@i$& *7&*2& These defo!"ities inflen#e the "#o#taneos tisses #asin$ displa#e"ent of nasal s5in on to the lip and !et!a#tion of la%ial s5in, as )ell as #han$es to the ve!"ilion and lip "#osa& All of these #han$es need to %e #onside!ed in plannin$ the s!$i#al !epai! of the nilate!al #left lip&ilate!al #left lip& In the %ilate!al #left lip, the defo!"it' is "o!e p!ofond %t s'""et!i#al& The t)o spe!io! "s#la! !in$s a!e dis!pted on %oth sides p!od#in$ a fla!in$ of the nose /de to la#5 of nasola%ial "s#le #ontinit'2, a p!ot!sive  p!e"a-illa and an a!ea of s5in in f!ont of the p!e"a-illa, 5no)n as the p!ola%i", devoid of "s#le /@i$& *7&=2& As in the nilate!al #left lip, the "s#la!, #a!tila$inos and s5eletal defo!"ities inflen#e the "#o#taneos tisses, )hi#h "st %e !espe#ted in plannin$ the !epai! of the %ilate!al #left lip&Cleft palate E"%!'olo$i#all', the p!i"a!' palate #onsists of all anato"i#al st!#t!es ante!io! to the in#isive fo!a"en, na"el' the alveols and ppe! lip& The se#onda!'palate is defined as the !e"ainde! of the palate %ehind the in#isive fo!a"en divided into the ha!d palate and, "o!e poste!io!l', the soft palate&Cleft palate !eslts in fail!e of fsion of the t)o palatine shelves& This fail!e "a' %e #onfined to the soft palate alone o! involve %oth ha!d and soft palate& When the #left of the ha!d palate !e"ains atta#hed to the nasal sept" and vo"e!, the #left is te!"ed in#o"plete& When the nasal sept" and vo"e! a!e #o"pletel' sepa!ated f!o" the  palatine p!o#esses, the #left palate is te!"ed #o"plete&Soft palate& In the no!"al soft palate, #los!e of the velopha!'n-, )hi#h is essential fo! no!"al spee#h, is a#hieved %' five diffe!ent "s#les fn#tionin$ in a #o"plete %t #oo!dinated fashion& In $ene!al, the "s#le fi%!es of the soft palate a!e o!ientated t!ansve!sel' )ith no si$nifi#ant atta#h"ent to the ha!d palate&In a #left of the soft palate, the "s#le fi%!es a!e o!ientated in an ante!oposte!io! di!e#tion, insettin$ into the poste!io! ed$e of the ha!d palate /@i$& *7&42&Ha!d palate& The no!"al ha!d palate #an %e divided into th!ee anato"i#al and  ph'siolo$i#al ones /@i$& *7&.2& The #ent!al palatal fl%!o"#osa is ve!' thin and lies di!e#tl' %elo) the floo! of nose& The "a-illa!' fi%!o"#osa is thi#5 and #ontains the $!eate! palatine ne!ovas#la! %ndle& The $in$ival fi%!o"#osa lies "o!e late!al and ada#ent to the teeth&In pe!fo!"in$ s!$i#al #los!e of #left palate the #han$es asso#iated )ith the #left "st %e nde!stood to o%tain an anato"i#al and fn#tional !epai!& In #o"plete #left  palate the "edian pa!t of the palatal valt is a%sent and the palatal fi%!o"#osa is !ed#ed in sie& The "a-illa!' and $in$ival fi%!o"#osa a!e not "odified in thi#5ness, )idth o! position& 

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