•Congenital Abdominal Wall Defects

. .•.. ....,..... "11'W1V~lJUfflWfflffi1~ tllt11'lflfflWfflffm

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tillUi9 1.i'

~ ~ d r.d ~ • .d Q.l4. 'WnnnllllJt111lJtlnV1nUfll1::umn'lfV'W'I1~::lml'l'W1'W congenital abdominal wall

•defects li~liv'WUll::l1n~~l~fl

~~ 1" ~I ,d""'.l .•.. )lt

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jj 2 hflA'1VO'W flV1. Gastroschisis

2. Omphaloce1e, ••• II

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1

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I. Gastroschisis•

l~ lJ 1111::fl11lJ i1fl1.ln~'111~H17~11li'1l\' II~~ ~U9iti 11Ui9 defectQ dlJ'IlJli9111n

li9V~11U'll'Wli9 2-5 1'lf'W~llJm 1l~l'1l~A'1'W'I11'1ll~lf1Vff::~1l hw1ujjQ~~lJ Vi11M'1l11~ri1'W

midgut lH<illllnlJllJllm;Il~li'v~' 'lill~ 1111'IJIl~H17mIi'1li'll~ ~::IWn~lnfflVff::iillli9vUClU'lJV~•••••.• d 4 I ••.• V 901 1 0 I _I .•••H111'W~UClUllln '1 ff1Vff::i9ll~::VI!UlJHlJ~11 'Wl'l1V~ lJ9l1Ul1'W~un9l

'.

2.,

Etiology

,J )I Q .d Q J ~ ••••.1. .~l'I'lm'Jl1Ul1~H'lJfJ~ Gastroschisis l1lnfl'IJU&UlJtjlJ(W~ lJm1lJUU'If~

•v .1Q ~Q • • ~ V Q d1ft• ~v • ~ V Q

ll!J~u!JlJ~01n'llJl1l'l'llJ11(l1111l111'lJlnflf1l1:;U 1'1&f1Ul1lll1'IJlfl folic acid 11lll1lnfl

~ v d'; • d -"l ~ ""1'U<l:;011 &l1l'1'11salicylates lJm1lJl'lffJl1 (J1~~:;lJ01HllJl1(J'l11l~m1lJl'IU~ 11'1

u~1u

hypoxia

Incidence

i )I ~ d '""'~ l.d......."..:. .,f .J.l'IlJ 'ltl 1:; lJllll I; 10,000 llH~Ofl<lfJfllJ'lfl'l U<l:;l'llJ111JlllJ9I011111ll'llJ'lJUlHW'1 l'IlJ

v ~ "d .1 d V •• ~ • '"gastroschisis U(JUM IU1J11~1111J!J1qlJ10'IJU lJ11tJ~lUUfJtJlJ1011l'1lJlU1J11~lfJ1q1J1nm1 30 u

l'IlJu(JtJ1 UL~Ofl<lfJ.1fifJuii111U'l l'I111UI~O'lf1tJU<l:;I~nmiJ~l'yh '1 flU

Diagnosis

gastroschisis0111iJ~li'tJ

ultrasound

•l'I'llJ11m'h 1~9i'~U~11l1nfJ~1U fln fi 1f1tJ011m 1~~1tJ

•~ .1)1'''')/)1 il'J.~~:;l~11<lOlJ1ll:;'lJfJ~<l1 l'I'!J~U(JOHU~11U111(J~'IJfJ~11l10 0119111~l'IlJ011:; Ui'l1tJ

• •ultrasound iJ~l'I'l1J11(llJ!J01i~m1lJ ~UUH 'IJfJ~h fln jj ~mh 1tT'1~ 0119111~l'IlJri (JUO1Hl<l!J'lUU

'liltJ1,rl1~UHU011fl<l (Jfl1ul'I'mU~~ijll<ll1"nl1l;~fl<lfJ~ 1.1'. . .l1lmnjj gastroschisis li'OU:;jjU111Uo9i'1U'fJtJm11JH~1:tl~11JOU'1 ~:;l'IlJU'fJtJo':h1u

Q .1' •• • •• ~ 11' ~Omphalocele m11JH~1:u11lJl1l'1lJlJ(JU '1fl(Jf1l1:; intestinal malrotation 'lt~l'I1J i'll1mltJ lU•

gastroschisis UfJO~loiJiJ~l'I1Jf1l1:;lh1ff~lJU<lniuM 10-15 %

~ ~ ~'V~' ~ •• ~vv l'111tJl1<l~fl<l(JfI~:;l'I1J(JltJl:;111tJ IU'If(J~11(J~11:;<lom U1:Iu 'l11HU~11U 111fJ~111~i'11U'1111

4 1.)I....d"'" , "J ... ~ 0 1v d'IIfJ~l'I'ltJl'I':;f1(JfJ(J01J1fJgUfJn 'lffJ~11M (J1tJl:;'YlUU(JfJnlJ1111UU fJn'lffJ~l1fJ~U lJ0 ~:;t11U <l1 l'I'l<ln•

11Nf1f~(Jl~l'I1Jih1ff1m1i m:;ll'll:;(J11111 U<l:; fallopian tube ~1tJ ti'nlJlll:;'IJfJ~I;,Mjjti'nlJlll:;

~H '1 flU ;fUflm:;u:;nll1~lh 1tT'u'li(J\i1UJ1f1i1 0'1U'li(J~U1U~:;jjti'nlJlll:;1J1lJUll:;~'l flUL~U. ..

nfJU HU~'IJtNti11tT'l1U1'lii'lfll1qU Hl'1m~ti11ff~:;~lU 1lil~uli'ul'liUtln~ ti11ffnu'li1uulfl11

1liulU~:;1J11J 1li1J1n UI1:;1li9i'lflUl~U nfJU

~loti'nlJlll:;'IJ(J~ti11ff~l'IlJ 'l'h1,rjj~uu~ gastroschisis fJ(Jm~u 2 O~1J fifJ

I. antenatal gastroschisis

2. prenatal gastroschisis

n~lJ antenatal gastroschisis I~Ul'I1n~til1fffJfJ OlJ1(J~U(In'lifJn\'fJ~ 1U1:;u:;um'IJ(J~•

nmi~flnfi 'l'h1M'til1tT'1J11JlJln 'lJU1'l'IJ(J~'li(J~n'(J~jj'IJu1~I~nlJ10

n~1J prenatal gastroschisis I~Ul'I1n~ ti11tT'fJ(Jn1J1fJliu(Jo'li(J~n'(J~1U 1:;U:;11ti'~'IJ(J~011.:: •.•. ,"''''' d .etd", .,.,,,,,, 1 .• ,91~mm III l'I'~:;lJ011LtllltJUutll1~lrW~1ll OU(JU 'lJm~'IJ(J~'lffJ~l1fJ~1J'lJU1f19111(Jm11nfJlJl11l

1m:;tln~

Management• • •

l~mmnnt~U gastroschisis li'n~:;jjllll1UnilU'fJU UI1:;~:;jjiltyl1llmiliJfi(J

•, .

1. Hypovolemia l{j!H~ln'VllUOll'm'I.i'i\' Hll~iJm~l);'llliTlJlh'Vll~ih'l1\r~ Hll~ril'ff'

2. Hypothermia ~~ijm~ l);'llliTVfl11l.1!ouoon~lniHmvllHril'ff' Hll~i,b'l1\r~, ' .

3. infection 1{j!J~~lnril'ff'll~li'noonl.lloguomi!J~~o~ '1111M'liTv~~!Jm~91Vll~!Jl.Iln, . ., '1 v... .... I "... •••••• a",4. intestinal ischemia 11l rrllll:::lln!Jonl.l1UOn'l!!J~ll!J~l.Ifl11l.1lt'W~1l~~"1'lIIl!JVlu. ,

l~V~l{jo~~lnm~\lnn'lli'lJl1~Om~,jVll1ljU"!HHnuri l'ff'

m~ 1'17Vl.I~lhm11Vl1li'~flll0Vl iJ~'lU~~rr~tllYi!Jl917Vl.I~i11V1M'Vi!Ol.ltfll1 flJ m~ ~1~'l, .

~ ,~~ _ ~ d' l' " ~d' ,~ ,1'~~mJ1 Hll~UO~nUm~lnVlm1~Hmn'lfOUml.lllnlll1l.11H{11 111rrllO~UOn'l!!J~llO~ fl1~ ~l~lJ. . ~ ...m~~Hl'I1Uff~ll1Vll{Ol.lViullO'lI;io 'I11m1l.1rr~Ol'l.11IJlhn~ O~U IjjOl'l'~OlVl~Hl{1~~MO~l.Ii\'1v

II ," If

~lnO'lfl1111V'1 iu lVlofJo~tiu nnl);'llliTlJl.hHll~m1l.1!ou Hll~llJum ~fJo~tiu m~91VlI;ioi\'1V. ,d ~ , , ~d "~ l' , '1 ~ - - ,1'U!Jn~lnUU~fl!J~~Hlllll rrllO!Jnl.l10~UOn'l!O~llO~1'I1Vl.I l11nrlm1~"lVllllOVl (ischemia) ~l

U1mf u ~~l~l.I1M'rrml1111'Vll~l1l'1OVlI~!JVl ~ Hl1 ~l111ff'Ul~OVl~llJ' l1t1lH"U (upper.. 'extrcmities) nou fl1~l1~m~v~nn'M'rrntl1'Vll~"l (lower extremities) I{jO~Uln1u~~v~l1li'~

I ••.• I So' .;. ••.• 11 v"':' .,f 01~' d -fn9lVl1JVl'l!!J~l'lO~ll.l0fl11l.1'lU U'I!!J~ll!J~lYil.ll.l1n"U~:::1l1l1m1 l11111VU"!J~lll!JVlUlnlower

.. "~ ' 1~ . .Iextremities l.I,,~Vl1n Hl'I~OlUll1 l1"lHll~ penneum lJ1l.1l.11n"U

1lirr1vrr1Um~IYi1~!J1l1n (oro-gastric tube) lVlofJ!J~tiu'1.i1M''Vll~I91UfJlm~iilnj!J~

l.I1n~u l'I'1Ul{1~ril' ff'il1qji\'1VJltniio~u lYiOll'lU'l.I1t1l"!J~~m1 lYi0'l111M'ri1Mij"ulVlI~nll ~

1, ~ , ~ , l' ,.I ~, 1 ' 21" . ,-rrll.11H1 rrnl'llJ'I!O~ll!J~ I'Inv"u f{'l'VllV'Vllnu l'I'l'I'lVrr1Um~IYi1~urrrrl1~ lVi!J~u~l.I1t1l"!J~

ill'l'l'I'11~ Hll~'li1vllVl"ulVl"O~m~lYi1~ilrrrr11~i\'1v 1U'Vllm~11~nl.l1n olu1lirrlV Foley's

,I.i,i\' 1l11i'rrlv NG tube llJ!J15 HllU lyjo~~'l.iln'l!iu9l11v~ovi!Jilrrrrl1~

U 1'Vll~m-.i'l€!JlJ (incubator) lYi!JmlJfJl.Illtlll1lJiJ"o~il~mv 1umlii~i\'!J~li ~'Vll~n

~!J'uflJnnfmJ1~h ~YiVllJl11gU~~i\'!J~~H11l1l1~fJo~tiuil'llm~1~ '1 llJUWll1l11mJl\lYi1~1u

m Iii~i\'!J~I~U'Vll~llJU~~V~llH , nil '1 '111u~iJlJtn'l1m~ rrll.11~o~ Hll.,hvm ii!J~i11Vl.Ili\'1V

l'lnn~llJu gastroschisis 'YjmlV ~lllJu"'!J~'i\'flJnnfnlll hWm~~l~Vl 1-ri!JUlm1

ril' ff'nli'lJl,j'lti'lio~~ O~

m~~1~Vlfn1l1'111'li\'311iiio

1. Primary fascial closure

2. Repaired with skin flap

3. Repaired with prosthelic silo

.' '10'1' ~~~1~".I11. Pnmarv fascial closure m~l'll Vlmu'l'l!!J~lJUflU~l1Ul110~l1n'jH"U utlU1

nl1l~~1 l-riO1M'rr1l.1nOU1101rilMm;lJl,j'lti'li!J~~!J~ 'i\'~lV~U 'I11nn1j9lfltJ~m!1~!J~'Yjn

HU1'Vll~ (abdominal wall stretching) f)/h~~1JU111 l-rio1M''lio~~o~ij"ulVl1mli~u ~lmfu

Ulmlih I ff'Ol;ul'li'l~'li!l~l1'!l~ l'hnmEuil~n~ llJlIi'!l rectus l'li'lllltlU n!lum1il'ln~llJlif!l

• ~ ~.'I ,. , ~ 'I ' ~ ".;.f ~~ .•~. 1rectus m1'1'1lmnh::llJuFJU1~n!lU1l~::l'1Ull!lfl1llJ~U U'I1!l~l'1!l~l'1l'1'llJ'UUlfll11!lllJ fl~'J

1::UUnl1111~'I~ 1::uml1 'lull<l::I1<l!l'llii!l~ 1l<l::.l1lil1ff'~'IffOl;ul'li'1lu'Iul1'!l~ nuilVllnr~

l1W'll1'!l~~~liiuI U~::liiflH<llff~ nl 'Il1nl11fl~!lU1111'U!l~m::,j~<llJ1l.iIT::~nn nl 'Il1'1'1l1nlll~ 'I~

lli'l'w 111!lfl1llJ.lU 'Iu'li!l~l1'!l~lT~l'h 'Il1nVllff'ulii!l'l inferior vena cava nl'l l11ii!l'lI 11<lOl;U• , .111'I~W'!l~<l~ l'h 1M'fl1llJ.lU1<l11Vl<l~MIII 111!lli1lff'lhu~VlllUU nl 'Il1'U1Vllii!lVllJllff~~nl'l l1

lil lff'IU1Vll~M

2. Repaired with skin flap nl'lumtli~1l.ilTllJ11f1nl911lJllUU~ I 'Ii'\' lIi!l~~lnlil1ff'

U1lJlJln 111tl'li!l~l1'!l~ii'UU1Vll~nlJlnii~u~':h~::nlnl1 /l~'U~1~mh~L~lJ~1l~1~mlJ M~IiilTllJ11f1ullil1ff'm;ul'li'l'li!l~l1'!l~11111lJ'l M1!l'IuUl~mtli m~Ull!lllil1ff'Ol;ul'li'l'li!l~l1' INIII

I1lJll u9imllJ.lU 'Iu'li!l~l1'!l~lvilJ~ulJlmiiu '1u ~UI~n'l'1l1n1U'llil,\' r11llJ.lU1<l1191~1<l~111!l. . .• ~~~ •• ~.I d 'I ~ • ~ ~ ~ ~ ~ ~ •• .•<ll lIHnVlnl1::'Ul~l<l!l~lUl<l~~ I1mm1Im::H1I1U~U1 nlll HU~I1Ull'1!l~ !l!ln~lnnl1llJIU!l

.: •• ~ d • '" ". d '~!!'I~'Irectus 1l<l::LlJ!lW!l'Ul~lfW~l'llllJU skin flap ll<l::~~ml skm flap lJ1WlJfl'llJ<l11l'f11 UUU1

~.. ~ 1 fOo"",Jd ,.. I ?I I )J '1'" Id .••• •n<ll~m 'If~mnmn 'l~11!Uf1~UlJ1l~::l'fllJ11f1uVl'l1!l~l'1!l~91 1191n~::ln'lm1:: ventral hernia• • •nm:: U~nlllll U1n<lH l1'!l~ iii \l 'Vi1::ff1U'U!l~fhmT~InlUun fl'llJ!l1~1::m~ 'IU!l~ '1l.iiJi'u. .'n~llJl{'j!ll<l~ nl 'I1fHU~I1W'll1'!l~lJ~nlll Q 'll.iiJmllJll~~1l1 ~ lil!l'l'1l1m!l~;191 '1u'lll ii~nlnl1

Hl.lVliinflf~ Irl!l!ll~UmJllll 6-12 1~!lU l'i~!l'li!llJU'lflJHU~mj'll1'!l~iinflf~l1rl~.' '

3. Repaired with prosthetic silo Hiumtlinnl911lJUuuli I ll<l:: 2 '11i'li'\'I{'j!l~~ln

• ~ ~ •• d • d • 'I~ . ~ d 'I~. d '"<l1llTlJ1lJlJln 111!llVlnml<lnlJln l'll 11'U\Jlll'l1!l~l'1!l~l<lnlJln 'I11lTij~lnm~lJ!lnlJ1WUllJU'l~

Lli!lm!lulil I ff'mlH ll<l::lgUUl1l~I;,~9iVltllJHU~I1W'll1'!l~11l~1!lu lil lff'~::!l~U!l n'li!l~l1'!l~II';

!l~m~ 'IU'l~ nlnUFJnu l1l~lJU'UlJ~'l~H 1l,,::nlnl1UU '1cilil1ff'I'li'l~'li!l~l1'!l~!lVH~l'1 !ll~ 'I~nm 1-2 ffU'll,.;' 1~!l1!ln"1'll1lil'lff'''1lU1lJM ll,,::'liINn'!l~'U!l~'I'1lm191~Ulrl!luu'lcilillff'Ol;u

~. ~ '111 tI «i 0 I.... d .•.. "SI 'J/ ••.•1'll1'l1!l~l'1!l~ i'll1lJ'l1l<l1~~'I'1lm1Hl91~WUHU~I1Ull'1!l~L'IIllf1nU

Result~ ,.. d.f •• 'I..... d~

nlnmnm1:: gastroschisis ~lH<lVl'llU911lJ<ll~UUu~~UU'ViU!l~1lnlnlJVl'l1191

lJlnnll 90% ~~liiVl~lnl'flll1~I1" l~mh~ l'liu nl11U~~~ '1i'\'n!lUfl<lllVlnl'Il1lTllJ11f1ff~.l1

lJUVlllJlfl<lll'l 'IUh~'Vi~lUl"~lTllJ11(l'JU<l1,\'~ ll'1flUflnl1Hl.lVl 1111::nl1tJll"I1li'~Hl.l~~~U

1~VI\l 'Vil::!l Vl~t~nl1 'I1fm1ll1l'1H 11"!l~llii!l~ U<l::Ifl~II~'li1~l1l~'I~rill11 Ul'llm~ii fJlllm'Vi~Post sur~ical complication

m1::11Vl1ni'llUI1li'~lh.l~ U\j~lnu 3 'li1~;i!l ~arly, intermediate ll<l::late 91llJ9111l~~I

4 ,

,5

LATEGrowth delayLowIQMalpositioned visceraAtypical appendicitisIntestinal obstruction

INTERMEDIATEInguinal herniaGastroesophageal

RefluxCryptorchidismVentral herniaSmall intestinal

obstructionMalrotation with

obstructionNECGastric outlet

obstructionMalabsorption

EARLYRespiratory distressSepsisWound infectionPostsurgical ileusMalabsorptionEnteric fistulaRenal vein thrombosisDehydrationPremature separation

of siloNECLower limb swellingGastric outlet

obstructionNEC- Necrotizing enterocolitis.

tl'Htl~mn D.R. Cooney, Pediatric Surgery, Mosby-Year book, 1998.'t' ow •••.• 0'l" .... ..e.... ~~U'jfNM<l~Hl91'J!Jl~YllJ1111:::<l1l'f'l'J91lJ 'lf~!Jl~lflfl~lfl adhesion, Ladd's band M~!J

Volvulus ~~lfifl11lJnlJ malrotation Wl~ Necrotizing enterocolitis ~lfiflnlJ~lbu

gastroschisis Ylu'l~U!JU lm:::YllJll1~lJl'f11l19Jm~91luii'~20%

II. Ornphalocele

'" ~ .1 ~ ~ ~ ~ d l' ~ ~ ~ '1' '. , ~ ~ ~lUlJmllJHfluf1l1l'IJ!J~HUmlJ1Yl!J~ lJm~l'fll~H\J~M\J1Yl!J~ lJl'flJ\l~W m IMH\J~M\Jl•

l1'!J~Ul~rhlJ'lJl'JM1UitJ iill.ilWU~oii'\JUl~'1 tJ~:::fl!JlJ~1U peritoneum, Wharton's jelly 1m:::

.1 ~"'.I ~ • d "~, ~ •amnion u ~:::fl!JUfllJIUlJlJ~u flfll)lJ!J1//1:::111//LlJYI!J!JfllJl!J~lJ!Jfl'lf!J~l1!J~l'f1lJ'IJ!J~!J1U1:::111U IlJ

~rllJ!J!JfllJl MilO lil'1tf'I~fl lil'1tf'1my flmYll:::!J1M1~ 91u 1l<l:::l!llJ l~lJ9\'U l'fl//l'f:::$i!J~:::~fl•

!J~UlJlJ~'lJlJl'J'IJ!J~fI11lJ~'JtJfl~lJlJHlr~M,r1l1'!J~jj91~Il.i4-12 l'lflJ~llJm

etiology

l'flmliJfl"lii'J'IJ!J~ omphalocele v~hjmllJmloii'fl YllJfI1llJ~fltJfl~'IJ!J~ chromosome

11lJii'~ 10'38% fhlJillqil~\J trisomy 13, 14, 15, 18 ll<l:::21 jj~I~UlJll<llU~lU~mh11l"'" ....)1..... Q,I

omphalocele lJf11llJlflU1'IJ!J~fllJn l~ ()lUl1!Jfll1l~YllJ~fl~~lJ

Incidence

J i d dd i d 'd ~YllJ omphalocele 1 ~:::lJ1W 1:5,000 Ulflflfl<l!J'JlJ'lfYlYllJ lJlflfl'lflUlJlflf11l1flflMlJ!~

tJ~:::lJlW 1.5 :1

Diagnosis•

lYllJl~{)'Mnn1ij~litJi~~~u~m~nvQhJt1nnlJl~fll 1fltJnWril ultrasound m~fl~1~

'YiU omphalocele ill fetus L1'Im1mj~;illnnfl11~tillllilJ1VilJ 1l1mllf111lJHfltlflVi'i1lJgll '1• • •

lY1VHL1'Ill'li'm;l"illm~~fliill hl'11 i Mm~~~t1n niillllfl"~l17'v ill l'Yi~1:; omphalocele linjj

fI11lJi1fltlnVi71lJ~,llU ~~ ~llL~mmn illlYllJl~{)mj1Miv itli,\'.", "'" _I co "" y" "" "" t.t ' 'JI .-!!ll11tJl1"~t1"V~ 'YiUt111lJHflu nmm ~Hll~11111'Y1V~ lJV1tJ1:; LII 'Il'V~'Y1V~tJllVVnlJ 1111tJllV n

1fltJihtmm '1 t1'llJVQ'lJVU'lJV~'l~Viflnui11mr~lYltJlY:;~vViflVQmJ'l~ 'lJlllflLff'llfhtlll1rn"l~VQ, . .

1:;l1'il~ 4-12 l'b'llVillJm ~1'l~l1'l1l~tl1fllJ H,r~UN lJVm'lllV1tJ1divQl11tJilliA' U1~t1i'~'l~U

iJ! • ~ ,'" ~. ~ ~ ~ ~lJl~Ufln ~I 'YI1111"V~l'll'fI11lJ":;lVtJ"fln~~"clnll\U:;1f111'IJV~'l~ L'YiVLWnmJgastroschisis nn. .m1~'YiU~U~lllJVfllJl11lJfl'livnllJ~11'l1l'li'lJti~;;'ilI1'l1l omphalocele, ill gastroschisis ~:;i11'YiU

, ''" .••• I ..., o!I d

flUtJllVVfllJl U" omphalocele ~:;'YiUflUtJllVVnlJ1{)~48%

m~ m 1~71~mtJ lim1'l1lfl1~" ~1~111fI11lJHfltlflVi71lJ ~'Yi1JtilJtJ~ll"fi VfI11lJi1fltl flVi. ,'lJvni1i~ lllJfl~lflUl1'l1lt111lJH"tln~iYi'YiuiA'iiv cloacal exstrophy , bladder exstrophy,

•~~anorectal Malformation, inguinal hernia, undescended testis U,,:; hypospadias llvn~lfllltJ~'YiU

•71lJii'umjlJlJlfln (syndromes) 9il~ '1 linl1"clltJVlh~~~u

I. Pentalogy of Cantrell

- Upper midline omphalocele

- Anterior diaphragmatic hernia

- Sternal cleft

- Ectopia cordis

- Cardiac anomaly

2. Lower midline syndrome

- Bladder or cloacal exstrophy

- Anorectal Malformations

- Colonic atresia

- Vesico-intestinal fistula

- Sacral vertebral anomalies

- Meningomyelocele

3. Beckwith - Wiedemann syndrome

- Giantism

- Macroglossia

- Omphaloale

b,

,- Pancreatic islet cell hyperplasia

4. Trisomy syndromes

- Trisomy 13 through 15

- Trisomy 18

- Trisomy 21

5. Reiger's syndrome

6. Prune-Belly syndrome• •

1'jUf11llJ~>l11n~'hlJtiu omphalocele 1f)'ullVlJln ij~lV~llJ9i'~ll~ 35-81% lJlln~lniJ

ti~1'jUf11llJ~>l11n~'Jll~I;"ff'l~ ll~'lJullmvhllJ gastroschisis (79% 1111::100% "llJih~u)d , JI

l1UlJ intestinal malrotation ~1lJi'l1V

Omphalocele

GASTROSCHISISLateral to cord, left sideSmall «4 cm)Absent, no remnantsNormalUsually edematous, leathery,foreshortened, with exudateUnusualPresentSometimes near normalRareGreater riskCommonCommon

Often presentPresentSmallCommon (40% to 80%)UncommonRareRare

.::t d ••.• ..,. ''''

m~H'V12 llff>l~\l~l1mJI\l::'V111.,nm~fllJ'Jll~gastroschisis LII1::

OMPHALOCELEUmbilical ringVariable (4 to 10 cm)Present, may be rupturedOnto the sacUsually normal

LocationFascial defectSacCord attachmentIntestinal appearance

Herniated liverMalrotationAbdominal cavityAssociated anomaliesBowel strangulationBowel atresiaAssociated intellectual anomalies

aHii~;nn D.R. cooney, Pediatric Surgery, Mosby-Year book, 1998.

Management~ d d

fll"Jlll1~fllJl Omphalocele lJ 2 lllJ1'V1Hfill

1. Non-operative treatment

2. Operative treatment& 91 ~ ,dod "'" ..,.~

fJ~ Omphalocele \l~UlJ~::LUlJfJ~Ul~'1 1l"fllJf111lJllllJV1l1llfl1~ ~lnff\l"fll~f1l1ll"

omphaloeelc UllIfl'11 ~::'hjffllJl~\l1~fll~~fllJ1UUUd •• ~ •••.••.•'HffllJl~ \l 'VlllllllJ'JlJIlIlllJIIUUI~W1flU gastroschisis

, , d

'V1Wll'll~fll1ll>ll1U11lJfJ~ omphaloeele•

ll\l1fl'JI\l::f1l1ll"Ii1V~ 4% IVhUlJ l1'lmnfJ~•• PI ,.. '" , ••..

conservative ~lllJ lJ9lll~~fllJ1UUU~11l1III

.• JI ' 1 ''''' d d .•fill \ll'l1ll~ M1lJ'J\Jl>lll1flfl'Vll primary

fascialclosure Cl1defect ij~\,mllhunn1~~iJ~~1V skin flap ll.iCl1defect ij~U1~1\1l1ilJln

~iJ~A',V prostheticsilo

Non operative treatment

1~rlll1flJ~lJ1V~ij omphalocele ~U1~111qj(defect 111qjn117 t'llU~IlJm) UCl~~lJ1V

~ijIl11lJi1mJn~11lJl1n1vmh~ I~WLllVl1~1l11lJi1mJn~m~chromosome lln~1l11lJi1~un~m~••• '1 cv QI Ja i'" lJ _Sf cv """.:. .J 0 1"1111~ l1nnmnmJ1lt1JlJUfHl VJllCllJ111l)~UmllCl~Il91ntm~m1lJlHnUnn91~t~ll 'Il~m \'1

1 ,~ Q .I •• '':1 .,~ ~.",~vm1l~ antiseptic Solulion mlJ1nwl)~ 'Il~~~tUUmHJlt~ll 111UCl~'l'l1ll1l)~lll1~Il~~tlJU

eschar ~1V

antisepticagent ~hftlllV '1 MUtl

.0.5% mercurochromein 65% alcohol

- 2% mercurochromesolution

- 70% alcohol

- povidenesolution

- silver sulfadiazine

mn11 antisepticagent 1\J'lh~U1n'1 llWl11tlllV'l l'liU H mercurochrome m'IJn

2-3 i',1lJ~ tljunCl1 2-3 i'u lrllll)~IU~VU1'ln1VltljUeschar u~~ '1 u~1ii~mtljui'uCl~ 2 I1f~

nn~l)~tll.ln,(I11Vlllju eschar uCl~11iu91n~lvn1hi'i11mr~lJ1nW'IJlllJl)~1~1tyl~lJ1911Jlfll)lJ•~,. , ~_'l ~ ~ 1" ~ . ~~lJlll)lJl1lJ~ml)~l~Vl'flJ\!1W I;jlJ1Vl'f1lJ11\lfinlJU1U~l Ull~U~lJ1mm11nll1 1111~Ventral

• ....t d ,. .1 v QI'" _ J """ d.J~ v """hernIa llJlll~n 191~U1111~llmn'llllU~ll~mnmml1V1YiU ln~~lnnn'(J~'IllJl'f1nl !~mmI1W

Q?tQ.l •. '''Q'~ Ql)~~UlnmlJUVllJ~U I~U l'fnlJ1lll'HlJUVllJ~lnnn!~ mercurochrome solution ln~1111~

hypothyroid ~lnm1'(J~~lJ1ll1ll~U ~ln povidene solution 11~llm1ij1~l>i'lJalcohol 1u.a ~ V .?I )I :to' """ I .J 4. """.: <\ vlClll~l);~~lnnn l~ 70% alcohol llJU91U 1111~Umn'llllUllnllV1~l1U~l1llm191~I~llI11V191

eschar ~~l'hu111qjt~unn~~t~ll~11i'{uuN l'f11J11tllltrl~1~1J1ll1vi~eschar lllln lln~m

~1V antibioticointment 1111~llmni'llU~'{UllN~I1~;'ill l)~ omphalocele 1191n~n~n11lY

~ll~tl.l~VU llHU III1f nlJ1tljU m1Ih.i~

Operative Treatment

nnfnlJ1l~Vm1Ih.i~ lttl~llllmlju 3 1ii;'ill primary fascial closure, repaired with

skin flap llCl~repairedwith prostheticsilo 1~vJ,11.l ~~tiillnliim1fnlJ1lllJ1JIh.i~trlll

ijm1lJi1~un~~ll~li1 1ff~~ll~lh.i~Il~ ~

l)~ omphalocclc 1191n

•defect ~U1mCln

1iim1fnlJ1l~vm1Ih~~ nlll1illlunu gastroschisis ~m'l1lJ1llll',

g'

;

Results

fol.,m~fmn omphalocele 1U'lh~u flfl. 1960-1970 vnJ'hih)Ol~lOlllJrr~1J1fl 1U'lh~u•flfl. 1970-1990 'r'llJ69l11mlJtln:1J1t1l 30-40% u,,:;1U'lh~u 1990-2000 'YnJ'h60l~10l11J"0l"~

"1.1 ,~. .l~: ~ '"1J1fl Ll1"ll ~:;1J1t1l 10-20% mJ11UOl~lm~0l11J IU omphalocele 'lJUflUU111Ufl'IJUHI1~mUU• •IYlflUJ u1mTml'UlJfl11 2500 flf1J 'r'llJljl1~lm(Jrr~~~ 66% ~lU111tTfl1J1flfl11 2500 flf1J• •• •

'r'lU6m10l1mY;lJ~ 14% I'vhtTU 'Ym111~flu111tTml1,j'UIJ~:;iitl1l1Ji1Y1t1fl91'I11JUlllJfl11

Complication

11111fuflli1J~fmn 1Y1IJconservative ~:;'r'lUI1l1:;umflofiuu ~~ci'•4 ••

1. Ufol"OlYll'l1U

2. 'l~Ul1fl

3. rr1~Yi11~lfltlmn, iu 1ll~U U":; alcohol• •

4. m~91Y114'lfl1uh~'r'l1J1Ul" l\ifl~mwifl~flgh~'YW1Ul"U1U

11111fu fl~1JM fl1111YIIJm ~H1.1YI 'r'lUI1l1:;Um flofiIIUIIIiiUUriu~ lnYltlU~ihlJgastroschisis m1J~urrOl~H1u0l1~1~~ 1

,flll1J

Conclusion

gastroschisis UtI:; ompha1ocele I~Uf1111Ji1Y1tlfl91'IJll~foltT~l1,j'1liu~ 1U'l11~fl~'YiUUfllJ~ j1 ~ I " t ," ..,. I d I J , ".•••A_ .".

11lflll1m~i;JU"~fl)J1U(JH\1flOlfl~ Ol~Ul1OlUrr11J1~[\'I1~:;'l11mYlmll"lU 1111J'l11mllYiU":;1J

"4" ~ i~ ,~ •• ~ 1" • • •.• •'lt1lI1l'Yi'll111'YlIJUmU~ Ul1ll111lfllllmnfl111 1J\1fll1U~ ~:;'l111111J1ll1:;umfl'll'flU1J1fl1J11J

• · i ....:" "li11mim1mnUYI;;:111U fllJ"~ u":;'lt1lI1l'YI4il11 1i~ YI~UUU'Yi'l1(JU":;1JfI"lm'l11~m~U'Yi'Yl1J• ••

~~.rfl~nfl1l1JiliiIJ1nU 1~fll1~rru~ih~u UVH~

• •

1. G.B. Mychalliska, Michael D. Klein. Gastroschisis. In: Peter Mattei, ed. Surgical directives

Pediatric surgery, Philadelphia: Lippincott Williarns& Wilkins, 2003: 511-515.

2. L.P. Abramson, Susan R. Luck, Robert M. Arensman. Omphalocele. In: Peter Mattei, ed.

Surgical directives Pediatric surgery, Philadelphia: Lippincott Williams & Wilkins, 2003 : 517-

520.

3. P. Gomall, J.D. Atwell. Anterior wall defects. In: John D. Atwell, ed. Pediatric surgery,

London: Arnold, 1998: 145-152.

4. D.R. Cooney, Defect of the abdominal wall. In: O'Neill, eds. Pediatric surgery, 5~ ed., St.

Louis, Missouri: Mosby-Year book, 1998: 1045-1065.

5. S.W. Bruch, J.C. Langer, Omphalocele and Gastroschisis. In: P. Puri, ed. Newborn Surgery,

od2 ed., London: Arnold, 2003: 605-613.