54 ~o}21:i1J- : All 8l':! All 1 ~ 2002';1 Vo l. 8. No. 1. June 2002

Hirschsprung's Disease~ {}~ {l B-

Differential Diagnosis of Hirschsprung's Disease

Soo-Young Yoo, M.D.

Department of Surgery, Yonsei University Wonju College of Medicine Wonju, Korea

Hirschsprung's disease (Hn) is usually diagnosed in the newborn period and early infancy. The common presentation of HD in newborns consists of a history of delayed passage of within the first 48 hours of life. The differential diagnosis in newborns is one of the clinical challenges of this disorder. A number of medical conditions which cause functional obstruction of the intestines are easily excluded. Neonates with meconium , meconium plug syndrome, distal ileal atresia and low often present in a manner similar to those with HD in the first few days of life. Abdominal radiographs may help to diagnose complete obstruction such as intestinal atresia. Microcolon on contrast enema can be shown in cases with total colonic aganglionosis, ileal atresia or meconium ileus. Suction rectal biopsy or frozen section biopsy at operation is essential for differential diagnosis in such cases. HD is also considered in any child who has a history of constipation regardless of age. Older children with functional constipation may have symptoms that resemble those of HD and contrast enema is usually diagnostic. However, children with other motility disorders generally referred to as chronic idiopathic intestinal pseudoobstruction present with very similar symptoms and radiographic findings. These disorders are classified according to their histologic characteristics.; visceral myopathy, visceral neuropathy, in­ testinal neuronal dysplasia (IND) , hypoganglionosis, immature ganglia, internal sphincter achalasia. Therefore, the workup for motility disorders should include rectal biopsy not only to confirm the presence of ganglion cells but also evaluate the other pathologic conditions. (J Kor Assoc Pediatr Surg 8(1):54-61), 2002.

Index Words: Hirschsprung's disease, Differential diagnosis, Motility disorder

AJ-Q.] {l~~A~]~ -¥-A~oJ] Q.]~ AJ--&-% AJ-0~ £ ~J,~~~ 7] oj] ~7,1~ A]1i!- ~~71] ;

I Delayed meconium passing I Dysmotility Disorders I l (Chr. idiopathic intestinal pseudo-obstruction) IMechanical obstructionsl Functional obstructions I I T • Meconium ileus • Prematurity • Meconium plug syndrome • Small left colon syndrome • Distal ileal or colonic atresia • Sepsis & electrolyte imbalance •Visceral myopathy ·Intest neuronal dysplasia • Low imperforate anus • Intestinal neuronal dysplasia •Visceral neuropathy • Hypoganglionosis • Achalasia of anal sphincter • Drug~nduced •Immature ganglia • Hypothyroidism •Chagas 'sdisease ·Int. sphincter achalasia

Fig . 1. Differntial diagmosis of Hirschsprung's disease in Fig . 2. Differntial diagmosis of Hirschsprung's disease in neonates. other age groups.

Pi~ :~:!A%oJ1 A1 ~-3] {lA}AJ- {l~~AiL'V } ~A~ ~oJl s:. ~~ ~~ 7-J-llll ~.2-s!- t-J--'foi ~ 'T 'U.2- u9 (J'\:J 1), ~tl 71~~ i5}Jl 7-J--8:-% 7-J- on7} 71t:11 ~7-J-% ~ ?ll w 7-js:.S!- {lztw ~-9- 7-J-lll]~%% 1:I~A11i5} 1l] ~q1 {l)'~0 } 7 1 oJl 71111~ i5}lf- AJ-:t!- lll] oJ]-c 01 oj] t:11 ~ {ltl~% ~{li5] Aji5}71 oiD1-8:- ~-9-7 } t:11 lf­ ~% ~.2-1 1 -c ~~~ J %fj-7} PiAl ?J-.2-E.s!- ~~ ZltloJl {f°lq. rrj- c.}A1 o ] ~ ~~~ ~~ q OJi5}oj 7}Aj lID (pseu­ ~ oiD1%~ ~q . 7 ] 11 ] ~ 7-J-llll ~%%- Ejj1(!).j 7-J-lll] ~% doHirschsprung's disease), ;,}).j ~ l~Fj 7-J- 7H:llll] ~% (meconium il eus)~ ~ 7-J- wtllf-oJjAi Ejj 1(! oJl Qj ~ AJ-lll] ~ 01 (chronic idiopathic intestinal pseudoobstuction; eIPS), 1(! % ~oit-J-u9 t:11 4'-{f0] l..-J-.±Aj --ilil-% ().Qj ~ ~ 3 lID (variant lID), -~A lID (allied lID) .2i oJl s:. ~-3]~:jAJ­ AJ- %AJ-.2-S!- t-J-s}t-J--c lfrq1 oJ1 E~ 1(! ).j ~ ~ %~-r (me­ ~).j % rrj-c.} 7-J-{l ~ tl!f1 ~ ).j ~-o~ (intestinal neuronal dys­ conium plug syndrome)~ AJ-t~ ~ AJ-J!} :9il t~ ~7-J- If-!f1 oJl Ell

plasia; IND), {l7cl7-JAi] '±~~% (hypoganglionosis), o ] ).j ~ 1(!0] ~} 'U-c ~:j% .!i!.0]u9 't.±).j --ilil-%J!} '?1:t!-).j~ ~~ {l ~ ~ (immature ganglia), 7-J-{l7cl ~ If-~ % (intestinal -3i.2-S!- .!i!. Jl~Jl ~J. 4 -7 . -9- t.1 t-J- c.} oJ]Ai -c Ejj~).j 7-J-lll]~ % o] dysganglionosis), l.·W~-Q.j:-2- ° 1~~~% (internal sphincter 't.±-"j 1lil-%J4 '?1:t!-~oi ~A~¥l ~-9--c °1-31 .!i!.Jl'fi e}7} achalasia), 7-J-{l ~ tl!f1 71 ~ (intestinal neuronal malfor­ ~.2-t-J- \t.±-"j 1lil-%J!} .!j!-:t!-i5}11 ] ~A~~-C Ejj ~).j 7-J-lll] ~%

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~q. s:. i5}Jl ~7-J-.Qj e}t.1% ~OJ~ AJ- ~~7-J- (microcolon)Qj ~ Ejj~ %% .!i!.0171 rrJl~oJl %~7-J- .!j!-{l~7-J Ail.±%J!} ~i51 )} ~ % li ~ q. ~!f1lf- ~ AJ- .!j!-i?-% s:. %~ 7-J- .!j!-{l7cl ~ Ail'± % J!} il-A } ~ ~ Ejj ~ .!i!.°171 rrJl~ oJ] -3]7-J- ~-3] {lA}t-J- 7~ -l!},A l {lA~0 } 7 ]oJl ~:j~-c lID ~ ~{l~ ~A~~ Ejj 1(! lJ n ~~ 'T~ ~:j ~-C ~7-J-~ ~-3] {lA}S!- ~ ~ % lii5}ll] ~ u9 ~ Al'?1 ~ -l!}lf- ll,I);'}.2-S!- ).~~ 48,A1Zl- °VJ- Ejj ~ lJ ~ ~Qj Al'?1 %If- -'fi?-% %lfr~ 15J-~ 317-J- 7 1 ~Qj ~-9-s:. i5}lf- ~7-J- Qj 0] 'U% ~-9- lID Q} ~ ~~% lii5}ll] 'fiq. AjAJ- AnEn 7]Zl­ lIDQ} )}~o ] ~lii5}t-J- 01-c t:11 7n ~ % lf-.Qj ~Ejj~ ~ ~~.2- oJl ~A~~ AjAJ-O } ~ 7cl-9--c 94 % Ajs:.7} A~~ 24,A]Zl- 01L11 S!- )}~0 1 %o ] ~ ~o l q. oJl Ejj 1(! % lJn ~ i5}11] ~ u9 48,A 1Zl- L11 oJ]-c 7-1 Qj Y ~ {l A~ ° }oj ] A1 Ejj 1(! lJn ~ ° 1 ~ oi t-J--c lfr q1 oJl1 An Ejj 71 Zl- 33i'- ° lAJ- '?l 7cl -9- 80%, 29-32i'- 65%, 28i'- 0li5}cJVi -c 57%;'}0] A~~ 3~ L11 0Jl Ejj1(!% lJn ~i5} -c -3i.2-S!- .!i!.Jl~Jl 'Ucf {lA~0 } 7] oJl 7-J- {l-,,~0 } 71 01 ~Qj )}~ Zltl~ 7 1 111 ~ 7-J-llll~ % .!i!.q-c 1111~ % ~.2-~ 'T 'U -c ~ '?l.2-S!--c 311] 7111] ~ 7-J-llll ~ J!} 7] 7-J-.Qj 7 1 ~~ -8:-% ~-on (dysmotility di sorder )~ ~.2--1 ] -C ~ 56

%%JJj-Qj {}~ ~li±o] ~xt} q (:::Lii:J 2). 7 ] .'g-~ ~% ~JoH S£:-i::- %"'} HD13~ ~~t} il :::L ::t.-3j~~ ~AcJ011 u:j-ct ~7 } 01] QjtH ~J}ijl~ '6'-AJ ol 1114i- ~ 78Jf- %-fJ'~~~ ~Acl ~~ 7.1 ~~~~ T*t}il ~q. J-cl 7Fcl ~J}ijl ~ '6'-4-r 0]c.J- ~~t}9 ~~L}8 ~~011-i::- 0] 7t. AJ {l7f:l\1~ ~J-dAJ-()fl (IND) : IND-i::- 1971~ 14 %~ ~AH1~ ~ S£:-i::- ~eJ5:-3j~-3:j l:~~~ **t}9 ~~ Meier-Ruge 011 QjtH ;iH- l:7H ~ ~~.2..~ ~cJ 5:-3j~~ t }il ~q?, l0 l:~011 utc.J- Type A, B ~ ~* ~q 01 n IND~ ;illi.-c Jlj-71 HD9-} %"'}t} 711 'T~~ ;iP17r ~xt}qil A~Zf~~~ 1. ~~ ~~ (degenrative disorders) L} ~~ .!i!.il011 ut.s1i'! INDQj 7f:lJf--i::- HD011 tll3Jl l A11 °l AJ 16 7r. tlJAJ- -2'op.J-%- (visceral myopathy) : Sfl ~H!- ~% 011"'1 ~li±~-i::- 78Jf-7} 50 % 01 AJ olil ,J7 '15- AJ ol H0011 tll'8'H ~Qj ~Acl011 QjtH ~~o] {l%5:-3j:& ~1i'!A1 ~'\~~-i::- ~~ 78 ul t}E.. ~ ~J {l 78::t.-3j %01 % [:1 Ad4i-~-i::- "']71lJ}7.1 'T ~ ~~ 0]C1~ ~%~ ~J-cl°l llJ%lJ}7.] {l~~ 78Jf- megacy­ % "'Pf~ ~X7 } ~qil t}~q. Gillick %-&16 33oJl~ IND stismicrocolon-intestinal hypoperistalsis synclrome~ ~~7 1 %A}'15- 21oJl(64%)7r 'T~ ~o l ~]%~ ~qil .!i!.ilt}~q. 711 ~qll. 1-}. {l7f:l1i 1JL~%- : {l781iA11 .¥.7} ~AH'O}~ :::L 'T7} {} Y-. tJlAJ- {l~oP'J-%- (visceral neuropathy) : 1:J1 7H A}OJ l:~o1 ~qE-Al S£:-i::- ulAcJ4i- {l781i A11 '¥'7 } L}E.}L}-i::- 78Jf­ 4- lJL:tJAJ ~~~-i::- ~~~~ l::&71-t!- Qj ~J~ {l78% %01]"'1 HD9-} %",}~ %-AJO] L}E.}'€t 'T ~q. 12 (myenteric plexus)Qj ~J-cl°] L}E.}L} -i::- [11 {}~ q~ Acl 1.-]1* q.. 1.-]1~~ -2' °l~~ :og.%- : 5:-3j~~~~-i::- ~l:{l:&~ tll {l'\~% type 2 9-} '?:!-t!-~o1 L}E.}L}71s:. ~q. (nitregeric) {l7f:l ~ {j 01 ~.2..1i'!"'1 tJ-lf--3j AJ ~ 11l~AJ ~!A ~ q.. ~% 'it% : ~1i'!-'11A1N ~t} -'11 Qj Jlj-s:.~ 'J%~~ 1-H 1.-]1 ~~~ °1~01 <{!- L}E.}L} -i::- ~~.2..~ 1.-]1~~~ 1i7H~ J-l ~J ~~Acl° ] i cJl~o1 ~'\~~q. i'i~J- l '15-AJ ~ ~~% 711:J1w 'T ~q. 4. Chagas ~ ~ : Jf-eJ L}c.J-011 -i::- 0}-3j .!2..il'El tl} ~.2.. 4. ~~-c °P'J ~ (smooth muscle disorder) : 01 ~~. L} Try]Xll1osorna cruzi 011 7f}~ ~ 78 Jf- ~J~{l78 %Qj ~ ~ ~ -& 1.-]1~J ~~~'6'- Jlj- %"'}t }r9 ~1 li.7 } ~7} .'g-tH 1:J1 7H ",}nJ i cJl t}9 :::L lJL*Qj 1JL*~ ~~J~ ~.2..71r9 ~J~% ~JoH~ oJl 01.s711 -Bq.

3. ~~~ ~1:11 2. ~% HD .9:e ~A~ HD HD~ "T- '15- AJ ~ t }1-}7} ~l:l1'15-0]71 uJl-lf-oJl q0J:~ ~~ HD9-} uH-9- %A}~ '15-AJ~ L}E.}1.-]11-} HD9-} q~ 1:1-& ::t. Qj ~tll '15- Jlj- {}~% xt}-i::-[11 ~Ad ~l:l]'15--& .ilL~ ~~.2..~ -3j :tJAPJ {l781i A11.¥.-i::- ~AHt}~ ~J~~o l L} {l78% ::t.-3j o ] %~-i::- l:o} ~A}~ ~ 3 %7r 0]01] tHrt~ ~js:.~ uHJf­ 011 9 cl q~ ~ EllQj ~~~ L}E.}1.-]1 -i::- ~~%% ~~ HD IO ~~ ~%18 ~011s:. ~Tt}il :J. 7.j QjoJl 1:J1tHAfc o}-3j s:. ~~ 1JL~0 1 ~q. ~}.d ~tll '15- Qj ~~.2..~-i::- T::t.~, ,\~cJ

Table 1. Characteristics of Intestinal Neuronal Dysplasia Type A Type B Incidence < 5 % > 95% Table 2. Clinical Comparison Between Hirschsprung's Pathology Ganglion cells'+' Ganglion cells'+' Disease and Idiopathic Constipation Hirschsprung's Idiopathic Increased AchE activity Increased AchE activity Manifestations disease constipation Hypoplasia of sym. Hyperganglionosis Common innervation Soiling Unusual Stool in Unusual Common Increase parasym. Giant ganglia nerve fiber Obstuctive Sx Common Rare Symptom Neonatal period Similar with HD Enterocolitis Possible Never Obstruction Milder Sx Rectal exam. Narrow Dilated Diarrhea Contrast enema Narrowed distal Dilated rectum Bloody stool segment

5 9 HD; Hirschsprung s disease (Fadda et ai, 1983 ) (Teitelbaum et ai, 1998 ) 57

~, iH!~ %Qj qOJ~ '?lA}%O] -l!}~~.2-S'- ~%-o]-Y- ~;:;­ tll(!-oJ1 5:-%% Y'c 5::;:! 01c1- . c1-% l(!-1l1oJ)Ai A 1t~ ~ Y ~ ::r ~'?l% ~~-o] '?l y 'ict 'c ~1flAcl ~1:I ] ~0 ] 7}AJ- ~~ t;:l 'c {jAt'c ~A}~ -l!}9}-'f1 S'- ~Jl ft l;J}-¥-~ %c:j ~~ AJ-EN EHO]q. HD9.\- ~Acl 1(11:1] ~Qj O,:P'J-~ ~Acl-€:- K 2'4 ~-c}. )'1 ~ -l!}-¥- l(!-~ ~Oa% A1'i;~-o} o-~ .6j7.J- ~ S A} ~:AJ-Q) -tt71 %oa_~S'- 0 ] t~ -¥--'f1 7 }

{t:Ac)oj1 ojc:j%01 aj- E-E-S'- ~;:;- .6j:AJ- :i:.6j ~A } L-} 7 11 -l!}~A l %~~IH :i:~l{jA}S'- (t~tH0): i5}'c 78-1'-7} %% ~q. :i:.6j i:JA}011 ?lojA15:- Jlj 7i011 'c {)78~ A11~Qj %4'- ~A}7} y {!­ ~Acl 1(11:1] EE'c £\11(1 tlH~Qj ;z]~, -l!}-¥- ll§uJ %Q) HD ~AJ­ {JA} ~~ L-} 30]-2-0]] %A} HD ~ ~% ojl cH~ ~Jl7} ~ ojy­ % L-}E}1-H'c ~;z}01]Ai 7}AJ- ~1l71 ] tll -ft y ~'c ~ ~ tll(!­ T?:!Al 1\%-~ HD9}Q) ~~ ~l(!-% -'f1tll {)78~ A~1~ ~ {)7(l llHl -€:- .6j:AJ- yA] {jA}S'- yA] {jA}AJ .6jAJ41 -l:t1(1Q) ~A] Ad %Q) t;:l El1 0 11 cH ~ ,111~ ~ :i:.6j -&~ ~Acl '?:! T9} W7Jl1 T?:! ~ ~-¥-9} .6j7.JQ) ll§r1l ~-¥- %~S'- HDQ) 7}'6- Acl% E} ~tll -ft :i:.6j~-& l,:lA}%o1 ~~-ol ~t~S1Jl ~q. Y ~ rl {)),~o }Qj ~i'-'c s:.{}% iiJ~01\ 1l-0Ji;}~ AJOJA\ :AJ- 0 1l 7} ~'c ~i'-'c iiJ-~ ~3} o]y- .6jAJ- 4'--tt~Q) 7}'6- Acl % A~ZftH -ft y ~Jl s:.{l-% *-l:t-o] ~'iJ4-s:. 5:-{l- ~t:Pf-01] -l:t1(10] % oj A~ y-~A] ?J-% ~-1'-'c 7 ]1l]~ :AJ- lfj]~~o]y- HD HD 9-1 tl l(!-~ cH1jl--l:tQ) 78-1'-011 ~ ojc:j%~ 'ict ~ Y- l1A11 ~ ~ Qj{Jtll -ft y ~lq . HD l1l~0 ] fJ- -€:- ~ o]oJl ;:;-~£\oj ~ AJoj]Al 'c y~1! HDS'- tll(!--o}9JA]~ y~~ c)-~ tll(!-ol L-} % ~-1'-01] 'c 15J-~ ~{l-o ]y- y A] l,:lA}},] -l:t1(1 x} -tt7]7} ~~ -& ~-'f-% Jlj- 'T~1! HD7)- o}tg -?;I~S'- A~Zf~~ Y- y~ 4- 0] -l:t~S1 oj Y-~1?1 Ai -l!}-¥- ll§J?!-0] :§:1\ S1715:- -o}E- S'- ~~ l-ID S'- tll(!-{l 78-1'-%, ~AJ ~ AJo 1Y- ~7.J:i:°a~ ~x}7} o}

Table 3. Motility Disorders Associated with Interstitial Cells of Cajal (ICC) Motility disorders Histologic findings of ICC Author, year 13 Allied HD Lack of ICC Yamataka, 1997 23 Aganglionic segment of HD Decrease of ICC Vanderwinden, 1997 24 Colon hypomotility in Defects of ICC population Kenny, 1998 anorectal malformations Transient neonatal Delayed maturation of ICC Kenny, 199825 pseudoobstruction 26 Chronic idiopathic Abnormal distribution of ICC Yamataka, 1998 intestinal pseudo-obstruction Intestinal neuronal dysplasia Hyperplasia of ICC Jeng, 2000227 Idiopathic constipation Decrease of ICC volume He, 200028 Chagastic megacolon Deficiency of ICC Hagger, 200029 Inflammation-induced Damage to ICC network Der, 200030 motor dysfunction

HD; Hirschsprung's disease 58

Fig. 3. Diffemtial diagmosis of Hirschsprungs Disease in patients with microcolon. A. Case 1 (total intestinal aganglionosis, 5day-old female). The barium study shows microcolon (A-1) and transition zone was not found at operation (A-2). Neither ganglion cells nor nerve plexus are present in the entire small and large intestines. B. Case 2 (ileal atresia, 2 day-old male): The barium study shows microcolon similar to case 1 (B-1) and the operative finding shows ileal atresia type III-a(B-2). C. Case 3 (total colonic aganglionosis: 3day-old female): Microcolon is seen in the contrast study (C-1) and ganglion cells are absent in entire colon and the transition zone is shown in the terminal (C-2). D. Case 4 (meconium obstruction without cystic fibrosis: 2day-old male): The contrast study shows microcolon (0-1) and ganglion cells are present in the colon. The terminal ileum is filled with sticky meconium (0-2).

Fig . 4. Differntial diagmosis of Hirschsprungs Disease in patients with transition zone in the sigmoid colon at contrast study. E. Case 5 (rectosigmoid aganglionsis: 15day-old male) : The barium study shows transition zone in the sigmoid colon (E-1) which is grossly identified at operation field (E-2). F. Case 6 (Meconium obstruction: 11-day old female ): The contrast study shows a similar finding with the Case 5 (F-1). Grossly, the transition area is seen in the terminal ileum (F-2, arrow) was confirmed by frozen section biopsy and C-kit+ cells are not seen in the colon at ileostmy time. C-kit immunoreactivity (F-3b, arrow; red stained area) appears 83 days later at the time of ileostomy closure. 59

cj'. ~?1 ] 4 (..::rt(:l 3D- l,2):c ~AJ ~~~191- ~P'J '?J'B-¥-01] -¥-{l 78~ ),i].¥.~ol ~)~1{i HD~~s- ~~} ~l~J ::t.if-~ Alt~4- ).~4- 47H~ ~n I(!~ Martin ~~01 Alt~~~ct . Jt(:l 4E, F:c {l),~OHAi *-¥- ll~'il ~ [HI(! l:IH~ A]~O] 'V.Oi tl}2-]% ::t. °8 ~ A]t~ ~~ ~ojl .2.~ S-7.} ~AJ01 1 °ltE -¥-!fi~ ~ :c I-ID~ ).~Z{sq~~y. %?11 5 (Jt(:l 4E-l,2):c S-7.} ~~J01 I 01 t~-¥-!fi% ~:c ~~~ HD~ ~B¥! ~Tt!01] ~?1] 6 (Jt(:l 4F-l,2,3) .g. A}~oj] Q]{l~~1{i olt~-¥-!fi:c -T ~ ~~VJ ~ % -T ~~~u:j ~lAJ 9lB-¥-011 °lt~-¥-~l~HJ y.Ej-lJ -¥-!fi7} ~~~ y. %~~l{1 7d A}AJ ~~J ~ §l~J01] {l78~ ).i] .¥.7} ~ :AHt};i;(Jl §lAJ 9lB-¥-01] E111(! 01 ~1' ~:c l:7,j% y.Ej-1-B~J:l. El]I(!).a ~Jlljl ~~~s- ~Bt} ~~~u:j§l~J ::t.if-~0 1 Alt~~~Jl ).~ 4- 94~;Y:H §l~J- ::t.if- *~~0 1 Alt~~~J:}. ~7.}:C QcjAl \l-.¥.).a 1l*~~:c -¥-t}t};i;(~u:j %?1] 3~ p } {}7}7.]~ §l~J

Fig . 5. Differntial diagmosis of Hirschsprungs Disease in ::t.if-~ Alt~Al 7 } ~ ).il.¥.7} ~{l~Oi ~~~ y. (Jt(:l 4F-3a) patients with segmental dilatation of colon . G. Case 7 *~~ A]t~A]:C 7}~ ).i] .¥.Q] ~.¥.7} Aj).J% y.Ej-LJ1~~u:j (total colonic aganglionosis: 5-day old female): The contrast study shows segmental dilatation of transverse (Jt(:l 4F-3b) -T~4- ~J-8:-%S=- A,PJ% y.Ej-LJ1~q. Jt(:l colon (G-2). However, ganglion cells are absent in entire 5G,H:c tl}c1% ::t.°8~ AJ §dt~ ~~J01] °lt~-¥-!fi7 } 'V.:C HD colon. H. Case 8 (segmental dilatation of transeverse colon: 15-month old male): The contrast material is not s- ).~Z{~~1{i %?1 ] ~s- ~?1 ] 7 (Jt(:l 5G-1,2).g. -T~AHs:. passed through the dilated segment of transeverse colon ~"'E~AJoJl 0ltE-¥-!fi7} ~):c ~~.£ ).~z)-£l~~l+ -T~).l % (H-1) and operative finding shows isolated segmental dilatation of transeverse colon (H-2). Ganglion cells are ~AJl{1 ::t.~ ~AVJ %?,PJQ] -¥-{l78~).il.¥.~~~ §l~J::t.-'f­ present in the colon including the dilated segment. ~o ] Alt~£mJl ~?1] 8 (Jt(:l 5H- 1,2).g. §dtE ~AJ Qj ~~ .~~~~ -¥-~~~~ ~'4- ~~ ~ ~~~~~ Jt(:l 6I-KQj ).i] ~?1]:c Z{Z{ 9).il, 7).il, lO ).il¥! 'J0}~ 'il).j I(!tl]

T %A}~~Y. -T~4- ~Bol AJ olt}111 l..-}.2_:C 78.q-~01 % ~ ~ *-¥- ~'il0l ~ Oi LJ1~t};i;(~u:j tl}c1% ::t.OJ~AJ .2.~ % ~q . ..::rt(:l 3A-D:c {l )'~0 }011Ai CdAJ %AJ ~ tl}c1% ::t. 7ltJ1 ~AJO] {l~ HD~ ).~Z{t} ;i;(~ y. %?1] 9 (Jt(:l 61-1,2) OJ~AJ l:~~JQ] %AH!' l:7,j% ~;i;(~y. 7fl*~ Al t~ 4- ~ 'il S-7.} ~~Jojl °lt~-¥-!fi7} ~:c ~~~ HD ;i;(Jl ~?1] 10 B~ol Ai~ qiO..l1] y.,g- 78.q-~~ ~?1] 1 (..::rt(:l 3A-l,2).g. (Jt(:l 6J-1,2).g. ~ t~~~JQj ~J%*~om~u:j ~?11 11 (Jt(:l ~AJ ~'il 0}L1c1- l:~J ~~~]oJl ~~ -¥-{l78~ ),il.¥.~~ ~7Jl] 6Kl,2,3).g. J:l:lc1 ::t.~~~ l:7,jAJ {l78~ ).i].¥.:C ~:AHt}~ ~J ~JLJ1 ~AJt-~ %~t- A}olQ] {l78% :E~' ~~ ~~~Oi ~ Qj t-% tl}~~ ~~ ° 1l: {l78 ~ (ectopic ganglia)Qj l:7,j ~ Al ?io} LJ1 ~J {l7801AJ~01 %~ ¥! HD ~ ).~Z{~ ~~ u:j ~ ~7Jll {l78% tll4- ~ achtylcholine esterase activity Qj ~7} o}:c ~AJ ::t.if-~ Alt~4- 40~ ~011 ~1* ~7 } ~~~ {l-Aa£l l:7,j 0l ~Oi IND~ ~B ~~q . Oi ~~t};i;(q. %?1] 2 (..::rt(:l 3B-l,2):c -T~ l:7,jAJ §l~J -¥­ HD:c l:0}Ql Oi ~ ~ ~ %01Vi y. y.Ej-\t -T ~Jl tJ1-¥-~ ~~ (Type III-a)~ ~ ~l(l' ~~Jl ~?1 ] 3 (..::rt(:l 3C-l,2).g. .g. -T~~ %olt}l1] ~B£l:c 't1°1y. !fiQ] 78.q- ~~ ~0 1 7H -T~ l:7,jAJ ~~~ E11I(! Aa ~Jlljl~ ~Q] l:7,j% y.Ej-LJ1~~y. *~ Al t~4- 011 -T~ l:7,j 0ly. ::t.~~~ l:7,jojl QjiiH ~B LJ1 L-J.¥.).j Jclit~~:c -¥-t}t};i;(Jl ~~} §lAJ::t.if-~ Alt~Al ~~J aj7.1:C -T7} %% ~)~E.~ -T ~~ ~~J ::t.~ 7d A}7} A l t~~ ::t.~ 7d AVJ {l78~ )'i].¥.:C ~:Afl~ ~Tt!011 ~J ~ %-8:-%% ::t. A] ?i.g. 78 .q-c1-Tt! -T~Al 01 t~-¥-!fi % *~~~ .~ ~ -T ~ ~t}:c 7} ~),i].¥.:C l..-}Ej-y.Al ?i~q. §l~J ::t.if- *~~.g. ~ q t}1:1c1-S=- ~£A l %~~'t1 ::t.~ ~A} % Alt~t}erj {l78~ !fi-¥- ~Jt}Q] t}AJ% IT?,]~~~Alt~t} erj ~J -8:-%01 AaAJ~~ )'i] .¥.Q] ~)\H it-¥-% .~iiHoF ~J:}. :E~ {l78 ~ ),il.¥.7} ~ s- §l*~~% uJ1~ ).~ 4- 87~01] Alt~t} ;i;(~u:j ..::r uJ1 Alt~ :AHt}:c 15.q- c1-S=- ~E11~~ ~).j% ).il~i5] t}~t}:c ~01 ~ ~ ::t.~ ~A} ~~:c 7}~),i].¥.Q] AaAJ~~ ~.¥.% y.Ej-1-B~ ,B,i5}u:j itA} HDQl 7}~).cl% Jlaji5}erj achtylcholine esterase 60

Fig . 6. Differntial diagmosis of Hirschsprungs Disease in patients with huge megacolon. I. Case 9 (HD with short segment: 9-year old male): The contrast study shows huge megacolon (1-1) and operative findging shows the same picture (1-2). Ganglion cells are not present in the distal narrowed segment. J. Case 10 (colon dupication: 7-year old male): the contrast study shows huge megacolon ad distal marrowing segment (J-1). The distended loop is duplicated transverse colon (J-2, arrow). K. Case 11 (intestinal neuronal dysplasia: 10-year old male): The contrst study shows distened colon without definite transition zone (K-1) and operative finding shows markedly distended colon (K-2). Microscopically, ganglion cells are present with hyperplasia of nerve plexus (K-3a, arrow), ectopic ganglia (K-3b, arrow) and increased acetylcholine esterase activity (K-3c).

~A}y- C-Kit Z;J~~l ~A} %Qj q:!~ ~-3J ~"Q:} ~A } %% '}7} Hirschsprung's disease and other pathologic states. Am ~~'~~~~WA~~~~4~ B~ 1 Roentgenol Rad Ther Nucl Med 129:342-347, 1974 7. Rosenstein Bl, Langbaum TS: Incidence of meconium abnormalities in newborn infants with cystic fibrosis. Am J dis Child 134:72-75, 1980 8. Hyman PE, Tomomasa T, McDiarmid SV: Intestinal pseudo-obstruction in childhood. in Milia PJ (ed): 1. Sherry NS, Kramer I: The time of passage of the first New stool and first urine by the newborn infant. 1 Pediatr Disorders of gastrointestinal motility in childhood. 46:158-162, 1955 York: lohn Wiley & Sons, Ltd. 1988, cited from 2. Weaver LT, Lucas A: Maturation of large bowel Dibbins A W: Meconium plug syndrome, diabetic left function in relation to gestational and postnatal age, colon syndrome, functional colonic obstruction, in feed volume and composition in the newborn. Pediatr Donnellan WL, Burrington lD, Kimura K, Schafer lC, Rev Comm 4:250-255, 1990 White J (eds): Abdominal Surgery of Infancy and 3. Fakhoury K, Durie PR, Levison H, Canny Gl: Childhood, chap 23, Ann Arbor, Michigan, Harwood Meconium ileus in the abscence of cystic fibrosis. Arch Academic Publishers 1996, Pp 2311-23114 Dis Chiold 67;1204-1206, 1992 9. Teitelbaum DH, Coran AG, Weitzman JJ, Ziegler MM: 4. Mikity VG, Hodgeman JE, Paciulli 1: Meconium Hirschsprung's disease and related neuromuscular blockage syndrome. Radiology 88:740-743, 1967 disorders of the intestine, in O'neill lA, Rowe MI, 5. Swischuk LE: Meconium plug syndrome: A cause of Grosfeld JL, Fonkalsrud EW, Coran AG (eds): Pediatric neonatal intestinal obstruction. Am 1 Roentgenol Rad Surgery(ed 5), chap 94. St Louis, Missouri, Mosby-Year Ther Nucl Med 103:339-342, 1968 Book Inc, 1998, Pp 1381-1424 6. Pochazevsky R, Leonidas JL: The meconium plug 10. Puri P: Variant Hirschsprung's disease. J Pediatr Surg syndrome: Roentgen evaluation and differentiation from 32:149-157, 1997 61

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