HIRSCHSPRUNG DISEASE
DISUSUN OLEH A!"#a A"$a%at" B"&t" Ha!%a&
C1110''0(
PENGUI
DIBAAKAN DALA) RANGKA TUGAS KEPANITERAAN KLINIK  UNIVERSITAS HASANUDDIN FAKULTAS KEDOKTERAN
BAGIAN RADIOLOGI  )AKASSAR
 3a&- berta&$ata&-a& $"ba%a4 "&" .e&5ataka& ba4%a
Na.a A!"#a A"$a%at" B"&t" Ha!%a&
NI) C111 0''0(
 +$+# refarat H"r,64,/r+&- D",ea,e*
 Te#a4 .e&5e#e,a"ka& t+-a, $a#a. ra&-ka ke/a&"teraa& k#"&"k /a$a ba-"a&
Ra$"o#o-" Fak+#ta, Ke$oktera& U&"7er,"ta, Ha,a&+$$"&*
)aka,,ar Oktober 2011
Pe.b".b"&-
Ra$+, Paka$a&-
)e&-eta4+"
Fak+#ta, Ke$oktera&
U&"7er,"ta, Ha,a&+$$"&
1
 
$+&"a #+ar* S/"&kter a&" ek,ter&a ter$"r" $ar" = ,#"&- ata, .e$"a#
$a& $e/a&*:1;
Ga.bar 2* )+,k+#+, ,/"&kter a&" eter&a /a&$a&-a& ,","
/e&r"&e+.* :;
Per,arafa& .otor"k ,/"&kter a&" "&ter&a bera,a# $ar" ,erab+t
,araf ,"./at", :&*45/o-a,tr"k+,; 5a&- .e&5ebabka& ko&trak,"
+,+, $a& ,erab+t ,araf /ara,"./at", :&*,/#a&k&"k+,; 5a&-
.e&5ebabka& re#ak,a," +,+,* Ke$+a 8e&", ,erab+t ,araf "&"
.e.be&t+k /#ek,+, rekta#",* Se$a&-ka& .+,k+#+, #e7ator a&"
$"/er,ara> o#e4 &*,akra#", = $a& ?* Ner7+, /+$e&$a#", .e&,ara>
,/"&kter a&" ek,ter&a $a& .*/+borekta#",* Saraf ,"./at", t"$ak
.e./e&-ar+4" otot rekt+.* Defeka," ,e/e&+4&5a $"ko&tro# o#e4
&*,/#a&k&"k+, :/ara,"./at",;* Ko&t"&e&,"a ,e/e&+4&5a
$"/e&-ar+4" o#e4 &*/+$e&$a#", $a& &*,/#a&k&"k+, /e#7"k :,araf 
/ara,"./at",;*:1;

1* P#ek,+, A+erba64 ter#etak $"a&tara #a/",a& otot ,"rk+#er $a&
#o&-"t+$"&a#
2* P#ek,+, He&#e ter#etak $",e/a&8a&- bata, $a#a. otot ,"rk+#er
=* P#ek,+, )e",,&er ter#etak $" ,+b.+ko,a*
Pa$a /e&$er"ta /e&5ak"t H"r,64,/r+&- t"$ak $"8+./a"
-a&-#"o& /a$a ket"-at"-a /#ek,+, ter,eb+t*:1;
(
 
F+&-," Sa#+ra& A&a#
P+borekta# ,#"&- $a& to&+, ,/"&kter a&" ek,ter&a
berta&--+&- 8a%ab ata, /e&+t+/a& ,a#+ra& a&a# ket"ka ",t"ra4at*
 "ka a$a /er",ta#t"k 5a&- k+at aka& .e&".b+#ka& re-a&-a& /a$a
,#ee7e a&$ ,#"&-* U&t+k .e&-4a.bat -eraka& /er",ta#t"k ter,eb+t
: ,e/ert" .e&6e-a4 at+, ; .aka $"/er#+ka& ko&trak," ,/"&kter
ek,ter&a $a& ,#"&- 5a&- k+at ,e6ara ,a$ar* S#ee7e a&$ ,#"&-
$a/at .e.be$aka& a&tara -a, be&$a /a$at be&$a 6a"r
.a+/+& -ab+&-a& ,erta $a/at .e&-e#+arka& ,a#a4 ,at+ ta&/a
.e&-e#+arka& 5a&- #a"&*
'
 
Defeka," $a& ko&t"&e&,"a a$a#a4 .eka&",.e 5a&- ,a#"&-
terka"t erat* Ko&t"&e&,"a a$a#a4 ke-"ata& /e&-e#+ara& "," rekt+.
,e6ara terko&tro# /a$a %akt+ $a& te./at 5a&- $""&-"&ka&*
Koor$"&a," /e&-e#+ara& "," rekt+. ,a&-at ko./#ek, &a.+&
$a/at $"ke#o./okka& ata, ? ta4a/a&
•  Ta4a/ I* Ta4a/ a%a# "&" a$a#a4 ber+/a /ro/+#," "," ko#o& 5a&-
#eb"4 /rok,".a# ke rekt+. ,e"r"&- $e&-a& frek%e&," /er",ta#t"k
ko#o& $a& ,"-.o"$ :2= ka#"4ar"; ,erta reek, -a,troko#"k* •  Ta4a/ II* Ta4a/ "&" $",eb+t ,a./#"&- ree ata+ rectal-anal
inhibitory reex  5ak&" +/a5a a&orekta# .e&-e&a#" "," rekt+.
$a& .ere#ak,a," ,/"&kter a&" "&ter&a ,e6ara "&7o#+&ter*
•  Ta4a/ III* Ta4a/ "&" ber+/a re#ak,a," ,/"&kter a&" ek,ter&a#
,e6ara "&7o#+&ter* Re#ak,a," 5a&- ter8a$" b+ka&#a4 re#ak,a,"
akt"f .e#a"&ka& re#ak,a," ak"bat ke-a-a#a& ko&trak," ,/"&kter
"t+ ,e&$"r"* •  Ta4a/ IV* Ta4a/ terak4"r "&" ber+/a /e&"&--"a& teka&a& "&tra
ab$o."&a# ,e6ara 7o#+&ter $e&-a& .e&--+&aka& $"afra-.a
$a& otot $"&$"&- /er+t 4"&--a $efeka," $a/at ter8a$"* :1;
IV* Et"o#o-"*
Pe&5ak"t H"r,64,/r+&- ter8a$" kare&a t"$ak a$a /#ek,+,
."e&ter"k+, A+erba64 $a& ,+b.+ko,a )e",,e&er /a$a rekt+.
$a& ata+ ko#o&* Ne+ro& e&ter"k bera,a# $ar" neural crest   $a&
ber."-ra," ,e6ara ka+$a# ber,a.a $e&-a& ,erat ,araf 7a-+, $"
,e/a&8a&- +,+,* Se#,e# -a&-#"o& t"ba $" ko#o& /rok,".a# /a$a '
."&--+ +,"a ke4a."#a& $a& /a$a rekt+. /a$a 12 ."&--+ +,"a
ke4a."#a&* Ke-a-a#a& ."-ra," &e+ro& e&ter"k /a$a ko#o& $a&
ata+ rekt+. "&" aka& .e.be&t+k ,e-.e& a-a&-#"o&"k* Ha# "&"
.e&-ak"batka& /e&5ak"t H"r,64,/r+&- k#"&",* :(;
V* Pato-e&e,",*
@
 
Pere&-a&-a& ko#o& ,a./a" -ar", te&-a4&5a #eb"4 $ar" ata+ (
6. :.e-ako#o&; $a/at ter8a$" ,eba-a" -a&--+a& ko&-e&"ta# ata+
$"$a/at* Pe&5ak"t H"r,64,/r+&- :.e-ako#o& ko&-e&"ta#; ter8a$"
b"#a ,aat /erke.ba&-a& ."-ra," ,e# 5a&- bera,a# $ar" neural
crest ke ara4 ka+$a# $" ,e/a&8a&- ,a#+ra& 6er&a ter4e&t" $"
,+at+ t"t"k ,ebe#+. .e&6a/a" a&+,* O#e4 kare&a "t+ terbe&t+k
,+at+ ,e-.e& a-a&-#"o&"k 5a&- t"$ak .e."#"k" /#ek,+,
,+b.+ko,a )e",,e&er $a& /#ek,+, ."e&ter"k+, A+erba64* Ha# "&"
.e&5ebabka& ob,tr+k," f+-,"o&a# $a& /ere-a&-a& /ro-re,"f 
$ar"/a$a ko#o& 5a&- ter#etak /rok,".a# $ar" ,e-.e& 5a&-
terke&a* Pa$a ,eba-"a& be,ar ka,+, 4a&5a rekt+. $a& ,"-.o"$
5a&- a-a&-#"o&"k teta/" /a$a ,ek"tar ,e/er#".a ka,+, 5a&-
terke&a a$a#a4 ,e-.e& 5a&- #eb"4 /a&8a&- $a& ba4ka&
ke,e#+r+4a& ko#o& :%a#a+/+& 8ara&-;*
Da,ar /ato>,"o#o-" $ar" HD a$a#a4 t"$ak a$a&5a -e#o.ba&-
/ro/+#,"7e $a& ab&or.a#"ta, ata+ 4"#a&-&5a re#ak,a," $ar"
,/4"&6ter a&+, "&ter&+, 5a&- $",ebabka& a-a&-#"o&o,",
4"/o-a&-#"o&o,", ata+ $",-a&-#"o&o,", /a$a +,+, be,ar*
• H"/o-a&-#"o&o,", 2
Pa$a /ro".a# ,e-.e& $ar" ba-"a& a-a&-#"o& ter$a/at area
4"/o-a&-#"o&o,",* Area ter,eb+t $a/at 8+-a .er+/aka&
ter",o#a,"* H"/o-a&-#"o&o,", a$a#a4 kea$aa& $".a&a 8+.#a4
,e# -a&-#"o& k+ra&- $ar" 10 ka#" $ar" 8+.#a4 &or.a# $a&
kera/ata& ,e# berk+ra&- ka#" $ar" 8+.#a4 &or.a#* Pa$a
6o#o& "&er7a," 8+.#a4 /#e+, .5e&tr"6+, berk+ra&- 0
$ar" &or.a#* H"/o-a&-#"o&o,", ka$a&- .e&-e&a" ,eba-"a&
/a&8a&- 6o#o& &a.+& a$a /+#a 5a&- .e&-e&a" ,e#+r+4
6o#o&*
10
 
Se# -a&-#"o& 5a&- ".at+r $e&-a& $e&$r"te 5a&- ke6"#
$"ke&a#" $e&-a& /e.er"k,aa& LDH :#aktat $e4"$ro-e&a,e;*
Se# ,araf ".at+r t"$ak .e."#"k" ,"to/#a,.a 5a&- $a/at
.e&-4a,"#ka& $e4"$ro-e&a,e* Se4"&--a t"$ak ter8a$"
$"fere&,"a," .e&8a$" ,e# S64%a&&J, $a& ,e# ,araf #a"&&5a*
Pe.ata&-a& $ar" ,e# -a&-#"o& $"keta4+" $"/e&-ar+4" o#e4
reak," ,+66"&5#$e45$ro-e&a,e :SDH;* Akt"7"ta, e&!". "&"
re&$a4 /a$a ."&--+ /erta.a ke4"$+/a&* Pe.ata&-a& $ar"
,e# -a&-#"o& $"te&t+ka& o#e4 reak," SDH 5a&- .e.er#+ka&
%akt+ /e.ata&-a& /e&+4 ,e#a.a 2 ,a./a" ? ta4+&*
H"/o-e&e,", a$a#a4 4+b+&-a& a&tara ".at+r"ta, $a&
4"/o-a&-#"o&o,",*
A-a&-#"o&o,", $a& 4"/o-a&-#"o&o,", 5a&- $"$a/atka& $a/at
bera,a# $ar" 7a,k+#ar ata+ &o&7a,6+#ar* 3a&- ter.a,+k
/e&5ebab &o&7a,6+#ar a$a#a4 "&fek," Tr5/a&o,o.a 6r+!"
:/e&5ak"t C4a-a,; $e>,"e&," 7"ta."& B1 "&fek," kro&",
,e/ert" T+ber6+#o,",* Ker+,aka& ",ke."k /a$a ,e# -a&-#"o&
kare&a a#"ra& $ara4 5a&- "&a$ek+at a#"ra& $ara4 /a$a
,e-.e& ter,eb+t ak"bat t"&$aka& /+## t4ro+-4 ,e6ara
S%e&,o& D+4a.e# ata+ Soa7e*
Se6ara -e&et", /e&5ak"t H"r,64,/r+&- "&" ber,"fat 4etero-e&
$a& $"keta4+" ter$a/at bebera/a $efek ber#a"&a& 5a&-
.e&".b+#ka& ak"bat 5a&- ,a.a* Sek"tar 0 ka,+, ter8a$" ak"bat
.+ta," $" -e& RET $a& #"-a& RET kare&a .er+/aka& 8a#+r ,"&5a#
5a&- $"/er#+ka& +&t+k .e.be&t+k /#ek,+, ,araf ."e&ter"k+,*
Ba&5ak ka,+, ,",a&5a ter8a$" ak"bat .+ta," $" e&$ote#"& = $a&
re,e/tor e&$ote#"&* :?;
H"r,64,/r+&- $"kate-or"ka& ber$a,arka& ,ebera/a ba&5ak 6o#o&
5a&- terke&a* T"/e H"r,64,/r+& $",ea,e .e#"/+t"
• U#tra ,4ort ,e-.e&t Ga&-#"o& t"$ak a$a /a$a ba-"a& 5a&-
,a&-at ke6"# $ar" re6t+.* • S4ort ,e-.e&t Ga&-#"o& t"$ak a$a /a$a re6t+. $a&
,eba-"a& ke6"# $ar" 6o#o&* • Lo&- ,e-.e&t Ga&-#"o& t"$ak a$a /a$a re6t+. $a&
,eba-"a& be,ar 6o#o&* • Ver5 #o&- ,e-.e&t Ga&-#"o& t"$ak a$a /a$a ,e#+r+4 6o#o&
$a& re6t+. $a& ka$a&- ,eba-"a& +,+, ke6"#*
VI* D"a-&o,",
Periode Neonatal.
A$a tr"a, -e8a#a k#"&", 5a&- ,er"&- $"8+./a" 5ak&"
/e&-e#+ara& .eko&"+. 5a&- ter#a.bat .+&ta4 4"8a+ $a&
$",te&," ab$o.e&* Pe&-e#+ara& .e6o&"+. 5a&- ter#a.bat :#eb"4
$ar" 2? 8a. /erta.a; .er+/aka& ta&$a k#"&", 5a&- ,"-&">ka&*
)+&ta4 4"8a+ $a& $",te&," ab$o.e& b"a,a&5a $a/at berk+ra&-
.a&aka#a .eko&"+. $a/at $"ke#+arka& ,e-era* Se$a&-ka&
e&teroko#"t", .er+/aka& a&6a.a& ko./#"ka," 5a&- ,er"+, ba-"
/e&$er"ta /e&5ak"t H"r,64,/r+&- "&" 5a&- $a/at .e&5era&-
/a$a +,"a ka/a& ,a8a &a.+& /a#"&- t"&--" ,aat +,"a 2? ."&--+
.e,k"/+& ,+$a4 $a/at $"8+./a" /a$a +,"a 1 ."&--+* Ge8a#a&5a
ber+/a $"are $",te&," ab$o.e& fe6e, berba+ b+,+k $a& $",erta"
$e.a.* S%e&,o& .e&6atat 4a./"r 1= ka,+, H"r,64,/r+&-
$ata&- $e&-a& .a&"fe,ta," k#"&", e&teroko#"t", ba4ka& $a/at
/+#a ter8a$" .e,k" te#a4 $"#ak+ka& ko#o,to."*
12
 
Bebera/a .e&-a#a." ko&,t"/a," .e&eta/ .e&-a#a."
/er+ba4a& /a$a /o#a .aka& /er+ba4a& .aka& $ar" ASI .e&8a$"
,+,+ /e&--a&t" ata+ .aka&a& /a$at* Pa,"e& $e&-a& /e&5ak"t
4"r,64,/r+&- $"$"a-&o,", kare&a a$a&5a r"%a5at ko&,t"/a,"
ke.b+&- berat $a& /er+t ,e/ert" to&- .a,,a fae,e, .+#t"/e#
$a& ,er"&- $e&-a& e&tero6o#"t", $a& $a/at ter8a$" -a&--+a&
/ert+.b+4a&* Ge8a#a $a/at 4"#a&- &a.+& bebera/a %akt+
ke.+$"a& ter8a$" $",te&," ab$o.e&* Pa$a /e.er"k,aa& 6o#ok
$+b+r ,/4"&6ter a&" teraba 4"/erto&+, $a& rekt+. b"a,a&5a
ko,o&-*2
U.+.&5a $"are $"te.+ka& /a$a ba5" $e&-a& /e&5ak"t
4"r,64,/r+&- 5a&- ber+.+r k+ra&- $ar" = b+#a&* Har+, $"/"k"rka&
/a$a -e8a#a e&tero6o#"t", $".a&a .er+/aka& ko./#"ka," ,er"+,
$ar" a-a&-#"o&o,",* Ba-a".a&a/+& 4+b+&-a& a&tara /e&5ak"t
4"r,64,/r+&- $a& e&tero6o#"t", .a,"4 be#+. $".e&-ert"* D".a&a
bebera/a a4#" ber/e&$a/at ba4%a -e8a#a $"are ,e&$"r" a$a#a4
e&tero6o#"t", r"&-a&* 2
E&tero6o#"t", ter8a$" /a$a 12' /a$a /a,"e& $e&-a&
/e&5ak"t 4"r,64,/r+&-* Ha# "&" kare&a ,ta,", fe,e, .e&5ebabka&
",ke."a .+ko,a# $a& "&7a," bakter" 8+-a tra&,#oka,"* D",erta"
/er+ba4a& ko./o&e& .+,"& $a& /erta4a&a& .+ko,a /er+ba4a&
,e# &e+roe&$okr"& .e&"&-kat&5a akt"7"ta, /ro,ta-#a&$"& E1
"&fek," o#e4 C#o,tr"$"+. $"6"#e ata+ Rota7"r+,* Pato-e&e,",&5a
.a,"4 be#+. 8e#a, $a& bebera/a /a,"e& .a,"4 ber-e8a#a
%a#a+/+& te#a4 $"#ak+ka& 6o#o,to.5* E&tero6o#"t", 5a&- berat
$a/at ber+/a to"6 .e-a6o#o& 5a&- .e&-a&6a. 8"%a* 3a&-
$"ta&$a" $e&-a& $e.a. .+&ta4 ber"," e./e$+ $"are 5a&-
.e&5e./rot $",te&," ab$o."&a# $e4"$ra," $a& ,5ok* U#,era,"
$a& &ekro,", ",ke."k /a$a .+ko,a 5a&- ber-a&-#"o& $a/at
.e&-ak"batka& ,e/,", $a& /erfora,"* Ha# "&" 4ar+,
$"/ert".ba&-ka& /a$a ,e.+a a&ak $e&-a& e&tero6o#",",
&e6rot"6a&* Perfora," ,/o&ta& ter8a$" /a$a = /a,"e& $e&-a&
1=
 
/e&5ak"t 4"r,64,/r+&-* A$a 4+b+&-a& erat a&tara /a&8a&- 6o#o&
5a&- a-a&-#"o& $e&-a& /erfora,"*
Ga.bar * Foto /a,"e& /e&$er"ta H"r,64,/r+&- ber+,"a = 4ar"*
 Ter#"4at ab$o.e& ,a&-at $",te&," $a& /a,"e& ke#"4ata& .e&$er"ta
,eka#"*:1;
Periode anak 
Pa$a a&ak 5a&- #eb"4 be,ar -e8a#a k#"&", 5a&- .e&o&8o#
a$a#a4 ko&,t"/a," kro&", $a& -"!" b+r+k :failure to thrive;* Da/at
/+#a ter#"4at -eraka& /er",ta#t"k +,+, $" $"&$"&- ab$o.e&* "ka
$"#ak+ka& /e.er"k,aa& 6o#ok $+b+r .aka fe6e, b"a,a&5a ke#+ar
.e&5e./rot ko&,",te&," semi-liquid  $a& berba+ t"$ak ,e$a/*
Pe&$er"ta b"a,a&5a b+a&- a"r be,ar t"$ak terat+r ,eka#" $a#a.
bebera/a 4ar" $a& b"a,a&5a ,+#"t +&t+k $efeka,"* :1;
1?
 
Ga.bar * Foto a&ak 5a&- te#a4 be,ar ,ebe#+. $a& ,e,+$a4
t"&$aka& $e>&"t"f be$a4* Ter#"4at ,tat+, -"!" a&ak .e.ba"k
,ete#a4 o/era,"*:1;
VII. Pemeriksaan penunjang
Foto polos abdomen (BNO)
S+#"t +&t+k .e.be$aka& a&tara $",te&," ko#o& $e&-a&
$",te&," /a$a +,+, ke6"# 8"ka 4a&5a .e#a#+" foto /o#o, ab$o.e&*
O#e4 kare&a "t+ 4ar+, $"#ak+ka& /e.er"k,aa& ra$"o#o-" #a&8+ta&
+&t+k .e&$"a-&o,a /e&5ak"t "&"* Pe.er"k,aa& $e&-a& bar"+.
e&e.a a$a#a4 /e.er"k,aa& 5a&- terba"k +&t+k .e#"4at ob,tr+k,"
5a&- $",ebabka& o#e4 /e&5ak"t H"r,64,/r+&- "&"* :';
Pemeriksaan barium enema
Pe.er"k,aa& 5a&- .er+/aka& ,ta&$ar $a#a. .e&e-akka&
$"a-&o,a H"r,64,/r+&- a$a#a4 bar"+. e&e.a $".a&a aka&
$"8+./a" = ta&$a k4a,
1* Ta./ak $aera4 /e&5e./"ta& $" ba-"a& rekt+. ke /rok,".a#
5a&- /a&8a&-&5a ber7ar"a,"
1
/e&5e./"ta& ke ara4 $aera4 $"#ata,"
=* Ter$a/at $aera4 /e#ebara& #+.e& $" /rok,".a# $aera4
tra&,","
A/ab"#a $ar" foto bar"+. e&e.a t"$ak ter#"4at ta&$ata&$a k4a,
/e&5ak"t H"r,64,/r+&- .aka $a/at $"#a&8+tka& $e&-a& foto
rete&," bar"+. 5ak&" foto ,ete#a4 2??' 8a. bar"+. $"b"arka&
.e.ba+r $e&-a& fe6e,* Ga.bara& k4a,&5a a$a#a4 ter#"4at&5a
bar"+. 5a&- .e.ba+r $e&-a& fe6e, keara4 /rok,".a# ko#o&*
Se$a&-ka& /a$a /e&$er"ta 5a&- b+ka& H"r,64,/r+&- &a.+&
$",erta" $e&-a& ob,t"/a," kro&", .aka bar"+. ter#"4at
.e&--+./a# $" $aerea4 re6t+. $a& ,"-.o"$*:1;
Ga.bar (* Pe.er"k,aa& bar"+. e&e.a .e&+&8+kka& !o&a
tra&,","* o&a "&" .er+/aka& tra&,"," $ar" $"#ata," +,+, 5a&-
b"a,a&5a $""&er7a," &or.a#* :(;
Ga.bar '* Pe.er"k,aa& bar"+. e&e.a /a$a /e&$er"ta $e&-a&
/e&5ak"t H"r,64,/r+&-* Ta./ak rekt+. 5a&- .e&-a#a."
/e&5e./"ta& $"#ata," ,"-.o"$ ,erta /e#ebara& $" ba-"a& ata, $ar"
!o&a tra&,","* :1;
Ga.bar @* o&a tra&,"," 5a&- k4a, ta./ak $"#ata," $" a&tara
ko#o& 5a&- ter"," .a,,a fe,e, $"ba-"a& ata, $a& rekt+. 5a&-
re#at"f .e&5e./"t $" ba-"a& ba%a4* :@;
1(
 
Ga.bar 10* Rekt+. /a$a ba5" bar+ #a4"r "&" ke#"4ata& #eb"4 ke6"#
$ar" ,"-.o"$ $a& ko#o& $e,6e&$e&, teta/" t"$ak ter$a/at !o&a
tra&,"," 5a&- 8e#a,* :@;
Ga.bar 11* Pe.er"k,aa& $e&-a& ko&tra, :bar"+. e&e.a; /a$a
ba5" #a"&&5a .e&+&8+kka& ,e-.e& a-a&-#"o&"k 5a&- "re-+#er $a&
.e&-a#a." ,/a,.e* :@;
/a$a foto bar"+. e&e.a ,"," #atera#*:10;
Se.ak"& #a&8+t +,"a /a,"e& ,aat ter$etek," /e&5ak"t "&"
.aka ,e.ak"& 8e#a, /erbe$aa& 5a&- ta./ak a&tara +,+, 5a&-
&or.a# $a& ab&or.a#*:';
Ga.bar 1=* Pe.er"k,aa& bar"+. e&e.a /a$a ba5" bar+ #a4"r
$e&-a& /e&5ak"t H"r,64,/r+&-* B"a,a&5a /er+ba4a& k#a,"k $ar"
/e&5ak"t "&" t"$ak be-"t+ 8e#a, /a$a /er"o$e &eo&ata#*:@;
1@
Ga.bar 1?* Pe.er"k,aa& bar"+. e&e.a 5a&- $"#ak+ka&
,e#a&8+t&5a .e./er#"4atka& -a.bara& .e-ako#o& 5a&- t"/"ka#
!o&a tra&,"," ,erta ba-"a& a-a&-#"o&"k 5a&- t"$ak .e#ebar*:@;
Ga.bar 1* Pe.er"k,aa& bar"+. e&e.a /a$a ,eora&- /r"a
.+$a $e&-a& /e&5ak"t H"r,64,/r+&- t"/e ,e-.e& /e&$ek* Pr"a
"&" .e&-a#a." ko&,t"/a," kro&", 5a&- ber#a&-,+&- ,e/a&8a&-
4"$+/&5a* Per4at"ka& a$a&5a $"#ata," +,+, be,ar $a& re,"$+
fe,e,* :@;
20
Ga.bar 1* Pe&5ak"t H"r,64,/r+&-* Pe.er"k,aa& bar"+. e&e.a
ta./ak /e&-+ra&-a& ka#"ber rekt+. $a& $"#ata," #oo/ +,+, be,ar
$e&-a& /er.+kaa& .+ko,a 5a&- "re-+#er :$",k"&e,"a;*:10;
Ga.bar 1(* Pe&5ak"t H"r,64,/r+&- /a$a ba5" 5a&- ber+,"a
b+#a& $e&-a& r"%a5at ko&,t"/a," kro&",* Foto bar"+. e&e.a ,","
#atera# "&" .e&+&8+kka& $"#ata," /a$a ,"-.o"$ ko#o& /rok,".a#
$a& ko#o& a,e&$e&,*:11;
21
 
Pa$a ora&- $e%a,a 5a&- .e&$er"ta /e&5ak"t "&" b"a,a&5a
#e," 4a&5a terbata, /a$a ba-"a& ,"-.o"$ ko#o& ata+ rekt+.*
Pe.er"k,aa& 5a&- $"#ak+ka& /a$a /e&$er"ta $e%a,a "t+ 4a./"r
,a.a ,e/ert" $e&-a& /e.er"k,aa& 5a&- $"#ak+ka& ke ata, ba5"
"a"t+ $e&-a& /e.er"k,aa& bar"+. e&e.a* Da#a. ,+at+ ,t+$"
$"$a/atka& /e.er"k,aa& $e&-a& CT ,6a& 8+-a ber.a&faat +&t+k
.e&e&t+ka& #etak !o&a tra&,"," $ar" /e&5ak"t "&"* Ha,"# -a.bara&
CT ,6a& 5a&- $"$a/atka& 8+-a ,e,+a" $e&-a& 4a,"# /e.er"k,aa&
4",to/ato#o-", /a$a b"o/," rekt+.* :=;
Ga.bar 1'* Ga.bara& /e&5ak"t H"r,64,/r+&- $e&-a& ,e-.e&
a-a&-#"o&"k $" ba-"a& ata, rekt+. /a$a ,eora&- /r"a .+$a
ber+,"a 1@ ta4+&* AC M ascending colon DC M descending colon*
Se-.e& ko#o& 5a&- #a"& $a#a. bata, &or.a#*:=;
22
ter"," .a,,a fe,e, :/a$a a&ak /a&a4;*:=;
Ga.bar 20* Foto CT ,6a& $e&-a& ko&tra, /oto&-a& tra&,7er,a#
ta./ak $"#ata," ba-"a& /rok,".a# rekt+. ,erta ba-"a&
rekto,"-.o"$ 5a&- ter"," .a,,a fe,e,* :=;
2=
 
Ga.bar 21* Foto CT ,6a& ko&tra, /oto&-a& tra&,7er,a#* Ta./ak
!o&a tra&,"," $a& /e&5e./"ta& $" ba-"a& $",ta# rekt+.*:=;
aboratorium !tudi
CC count  Te, "&" $"#ak+ka& +&t+k .e&$etek," ter8a$"&5a
ko./#"ka," ,e/ert" e&teroko#"t", 5a&- $",ebabka& o#e4 /e&5ak"t
H"r,64,/r+&-* Pe&"&-kata& !C count   ata+ ba&$e."a 4ar+,
$"6+r"-a" ter8a$"&5a e&teroko#"t",*:(;
Anorektal manometri
Pa$a a&ak ber+,"a #eb"4 #a&8+t $e&-a& ke#+4a& ,e.be#"t
kro&", $a& r"%a5at at"/"ka# ba"k +&t+k /e&5ak"t H"r,64,/r+&- ata+
ko&,t"/a," f+&-,"o&a# .a&o.etr" a&orekta# $a/at .e.ba&t+
$a#a. .e.b+at $"a-&o,",* A&aka&ak $e&-a& /e&5ak"t
H"r,64,/r+&- -a-a# +&t+k .e&+&8+kka& ree re#ak,a," /a$a
,/"&kter a&" "&ter&a $a#a. .e&a&--a/" "&a," ba#o& $+b+r* :(;
Pe.er"k,aa& "&" $"#ak+ka& +&t+k .e#"4at reek, a&orekta#
/a$a /a,"e& 5a&- $"6+r"-a" $e&-a& /e&5ak"t H",64,/r+&-* Ora&-
5a&- .e&$er"ta /e&5ak"t "&" b"a,a&5a aka& ke4"#a&-a& ata+
berk+ra&- reek, a&orekta#&5a* Pe&+r+&a& reek, a&orekta#
2?
 
5a&- $".ak,+$ka& a$a#a4 k+ra&-&5a re#ak,a," /a$a ba-"a& a&+,
,ete#a4 $"#ak+ka& "&a," ba#o& $" ba-"a& rekt+.* Ba-a".a&a/+&
ter$a/at ba&5ak /erbe$aa& /e&$a/at te&ta&- /e&"#a"a& /a$a te,
$"a-&o,t"k "&"* :12;
Biopsi rektum
B"o/," rekt+. .er+/aka& te, 5a&- /a#"&- ak+rat +&t+k
.e&$etek," /e&5ak"t H"r,64,/r+&-* Dokter .e&-a.b"# ba-"a&
,a&-at ke6"# $ar" rekt+. +&t+k $"#"4at $" ba%a4 ."kro,ko/* A&ak
a&ak $e&-a& /e&5ak"t H"r,64,/r+&- t"$ak .e."#"k" ,e#,e#
-a&-#"o& /a$a ,a./e# 5a&- $"a.b"#* Pa$a b"o/," 4",a/ 8ar"&-a&
$"ke#+arka& $ar" ko#o& $e&-a& .e&--+&aka& a#at /e&-4",a/*
Kare&a t"$ak .e#"batka& /e.oto&-a& 8ar"&-a& ko#o& .aka t"$ak
$"/er#+ka& a&e,te,"*
 "ka b"o/," .e&+&8+kka& a$a&5a -a&-#"o& /e&5ak"t
H"r,64,/r+&- t"$ak terb+kt"* "ka t"$ak ter$a/at ,e#,e# -a&-#"o&
/a$a 8ar"&-a& 6o&to4 b"o/," full-thickness  b"o/," $"/er#+ka&
+&t+k .e&-ko&>r.a," /e&5ak"t H"r,64,/r+&-* Pa$a b"o/," full-
thickness #eb"4 ba&5ak 8ar"&-a& $ar" #a/",a& 5a&- #eb"4 $a#a.
$"ke#+arka& ,e6ara be$a4 +&t+k ke.+$"a& $"/er"k,a $" ba%a4
."kro,ko/* T"$ak a$a&5a ,e#,e# -a&-#"o& .e&+&8+kka& /e&5ak"t
H"r,64,/r+&-*:1=;
VIII* D"a-&o,", Ba&$"&-*
Ke-a-a#a& ba5" 6+k+/ b+#a& 5a&- ,"4at .e&-e#+arka&
.eko&"+. /a$a %akt+ 2? 8a. /erta.a ,ete#a4 #a4"r $a/at
$"6+r"-a" a$a&5a ob,tr+k," /a$a +,+, ba5" ter,eb+t* D"a-&o,",
ba&$"&- +&t+k obt,r+k," +,+, be,ar a$a#a4 ,e/ert" /e&5ak"t
H"r,64/r+&- ,e&$"r" $a& bebera/a /e&5ak"t #a"& ,e/ert"
.a#for.a," a&orekta# $a& "econium #lug syndrome$ U&t+k
2
.e.be$aka& ket"-a 8e&", /e&5ak"t "&" .aka 4ar+, $"#ak+ka&
/e.er"k,aa& ra$"o#o-" 5a&- te/at* Pa$a foto /o#o, /e&$er"ta
$e&-a& ke#a"&a& "econium #lug syndrome ta./ak $",te&,"
$ar"/a$a ba-"a& +,+, ke6"# $a& +,+, be,ar 5a&- .e&-"," ,e#+r+4
ba-"a& ab$o.e& &a.+& t"$ak ter#"4at air uid level* Se.e&tara
/a$a /e.er"k,aa& bar"+. e&e.a aka& ta./ak -a.bara&
meconium plug$ Pe.er"k,aa& "&" $"kataka& .e."#"k" efek
tera/e+t"k a/ab"#a .eko&"+. ke#+ar $e&-a& ,e&$"r"&5a ,ete#a4
bebera/a %akt+ ke.+$"a&* Pa$a ,eba-"a& ba5" ,t".+#a," /a$a
ba-"a& rekt+. $e&-a& .e&--+&aka& ter.o.eter rekta#
/e.er"k,aa& rectal touch%& $a& /e.ber"a& ,a#"&e e&e.a
b"a,a&5a aka& .e&-"&$+k," ke#+ar&5a .eko&"+. tereb+t*
Ba-a".a&a/+& ba5" $e&-a& ke#a"&a& or-a&"k ,e/ert" /e&5ak"t
H"r,64,/r+&- "&" 8+-a terka$a&- aka& .e&-e#+arka& meconium
 plug  $a& ,e#a&8+t&5a aka& .e&8a$" &or.a# +&t+k ,e.e&tara*
O#e4 kare&a "&" 4ar+, $"#ak+ka& ob,er7a," ,e6ara ter+, .e&er+,
+&t+k ba5" 5a&- .e,k"/+& te#a4 .e&-e#+arka& meconium plug
.ereka$ A/ab"#a -e8a#a ob,tr+k," .e&eta/ .aka /e.er"k,aa&
#eb"4 #a&8+t 4ar+, $"#ak+ka&* :1?;
2
/a$a ,eora&- ba5" bar+ #a4"r $e&-a& "econium #lug syndrome*
:1?;
D"a-&o,", ba&$"&- ke#a"&a& "&" a&tara #a"& .eko&"+. "#e+,
ak"bat /e&5ak"t >brok",t"k atre,"a "#e+. atre,"a rekt" .a#rota,"
$+/#"ka," "&te,t"&a# $a& ,"&$ro. /,e+$o ob,tr+k," "&te,t"&a#* P+r"
:1@@(; .e&5ataka& ba&5ak ke#a"&a&ke#a"&a& 5a&- .e&5er+/a"
/e&5ak"t H"r,64,/r+&- aka& teta/" /a$a /e.er"k,aa& /ato#o-"
a&ato." ter&5ata $"$a/atka& ,e#,e# -a&-#"o&* Ke#a"&a&ke#a"&a&
ter,eb+t a&tara #a"& 'ntestinal neuronal dysplasia&
hypoganglionosis& 'mmature ganglia& (bsence of argyrophyl
 plexus& 'nternal sphincter achalasia  $a& ke#a"&a&ke#a"&a& otot
/o#o,*:1;
Stab"#",a," /e&$er"ta .e&6ak+/ ke,e".ba&-a& 6a"ra& $a&
e#ektro#"t a&t"b"ot"ka 8"ka ter8a$" e&teroko#"t", ,erta e7ak+a,"
ko#o& $e&-a& e&e.a*
2* Pe&a&-a&a& k4+,+,
 T"&$aka& be$a4 $"#ak+ka& ko#o,to." $a& ke.+$"a& $"#a&8+tka&
$e&-a& /e.be$a4a& $e>&"t"f* :1;
Ke,"./+#a&&5a ,e#a"& ka,+, ba5" ,e4at $e&-a& ,e-.e&
a-a&-#"o&o,", 5a&- /e&$ek o/era," .er+/aka& /"#"4a& tera/"
5a&- terba"k +&t+k $"#ak+ka&* Ba-a".a&a/+& b"a,a&5a ,ete#a4
/ro,e$+r o/era," "&" kea$aa& ko#o& teta/ $a#a. ke$aa&
ab&or.a# :k+ra&- ba"k; $a& 4a,"# /e&a&-a&a& o/era,"
,e#a&8+t&5a aka& #eb"4 ber7ar"a,"* :1(;
* Ko./#"ka,"
2(
/e&5ak"t H"r,64,/r+&- $a/at $"-o#o&-ka& ata, kebo6ora&
a&a,to.o,e ,te&o,", e&teroko#"t", $a& -a&--+a& f+&-,"
,/"&kter* Se$a&-ka& t+8+a& +ta.a $ar" ,et"a/ o/era," $e>&"t"f 
a$a#a4 .e&5e#e,a"ka& ,e6ara t+&ta, /e&5ak"t H"r,64,/r+&-
$".a&a /e&$er"ta .a./+ .e&-+a,a" $e&-a& ba"k f+&-,"
,/"&kter a&" $a& ko&t"&e&* :1;
Da"tar Pustaka
1* B+$" Ira%a& Bab 1 $a& Bab 2 $a#a. Pe&-a.ata& f+&-,"
a&orekta# /a$a /e&$er"ta /e&5ak"t H"r,64/r+&- /a,6a o/era,"
/+## t4ro+-4 *Ba-"a& "#.+ be$a4 fak+#ta, ke$oktera&
2'
1=?('@1011 $a& 1*
2* Sa.+e# N+rko )D )PH )irschprung *isease  $a#a.
A.er"6a& )ot"#"t5 So6"et5 :A)S; a&$ t4e I&ter&at"o&a#
Fo+&$at"o& For F+&6t"o&a# Ga,tro"&te,t"&a# D",or$er, :IFFGD;
=* H5e "& K". )D A4 3o+&- K".)D C4oo&- ok Lee )D
C4a&- S"k 3+ )D+&- S+& K". )D P5o N5+& K".)D )oo&
Ca5+ Lee )D a&$ H5+& K%o& Ha )D *H"r,64/r+&- D",ea,e
a&$ H5/oa-a&-#"o&o,", I& A$+#t,* )a5 200'*
?* K+.ar Abba, a&$ Fa+,to )"t64e## C4a/ter 1 *evelopmental
 (nomalies $a#a. Rob"& Pat4o#o-"6 Ba,", of D",ea,e 't4 E$"t"o&
200* Ha#a.a& 01*
* P+r" a&$ )*Ho##%art4 $a#a. Pe$"atr"6 S+r-er5* S/r"&-erVerb5
Ber#"& 200* Ha#a.a& 2(*
* Fra&k H* Netter )D At#a, of Netter ?t4 E$"t"o& 200* P#ate
=12 P#ate =@ /#ate =(1 $a& /#ate ='
(* Ho##5 L Ne7"##e )D C4"ef E$"tor Car.e& C+ar" )D* Pe&5ak"t
H"r,64/r+&- Pe$"atr"6 +/$ate$ o& +# 1= 2010** D"+&$a4
%%%*e.e$"6"&e*6o.
'* Pe$"atr"6 S+r-"6a# Prob#e. C4a/ter 1'*Co#o& a&$ Re6ta#
S+r-er5*)ar%"& L*Cor.a&* E$"," ke * L"//"&6ott "##"a., a&$
"#k"&, 200* Ha#a.a& @ $a& 0*
@* Pe$"atr"6 Ra$"o#o-5 C4a/ter 2 Pe$"atr"6 Ab$o.e& a&$
Pe#7",F+&$a.e&ta#, of D"a-&o,t"6 Ra$"o#o-5 $a#a. =r$ E$"t"o&
$"t+#", o#e4 "##"a. E* Bra&t )D FACR $a& C#5$e A* He#., )D*
Ha#a.a& 12@=*
10* C"ro 3o,4"$a r )D H"r,64/r+&- D",ea,e I.a-"&- $a#a.
)e$,6a/e Referre&6e Dr+-* D",ea,e a&$ Pro6e$+re +/$ate$
o& )a5 22011* D"+&$a4 $ar" %%%*e.e$"6"&e*
.e$,6a/e*6o.
11* Tere,a Berro6a# )D )a&+e# La.a, )D +#"a G+t"erre!
)D I,abe# Torre, )D Co&,+e#o Pr"eto )D a&$ )ar"a L+",a
2@
 
$e# Ho5o )D* Co&-e&"ta# a&o.a#"e, of t4e ,.a## "&te,t"&e
6o#o& a&$ re6t+.* D"+&$a4 $ar" Ra$"o-ra/4"6,*r,&a*or-*
Se/te.ber 1@@@*
12* A#berto Pe&a $a& )ar6 A Le7"tt S+r-"6a# T4era/5 of 
H"r,64/r+&- D",ea,e $a#a. Co&,t"/at"o& Et"o#o-5 E7a#+at"o&
a&$ )a&a-e.e&t* D"t+#", o#e4 Ste7e& e&er $a& Grae.e S*
D+t4"e* S/r"&-er Ver#a- Lo&$o& L"."te$ 200* Pe$"atr"6
S+r-"6a# Prob#e. C4a/ter 1' $a#a. Co#o& a&$ Re6ta# S+r-er5
$"t+#", o#e4 )ar%"& L*Cor.a&* E$"," ke * L"//"&6ott "##"a.,
a&$ "#k"&, 200*
1=* Pe&ata#ak,a&aa& Pa,"e& $e&-a& /e&5ak"t H"r,64/r+&-
$"+&$a4 $" %%%*"&foke$oktera&*6o.*
1?* Vera Loe&"&-Ba+6ke )D a&$ Ke& K".+ra)D Fa"#+r to
Pa,, .e6o&"+. D"a-&o,"&- Neo&ata# I&te,t"&a# Ob,tr+6t"o&
1@@@ $"+&$a4 $ar" %eb,"te %%%*A.er"6a& Fa."#5
P45,"6"a&*6o.
1* )e-a6o#o& Ko&-e&"ta#H"r,64/r+&- D",ea,e 2010 $"+&$a4
$ar" %eb,"te %%%*"&foke$oktera& UG)*6o.*
1* A#/4a Far$a4 A IG*) Re!a G+&a$" Ra&+"& S+#a8a&to )arto
S+$ar&o Pe&5ak"t H"r,64/r+&- 2011 $"+&$a4 $ar"
%%%*/e$"atr"6*6o.*
1(* o& A* Va&$er4oof A&$ Ro,e.ar5 * 3o+&- C4a/ter 1=0
H"r,64/r+&- D",ea,e $a#a. C+rre&t Pe$a"tr"6 T4era/5 1't4
E$"t"o&* Sa+&$e5 200*