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CASE PRESENTATION SUBDIVISI BEDAH ANAK
I. IDENTITAS PASIEN
RM : 725404
Ruang : Lontara 2 Atas Depan
Nama : An. E.N.F
Tanggal Lahir : 2!0"!20
#enis $elamin : %erempuan
Alamat : Desa Matara&a' MunaTanggal Masu( Rumah )a(it : 2*!0+!205
II. ANAMNESIS
Keluhan Utama:
,uang air -esar tia( lan/ar
Anamnesis Terpimpin:
Dialami sea( umur 4 -ulan' mem-erat saat umur * tahun 1ang lalu
se-elum masu( Rumah )a(it. ,uang air -esar tia( teratur an (aang (ali
tiap minggu. Ri&a1at -uang air -esar i-antu engan o-at pen/ahar aa. Mual
tia( aa. Ri&a1at muntah se-elumn1a aa' tia( men1emprot &arna hiau.
Ri&a1at emam tia( aa.
Ria!at Pen!a"it Se#elumn!a:
-u pasien men1aari i(a terapat gangguan e3e(asi paa ana(n1a
saat usia 4 -ulan. )aat itu orang tua pasien han1a mem-eri(an o-at pen/ahar
1ang i-eli seniri' setelah itu (eluhan menghilang. )etelah itu (eluhan susah
,A, mun/ul lagi an mem-erat paa usia 4 tahun' an tia( mengalami
per-ai(an setelah pem-erian o-at ul/ola. Terapat ri&a1at (eterlam-atan
(eluarn1a me(oneum 6 * hari setelah lahir.
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Ria!at Pen!a"it Dahulu$
Ri&a1at alergi o-at : isang(al
Ria!at Pen!a"it Keluar%a$
Ri&a1at (eluhan 1ang sama : isang(al
Ria!at Kelahiran$
Ri&a1at (elahiran : spontan ari i-u %A0 itolong -ian' langsung menangis'
,,L tia( i(etahui orang tua
Ri&a1at (ehamilan : aterm' peri(sa rutin i -ian
8sia i-u saat melahir(an : 2* tahun
Ri&a1at me(oneum : 9 * hari
III. PEMERIKSAAN &ISIS
Status Generalisata:
$eaaan umum: )a(it seang;i<i (urang=ompos mentis
,,: * (g T,: +" /m MT: 4' (gm2 'i(i "uran%
Status Vitalis:
,%: 00"0 mm>g RR: 24 menit' simetris' ?esi(ular
>R: 0 menit' regular' ae(uat Temp ailla: *"'5@= a(ral hangat
Status Lokalisata:
Regio a-omen:
! nspe(si : atar' i(ut gera( napas' arm /ontour !' arm
stei3ung !! Aus(ultasi : %eristalti( aa (esan mening(at
! %alpasi : N1eri te(an !' massa tumor !' supel' e3ans
mus(uler !
! %er(usi : N1eri (eto( tia( aa
Rectal Toucher:
)3ingter ani men/e(i(' mu(osa li/in' ampulla -erisi 3eses' tia( tera-a
massa tumor' an paa handschoen terapat 3eses' lenir an arah tia( aa.
IV. &OTO K)INIS
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V. PEMERIKSAAN )ABORATORIUM
**+,-+*,/
Hasil Nilai Ru0u"an Satuan
B,= C."* 4.00!0.0 0*uL
R,= 4.74 4.00!".00 0"uL
>;, 0.+ 2.0!".0 gL
>=T **.C *7.0!4C.0
%LT *7 50!400 0*uL
Natrium 44 *"!45 mmol
$alium *.C *.5!5. mmol
$loria +7! mmol
%T 0.* 0!4 eti(
NR 0.+" !! !!
A%TT 2C.5 22.0!*0.0 eti(
;D) 4+ 40 mgL
8reum *0 0!50 mgL
$reatinin 0.55 L.* %. mgL
VI. PEMERIKSAAN PENUN1AN'
Foto Polos Abdomen (18-!-"1#$
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! Tampa( uara teristri-usi sampai (e istal /olon isertai 3e/al mass
1ang -an1a(
! Tia( tampa( ilatasi loop!loop usus maupun gam-aran herring -one
! $eua psoas line an preperitoneal 3at line -ai(
! Tulang!tulang inta(
Kesan: Ter2apat 3e4al mass !an% #an!a" pa2a l55p+l55p usus
Foto %olon- &nloo' (18-!-"1#$
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Foto =olon nloop:
! $ontras oine se-an1a( 50 // imasu((an engan (ateter (e alam
anus
! Dengan 3luoros/ope tampa( (ontras mengisi re/tum' /olon sigmoi'
an /olon es/enens. Tampa( reunant an ilatasi paa /olon
sigmoi' mu(osa alam -atas normal' tia( tampa( 3illing e3e/tmaupun aitional shao&.
Foto 24 am :
! Masih tampa( sisa (ontras paa re/tum' /olon sigmoi' /olon
es/enens' an /olon trans?ersum engan ilatasi paa /olon
sigmoi.
Kesan: +Dilatasi 45l5n si%m5i2 su%esti3 Hirs4sprun%6s Disease
+Re2un2ant 45l5n si%m5i2
Foto Thora PA ("1-!-"1#$
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! %osisi simetris' (onisi 3ilm -ai(' inspirasi (urang
! =ora(an -ron(o?as(ular alam -atas normal
! Tia( tampa( pemaatan (eua hilus
! =or: -entu( an u(uran alam -atas normal
! $eua sinus an ia3ragma -ai(
! Tulang!tulang inta(
Incidental Finding : Densitas kontras pada loop-loop colon (rest contrast)
Kesan: Ti2a" tampa" "elainan ra2i5l5%i" pa2a 35t5 th5ra7 ini
VII. RESUME
)eorang pasien ana( perempuan' usia 4 tahun' masu( rumah sa(it
engan (eluhan -uang air -esar tia( lan/ar. Dialami sea( umur 4 -ulan'
mem-erat saat umur * tahun 1ang lalu se-elum masu( Rumah )a(it. ,uang
air -esar tia( teratur an (aang (ali tiap minggu. Ri&a1at -uang air -esar
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i-antu engan o-at pen/ahar aa. Mual tia( aa. Ri&a1at muntah
se-elumn1a aa' tia( men1emprot &arna hiau. Ri&a1at emam tia( aa.
-u pasien men1aari i(a terapat gangguan e3e(asi paa ana(n1a
saat usia 4 -ulan. )aat itu orang tua pasien han1a mem-eri(an o-at pen/ahar
1ang i-eli seniri' setelah itu (eluhan menghilang. )etelah itu (eluhan susah
,A, mun/ul lagi an mem-erat paa usia 4 tahun' an tia( mengalami
per-ai(an setelah pem-erian o-at ul/ola. Terapat ri&a1at (eterlam-atan
(eluarn1a me(oneum 6 2 hari setelah lahir. %asien ini lahir i rumah' /u(up
-ulan' -erat -aan lahir tia( i(etahui orang tua' itolong oleh -ian' se/ara
per ?aginam.
%aa pemeri(saan 3isis itemu(an (eaaan umum sa(it seang' gi<i
(urang' an /ompos mentis. %aa status ?italis semua alam -atas normal.
%aa regio a-omen iapat(an -un1i peristalti( aa (esan mening(at. %aa
rectal toucher iapat(an s3ingter ani men/e(i(' mu(osa li/in' ampulla -erisi
3eses' tia( tera-a massa tumor' an paa handschoen terapat 3eses' lenir
an arah tia( aa.
%aa pemeri(saan 3oto polos a-omen Terapat 3e/al mass 1ang
-an1a( paa loop!loop usus. %aa 3oto =olon nloop iapat(an ilatasi
/olon sigmoi sugesti3 >irs/sprungs Disease an reunant /olon
sigmoi.%aa 3oto thora semua alam -atas normal.
VIII. DIA'NOSIS
! )uspe( >irs/hprung isease
I8. RENCANA TINDAKAN
- Ren/ana biopsy rectum
- $onsul peiatri i?isi tum-uh (em-ang
- Diet T$T%
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8. )APORAN OPERASI
Nama Gperasi : ,iops1 re/tum 3ull thi/(ness
Tanggal Gperasi : 02!0!205Laporan Gperasi :
. %asien -aring engan posisi litotomi i -a&ah pengaruh anestesi
umum.
2. Disin3e(si an rapping pro/eure.
*. enti3i(asi lo(asi -iopsi se(itar 2 /m ari linea entata.
4. %asang teugel an la(u(an proseur -iopsi engan insisi re(tum 3ull
thi/(ness engan u(uran iameter /m.
5. $ontrol perarahan an ahit lu(a operasi se/ara interuptus serta
pasang -uah tampon.". Gperasi selesai.
8I. HASI) PEMERIKSAAN PATO)O'I ANATOMI
$esimpulan: Re4tum ter2apat sel %an%li5n 9 C5l5n a%an%li5ni"
HIRSCHSPRUN':S DISEASE
I. DE&INISI
>irs/hsprung terai (arena aan1a permasalahan paa persara3an usus
-esar paling -a&ah' mulai anus hingga usus i atasn1a. )ara3 1ang -erguna
untu( mem-uat usus -ergera( mele-ar men1empit -iasan1a tia( aa samase(ali atau (alopun aa sei(it se(ali. Namun 1ang elas (elainan ini a(an
mem-uat ,A, -a1i tia( normal' -ah(an /enerung sem-elit terus menerus.
>al ini i(arena(an tia( aan1a sara3 1ang apat menorong (otoran (eluar
ari anus.
Dalam (eaaan normal' -ahan ma(anan 1ang i/erna -isa -eralan i
sepanang usus (arena aan1a (ontra(si ritmis ari otot!otot 1ang melapisi
usus (ontra(si ritmis ini ise-ut gera(an peristalti(. $ontra(si otot!otot
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terse-ut irangsang oleh se(umpulan sara3 1ang ise-ut ganglion' 1ang
terleta( i-a&ah lapisan otot. %aa pen1a(it >irs/hsprung' ganglion ini tia(
aa' -iasan1a han1a sepanang -e-erapa sentimeter. )egmen usus 1ang tia(
memili(i gera(an peristalti( tia( apat menorong -ahan!-ahan 1ang i/erna
an terai pen1um-atan. %en1a(it >irs/hsprung 5 (ali le-ih sering itemu(an
paa -a1i la(i!la(i. %en1a(it ini (aang isertai engan (elainan -a&aan
lainn1a' misaln1a sinroma Do&n. 2'*
'am#ar $ %er-eaan antara /olon 1ang normal an ilatasi /olon
paa %en1a(it >irs/hsprung.
II. EPIDEMIO)O'I
%en1a(it >irs/hsprung terai paa ari setiap 5.000 -a1i 1ang lahir
an ini -erhu-ungan paa sampai terai engan 4 ari o-stru(si usus paa
-a1i -aru lahir ini 5 (ali le-ih sering paa la(i!la(i an (aang!(aang terai
engan (onisi /ongenital lainn1a seperti Do&n )1nrome.5
III.ETIO)O'I
%en1a(it ini ise-a-(an aganglionosis Meissner an Aur-a/h alam
lapisan ining usus' mulai ari spingter ani internus (e arah pro(simal' 70
ter-atas i aerah re(tosigmoi' 0 sampai seluruh (olon an se(itarn1a 5
apat mengenai seluruh usus sampai pilorus. Dalam (eaaan normal' -ahan
ma(anan 1ang i/erna -isa -eralan i sepanang usus (arena aan1a
(ontra(si ritmis ari otot!otot 1ang melapisi usus (ontra(si ritmis ini ise-ut
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gera(an peristalti(. $ontra(si otot!otot terse-ut irangsang oleh se(umpulan
sara3 1ang ise-ut ganglion' 1ang terleta( i-a&ah lapisan otot."'7
%aa pen1a(it >irs/hsprung' ganglion ini tia( aa' -iasan1a han1a
sepanang -e-erapa sentimeter. )egmen usus 1ang tia( memili(i gera(an
peristalti( tia( apat menorong -ahan!-ahan 1ang i/erna an terai
pen1um-atan.
Diuga terai (arena 3a(tor geneti( sering terai paa ana( engan
Do&n )1nrom' (egagalan sel neural paa masa em-rio alam ining usus'gagal e(sistensi' (ranio (aual paa mesentri( an su- mu(osa ining
pleus.5
IV. MANI&ESTASI K)INIK
,a1i -aru lahir tia( -isa mengeluar(an me/onium alam 24 H 2C am
pertama setelah lahir. Tampa( malas meng(onsumsi /airan' muntah -er/ampur
engan /airan empeu an istensi a-omen. C
;eala pen1a(it >ir/hsprung aalah o-stru(si usus leta( renah' -a1i
engan pen1a(it >irs/hsprung apat menunu((an geala (linis se-agai
-eri(ut. G-stru(si total saat lahir engan muntah' istensi a-omen an
(etia(aaan e?a(uasi me(onium. $eterlam-atan e?a(uasi me/onium ii(uti
o-stru(si (onstipasi' muntah an ehirasi. ;eala rigan -erupa (onstipasi
selama -e-erapa minggu atau -ulan 1ang ii(uti engan o-stru(si usus a(ut.
$onstipasi ringan entro(olitis engan iare' istensi a-omen an emam.
Aan1a 3eses 1ang men1emprot pas paa /olo( u-ur merupa(an tana 1ang
(has. ,ila telah tim-ul entero(olitis ni(rotis(ans terai istensi a-omen
he-at an iare -er-au -usu( 1ang apat -erarah.2'*'"
;eala paa -a1i an ana( H ana( :
• $onstipasi
• Tina seperti pita an -er-au -usu(
• Distensi a-omen
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• Aan1a massa 3e/al apat ipalpasi
•,iasan1a tampa( (urang nutrisi an anemi
*
'am#ar * $ Neonatus -erumur 2 hari engan istensi a-omen an
gagal mengeluar(an me(onium. ,iopsi re(tal telah menega((an
iagnosis pasien ini se-agai pen1a(it >irs/hsprung.
V. PATO&ISIO)O'I
%aa pen1a(it ini' (olon mulai ari 1ang paling istal sampai paa
-agian usus 1ang -er-ea u(uran penampangn1a' tia( mempun1ai ganglion
parasimpati( intramural. ,agian (olon aganglioni( itu tia( apat
mengem-ang sehingga tetap sempit an e3e(asi terganggu. A(i-at gangguan
e3e(asi ini (olon pro(simal 1ang normal a(an mele-ar oleh tina 1ang
tertim-un' mem-entu( mega(olon.5
%le(sus mesenteri( Auer-a/h an ple(sus su-mu(osal Meissner
tia( itemu(an' men1e-a-(an -er(urangn1a peristalti( usus an 3unsi
lainn1a. Me(anisme a(urat mengenai per(em-angan pen1a(it ini tia( i(etahui. )el ganglion enteri/ -erasal ari i3erensiasi sel neuro-last. )elama
per(em-angan normal' neuro-last apat itemu(an i usus halus paa minggu
(e 7 usia gestasi an a(an sampai (e (olon paa minggu (e 2 usia gestasi.
$emung(inan salah satu etiologi >irs/hsprung aalah aan1a e3e( paa
migrasi sel neuro-last ini alam alurn1a menuu usus -agian istal. Migrasi
neuro-last 1ang normal apat terai engan aan1a (egagalan neuro-last
alam -ertahan' -erpoli3erase' atau -eri3erensiasi paa segmen aganglioni(
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istal. Distri-usi (omponen paa usus 1ang aganglioni( aalah 3i-rone(tin'
laminin' neural /ell ahesion mole/ule' an 3a(tor neurotrophi/. +
)e-agai tam-ahan' pengamatan sel otot polos paa (olon anganglioni(
menunu((an -ah&a -agian terse-ut tia( a(ti3 (eti(a menalani pemeri(saan
ele(tro3isiologi' hal ini menunu((an aan1a (elainan m1ogeni( paa
per(em-angan pen1a(it >irs/hsprung. $elainan paa sel =aal' sel pa/ema(er
1ang menghu-ung(an antara sara3 enteri/ an otot polos usus' uga telah
ipostulat menai 3a/tor penting 1ang -er(ontri-usi. Terhaap tiga ple(sus
neuronal 1ang menginner?asi usus' $etiga ple(sus neuronal 1ang
menginner?asi usus' ple(sus su-mu(osal Meissner' ntermus(uler
Auer-a/h' an ple(sus mu/osal. $etiga ple(sus ini terintegrasi an -erperan
alam seluruh aspe( 3ungsi usus' termasu( a-sor-s' se(resi' motilitas' an
aliran arah.+
Motilitas 1ang normal utaman1a i(enali(an oleh neuron intrinsi/.
;anglia ini mengenali(an (ontra(si an rela(sasi otot polos' imana
rela(sasi menominasi. Fungsi usus telah ae(uat tanpa inner?asi e(strinsi(.
$enali e(strinsi( utaman1a melalui serat (olinergi( an arenergi(. )erat
(olinergi( ini men1e-a-(an (ontra(si' an serat arenergi/ men1e-a-(an
inhi-isi. %aa pasien engan pen1a(it >irs/hsprung' sel ganglion tia(
itemu(an sehingga /ontrol intrinsi/ menurun' men1e-a-(an pening(atan
/ontrol persara3an e(strinsi(. nner?asi ari s1stem arenergi( iuga
menominasi s1stem (olinergi(' menga(i-at(an pening(atan tonus otot polos
usus. Dengan hilangn1a (enali sara3 intrinsi/' pening(atan tonus tia(
iim-angi an menga(i-at(an (etia( seim-angan (ontra(tilitas otot polos'
peristalti/ 1ang tia( ter(oorinasi' an paa a(hirn1a' o-stru(si 3ungsional. +
$lasi3i(asi (eaaan anganliani( apat i-ea(an menai segmen
sangat pene( se(itar 2 /m ari garis mu(o(utan. )egmen pene(
aganglioni( sepanang netosigmoi' segmen panang -ila agangliani(
sepanang re/tum (e uon trans?ersum' segmen total sepanang ne(tum (e
se(an an segmen uni?ersal -ila aging lioni( men/a(up hampir seluruh usus.4
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VI. PEMERIKSAAN PENUN1AN'
Pemeriksaan Radiolo)i
. Foto %olos A-omen apat menunu((an aan1a loop usus 1ang
istensi engan aan1a uara alam re/tum.7
'am#ar ; $ Foto polos a-omen paa pen1a(it >irs/hsprung engan
ilatasi (olon.
*. ,arium enemaa. #angan mem-ersih(an (olon -agian istal engan enema se-elum
memasu((an (ontras enema (arena hal ini a(an menga-ur(an
gam-ar paa aerah <ona transisi. -. $ateter ileta((an ialam anus' tanpa mengem-ang(an -alon'
untu( menghinari (a-urn1a <ona transisi an -eresi(o terain1a
pe3orasi. 3oto segera iam-il setelah ine(si (ontras' an iam-il
lagi 24 am (emuian./. $olon -agian istal 1ang men1empit engan -agian pro(simal
1ang mengalami ilatasi merupa(an gam-aran (lasi( pen1a(it
>irs/hsprung. A(an tetapi temuan raiologis paa neonatus le-ih
sulit iinterpetasi an sering (ali gagal memperlihat(an <onatransisi.
. ;am-aran raiologis lainn1a 1ang mengarah paa pen1a(it
>irs/hsprung aalah aan1a retensi (ontras le-ih ari 24 am
setelah -arium enema ila(u(an.7
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'am#ar < $ Dilatasi /olon 1ang terisi (ontras paa pen1a(it
>irs/hsprung.
*anometri anarektal
Manometri anore(tal menete(si re3le rela(sasi ari
internalsphi/ter setelah istensi lumen re/tal. Re3le inhi-itori( normal ini
iper(ira(an tia( itemu(an paa pasien pen1a(it >irs/hsprung.
)&enson pertama (ali mengguna(an pemeri(saan ini. %aa tahun +"0'
ila(u(an pe-ai(an a(an tetapi (urang isu(ai (arena memili(i -an1a(
(eter-atasan. "'0
)tatus 3isiologi( normal i-utuh(an an seang sering(ali penting.
>asil positi3 palsu 1ang telah ilapor(an men/apai "2 (asus' an
negati?e palsu ilapor(an se-an1a( 24 ari (asus. $arena (eter-atasan
ini an relia-ilitas 1ang ipertan1a(an' manometri anore(tal arang
iguna(an i Ameri(a )eri(at. $arena mal3ormasi (aria( 2!5 an
trisom1 2 5!5 uga ter(ait engan anganglionosis /ongenital'
pemeri(saan (ariologis an geneti/ ianur(an. 0
+io'si Rektal
Diagnosa e3initi?e >irs/hsprung aalah engan -iops1 re(tal'
1aitu penemuan (etia(-eraaan sel ganglion. Metoe e3initi3 untu(
mengam-il aringan 1ang a(an iperi(sa aalah engan -iops1 re/tal 3ull!
thi/(ness. )pesimen 1ang harus iam-il minimal -erara( '5 /m iatas
garis entate (arena anganglionosis -iasan1a itemu(an paa ting(at
terse-ut. $e(urangan pemeri(saan ini 1aitu (emung(inan terain1a
perarahan an pem-entu(an aringan parut an penggunaan anastesia
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umum selama proseur ini ila(u(an. "'7
VII. DIA'NOSIS BANDIN'
DIA'NOSIS
BANDIN'
'E1A)A =AN'
MENBEDAKAN DEN'AN
PEN=AKIT HIRSCHSPRUN'
PEMERIKSAAN PENUN1AN'
Me45nium+
plu% s!n2r5me
%en1emprotan me(onium
1ang men1e-a-(an resolusi
geala paa ana(' tanpa
geala!geala pen1a(it
>irs/hsprung 1ang lain.
8ara 1ang -er/ampur engan
me(onium a(an tampa( se-agai
I soap-bubble appearanceJ atau
Icalcification-like apperanceJ
paa gam-aran raiologi.
Me45nium ileus
Mani3estasi (lini( sesuai
engan o-stru(si usus
engan geala respiratori(.
Ri&a1at (eluarga engan
/1sti/ 3i-rosis.
%aa 3oto polos a-omen posisi
tega(' tia( tampa( air!3lui
le?el an aan1a gam-aran
I ground-glass appearanceJ
paa -agian -a&ah a-omen.
Small le3t 45l5
s!n2r5me
;eala hilang setalah -e-erapa
minggu sesuah pemeri(saan
(ontras enema.
Ri&a1at i-u engan DM.
Tampa( (olon sinistra 1ang
menge/il paa 3leura lien.
H!p5thir5i2is
m
leus (olon an istensi
a-omen engan -rai(ari'
anoreia an geala lain
hipotiroiisme.
%ening(atan T)> paa
hipotiroi primer.
Cere#ral in0ur!
lles (olon an istensi
a-omen.
Ri&a1at trauma lahir menai
pen1e-a- ileus (olon.
Diagnosis se/ara (linis.
Chr5ni4
45nstipati5n
(olon proimal 1ang mele-ar
an terisi massa 3e/es.
Tampa( geala simtomati(
%aa pemeri(saan re(tal' tera-a
massa 3e/es paa (anal anus.
$ontras enema mem-eri(an
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seperti muntah atau status
(esehatan 1ang menurun paa
-a1i -erumur " -ulan (eatas.
gam-aran re(tosigmoi 1ang
mele-ar an (ali-er (olon
alam -atas normal.
Distal small
#5el atresia
Distensi a-omen engan
muntah I-iliousJ.
%aa 3oto polos tampa(
gam-aran atresia (olon.
%aa (ontras enema tampa(
gam-aran mi(ro(olon.
VIII. TERAPI
Pen)obatan medis
Tuuan umum ari pengo-atan ini men/a(up * hal utama:
. 8ntu( menangani (ompli(asi ari pen1a(it
>irs/hsprung 1ang tia( terete(si'
2. )e-agai penatala(sanaan sementara se-elum
operasi re(onstru(ti3 e3initi3 ila(u(an' an
*. 8ntu( memper-ai(i 3ungsi usus setelah
operasi re(onstru(si.
%enatala(sanaan (ompli(asi iarah(an paa pen1eim-angan
/airan an ele(trolit' menghinari istensi -erle-ihan' an mengatasi
(ompli(asi sistemi(' seperti sepsis. Ma(a ari itu' h1rasi intra?ena'
e(ompressi nasogastri(' an i(a iini(asi(an' pem-erian anti-ioti(
intra?ena memili(i peranan utama alam penatala(sanaan meis a&al.
%em-ersihan (olon' 1aitu engan mela(u(an irigasi engan re/tal tu-e
-erlu-ang -esar an /airan untu( irigasi. =airan untu( men/egah
terain1a (etia(seim-angan ele(trolit. rigasi /alon se/ara rutin an
terapi anti-ioti( pro3ila(sis telah menai proseur untu( mengurangi
resi(o terain1a entero/olitis."
Pembedahan
%em-eahan paa pen1a(it hirs/prung ila(u(an alam ua
tahap. Mula!mula ila(u(an (olostomi loop atau double–barrel
sehingga tonus an u(uran usus 1ang ilatasi an hipertro3i apat
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(em-ali normal memerlu(an &a(tu (ira!(ira * sampai 4 -ulan. ,ila
umur -a1i itu antara "!2 -ulan atau -ila -eratn1a antara + an 0 (g'
satu ari tiga proseur -eri(ut ila(u(an engan /ara memotong usus
aganglioni( an menganastomosis(an usus 1ang -erganglion (e
re/tum engan ara( /m ari anus.4'"
%roseur Duhamel umumn1a ila(u(an terhaap -a1i 1ang
-erusia (urang ari tahun. %roseur ini teriri atas penari(an (olon
normal (e arah -a&ah an menganastomosis(ann1a i -ela(ang anus
aganglioni(' men/ipta(an ining gana 1ang teriri ari selu-ung
aganglioni( an -agian posterior (olon normal 1ang itari( terse-ut.
%aa proseur )&enson' -agian (olon 1ang aganglioni( itu
i-uang. $emuian ila(u(an anastomosis en!to!en paa (olon
-erganglionengan saluran anal 1ang ilatasi. )3interotomi ila(u(an
paa -agian posterior.
%roseur )oa?e ila(u(an paa ana(!ana( 1ang le-ih -esar an
merupa(an proseur 1ang paling -an1a( ila(u(an untu( mengo-ati
pen1a(it hirsr/prung. Dining otot ari segmen re(tum i-iar(an tetap
utuh. $olon 1ang -ersara3 normal itari( sampai (e anus' tempat
ila(u(ann1a anastomosis antara (olon normal an aringan otot
re(tosigmoi 1ang tersisa.
8ntu( ana( engan pen1a(it >irs/hsprung engan segmen
1ang sangat pene(' mem-uang sei(it -agian miline posterior re/tal
merupa(an alternati?e operasi lainn1a. %roseur ini i(enal se-agai
m1ome/tom1 anore/tal. %aa proseur ini' /m ining re/tal
e(stramu(osal 1ang -ermula se(itar pro(simal garis entate i-uang.
Mu(osa an su-mu(osa ipertahan(an an itutup. %ene(atan
laparas(opi( se-agai penatala(sanaan pen1a(it >irs/hsprung pertama
(ali ies(ripsi(an paa tahun +++ oleh ;eorgeson. Kona transisi
itentu(an a&aln1a itentu(an se/ara laparas(opi(' ii(uti engan
mo-ilisasi re(tum i-a&ah peritoneal. Mu(osa transanal ise(si
ila(u(an' ii(uti engan mengeluar(an re(tum melalui anus an
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anastomosis. >asil 3ungsional sepertin1a sama engan te(ni( ter-u(a
-erasar(an hasil ang(a pene(.
,onserati.
%aa neonatus engan o-stru(si usus ila(u(an terapi
(onser?ati3 melalui pemasangan sone lam-ung serta pipa re/tal
untu( mengeluar(an me(onium an uara.*
Tindakan bedah sementara
$olostomi i(era(an paa pasien neonatus' pasien ana( an
e&asa 1ang terlam-at iiagnosis an pasien engan entero(olitis
-erat an (eaaan umum mem-uru(. $olostomi i-uat i (olon
-erganglion normal 1ang paling istal.*
/iet
Ma(anan -erserat tinggi an menganung -uah!-uahan segar
apat mengoptimal(an 3ungsi usus post!operati3 paa -e-erapa pasien.4
Aktiitas
,atasi a(ti?itas 3isi( selama se(itar " minggu untu(
pen1em-uhan lu(a se/ara -ai(.4
I8.KOMP)IKASI
)etelah operasi' (e-an1a(an ana(!ana( melepas(an 3eses se/ara
normal. ,e-erapa apat mengalami iare' tetapi setelah -e-erapa &a(tu 3eses
a(an menai le-ih paat. Itoilet training J apat mengam-il &a(tu lama
(arena -e-erapa ana(!ana( memili(i (esulitan meng(oorinasi(an otot!otot1ang iguna(an untu( melepas(an 3eses. ni mening(at paa (e-an1a(an
ana(!ana( seiring &a(tu. $onstipasi apat -erlanut paa -e-erapa ana(!ana('
mes(ipun la(sati? seharusn1a mem-antu. Ma(an ma(anan tinggi serat uga
apat mem-antu paa iare an (onstipasi."'7
Ana( uga -eraa paa resi(o pening(atan enteroloitis alam (olon
atau usus halus setelah operasi. Baspaalah paa geala an tana ari
entero/olitis' an hu-ungi o(ter segera -ila salah satu ari ini terai: emam'
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perut (em-ung' muntah' iare' an perarahan ari re/tum7
8. PRO'NOSIS
A(i-at 1ang ihasil(an setelah per-ai(an pen1a(it >irs/hsprung
se/ara e3init aalah sulit untu( eterminasi. ,e-erapa peneliti melapor(an
ting(at (epuasan tinggi. )ementara 1ang lain melapor(an (eaian 1ang
signi3i(an alam (onstipasi an in(ontinensia. $ira ari pasien engan
pen1a(it >irs/hsprung mem-utuh(an (olostomi permanent untu(
memper-ai(i in(ontinensia. 8mumn1a' le-ih ari +0 pasien engan
pen1a(it >irs/hsprung memili(i hasil memuas(an.2
DA&TAR PUSTAKA
. Lee )L' )he(herimian ). >irs/hsprungs Disease. online /ite De/em-er
200+ : A?aila-le 3rom : 8RL : emei/ine.mes/ape./om
2. Kessmann J !ylie "# $irschsprung%s Disease. online /ite No?em-er 200+ : A?aila-le 3rom : 8RL :
&&&#nlm#nih#go'medlineplusencyarticle**+#htm
*. The 8ni?ersit1 o3 G(lahoma. >irs/hsprungs Disease. online /ite
De/em-er 200 : A?aila-le 3rom :8RL :
&&&.ouhs/.eusurger1 >ir/hsprungs O Disease .asp
4. National nstitute o3 Dia-etes an Digesti?e an $ine1 Diseases NDD$.
>irs/hsprungs Disease. online /ite Fe-ruar1 200 : A?aila-le 3rom : 8RL
: digesti'e#niddk#nih#go'
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5. Meina M. $irschsprung%s Disease. n : ,ian/hi DB' =rom-leholme TM'
DAlton ME'eitors. Fetolog1. 4th eition. Ne& Por( : M/;ra&
>ill2000.p.54!545.
". $olschneider ,# $irschsprung%s Disease and .ariants# In : /uri /
$oll&arth editors# /ediatric 0urgery# 1nd edition# 2e& 3ork : 0pringer-
.erlag 4erlin $eidelberg516# p#+78-+79#
7. Ma1o =lini/ sta33. >irs/hsprungs Disease. online /ite No?em-er 200C :
A?aila-le 3rom :8RL : &&&.ma1o/lini/./omhealth hirs/hsprung s! isease
C. Teas %eiatri/ )urgi/al Asso/iates. >irs/hsprungs Disease Aganglioni/
Mega/olon. online /ite )eptem-er 200+ : A?aila-le 3rom : 8RL :
&&&.peisurg./ompteu/hirs/hprungsO isease .htm
+. Anon1m. >irs/hsprungs Disease. online /ite Mar/h 200 : A?aila-le
3rom : 8RL : &&&.mei/inenet./om
0. 4aucke . Kimura K# Failure to /ass econium : Diagnosing 2eonatal
Intestinal ;bstruction# <I0=# *9995 *:866#
. 0&enson ;# $irschsprung%s Disease : , "e'ie&# /ediatrics# 1157:*9#
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