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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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