68 :±,o}£13!\- : All 8 l'! Al1 1 ~ 2002';1 Vo l. 8. No . 1. J une 2002

Enterocolitis In Hirschsprung's Disease

Enterocolitis In Hirschsprung's Disease

Jung Hong M.D.

Department of Surgery, Ajoo University Medical College

Suwon, Korea

Enterocolitis associated with Hirschsprung's disease has been a major cause of morbidity and even mortality. and before and after definitieve surgical treatment . It shows typical clinical characteristics, however . its pathogenesis has been poorly understood. Treatment is diverse, and consists of conservative tertment with intravenous hydration . antibiotics and rectal wash out . and surgical t ertment with t emporatory enterostomy. and other surgical procedures. (J Kor Assoc Pediatr Surg 8(1):68-70). 2002.

Index Words : Hirschsprung's disease, Enterocolitis

Hirschsprung's disease (HD)~ ).~el , *1.!~ 7]l.! oJ] rJl tB

"'i~ ~-2-7J}A] 'Bl.g- ~1.! 0] ~Jj:t.Q.t..} HD~ -tl-~-B

Enterocolitis~ ~).~oJ] rJl tB "'i ~ 0}:3j ~~~~ -¥-~o] ~Oi

*~%J1} "'}tlJ-~~ A] 4f~ ~ ~ ~.Q..5!.. ~C;jA:j ~q.

0] ~%-& Harald Hirschsprungo] ;;,H-.Q..5!.. 7] ~<5} ~.Q.uj l

-2-111oJ]~ Swenson & Fischer %0] .!il..Jl<5}of >g el ~C;jA] 71]

~~q. J[}~ ~ <5}.Jl :AJ-7];QoJ] ~~ HD~ 3:"'}oJ1"'i 0] ~%

-& -2-~] ~ 'T~ l.!~oJ] s:. ~).~<5}uj 'T~~ "'}1JJ-~~ 'T.B.~

~.Q..5!.. ~C;j 1J.Q.uj 3, :I ~).~ s:. oj]~<5}7] OiC;jJf-uj4 EE~ -2-

~]~ AB 'T~~ 'T.B.~ ~ .Q..5!.. 3:"'}~Oi HD~ ~]liJ1}Acl"5- ~J­

AJ- ~foJ] fOi0F ~ ~%.Q..5!.. ~C;j A:j ~q.

'i'~<k~l~~!ii'tt,; ff t~ ' > ft';i"" .~, ,i:o;.:\\~,,;;,;i. '0 :CQrreSpondence : Jeong Hong,,;Pediatric' Surgery, 'Ajou~Univerity

;'SJluJol of Medidlie, Pal . IiaI~Ku. Won Ckon-do~g 5/ Suwon, . Kyung,Kee.qp 442;7.49, korel ' . . . " . -.4:- •

INCIDENCE

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CLINICAL FEATURES

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%11 : Enterocolitis In Hirschsprung's Disease 69

A~ A;il"'} (explosive diarrhea), T.£, ~~, ~+ (lethargy) ~

7cl'* ~, iL3.Q.] 'i!:-~.£ :J. ~A~O] :s::"'}s:l ;ctcl, %-AJQ.] :AjS:ol]

uj-2.}Ai q%J2} i{0] %B'~.£ KA]S:]7]s=. ~q13 (K n

Table 1. Clinical Grading System

I Mild explosive diarrhea; mild or moderate abdominal distension; no systemic manifestations

II Korea Moderate explosive diarrhea; moderate to se­vere abdominal distension; mild systemic symptoms

III Severe explosive diarrhea; marked abdominal disten­sion; shock or impending shock

POST -PULL-THROUGH ENTEROCOLITIS

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.q-, ¥{l~1i ~~o] Zl ~.q-oJ] §.~ii}oj HDoJ] r11~ q0J=~

~~] ~ +~ loIJ ~ oj] uj-c}"'1 s=. q-e ~!~ % .!i!. cj q. Surgical

section of Americal Academy of PeciiatricsQ.] :s::"'}oJ] uj-.s

1i1 Swenson~~~oJ]-i::- 15 %, Duhamel if~~oJ] -i::- 5,9 %,

modified Soave~~~oJ] -i::- 1-2 %Q.] q-e ~!~%o] {!-~s:1;ct

t:.j.14

RADIOLOGIC STUDY

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;"}AJ ~~-¥- ~AJQ.] ~AJJ!} ~AJ-S~AJ -¥-~oJ] "'l cut-off

sign % .!i!.0]oj (74%) q~A~ .:g.7]~;(~]* (multiple air-fluid

leveD (79 %), ~AJ ~AJ (74 %)0] {!-~'tIq, Cut-off sign .g.

~AJo1 ~-i::- ~.q-oJ1s:. {!-~s:17]s:. ii}oj (14%) ~~01 11~

~.q- 7.JA}~ .:g.71 \t~ (Pneumatosis intestinalis) ~7,j°1 {l­

~s:171s:. ~q15,

r11AJ:s::~~ ~7,jAJ ~y (sawtooth) s:. °J=01 {l-~'tIq, :J.

elL-} ;tl.:g.Q.] ~~01 ~7] TI11~oJ] ?Q.]iiR0j: ii}oj ~ii1 ~AJo1

{l~ 7cj-q---i::- ;..1i;~iiRA1-i::- ~'tIq,

PATHOLOGY

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% l.H Q.] 11 ~ + :Aj ;(~](mucin retention) ~ 7,:!.g. HDJ!} \t~A~

Arl%%(cystic fibrosis)oJjA1J?l- {l-~s:1-i::- ~7cl~cj ~7,:!°lq,

~o] ~i;~s:l1i1Ai {l-~s:1-i::- ~7,j.g. T~]°J=AJ AJ~J!} %"'}ii}oj16

~Q.] ~ i;~oJ] uj--e :S::~~~ ~7,j.g. %B'oJ] uj-e!- q %J!} i{o]

L-prH"'l T~'tIt:.j· (K 2) ,

Table 2, Pathological Grading of Hirschsprung's-associated Enterocolitis

o Normal mucosa

crypt dilatation; mucin retnetion

II cryptitis or < crypt abscesses per HPF

III multiple crypt abscesses per HPF

IV fibrinopurulent debris and mucosal ulceration

V transmural necrosis or perforation

ETIOLOGIES

:Aj~~ ~~.g. ~F):lA1A1 '?i'~.Q.oj ~AJtl1 :Aj~] (colonic

stasis) oj] Q.]~ AJ{l-tl1 1i1 ~7] ~Q.] ~TI--&J, AJ{l-tl1 J~mQ.] J!}

q~~%ol ~F1lJ,~cVJ %-.B.i5}q, "'HJs:1-i::- .B.~~.g. a}1]!­

~~{l-Q.] iJl]Sll12, J~]TI- (Clostridium difficile17, rotaviruS18), 11

~~ (mucin) -TA~ A~~Q.] 0VJ, 111:I.f 11~ (mucus)Q.] loIJOi

7]~ O]AJ, AJJ~].¥.1]!-~ (enterocyte adherence)Q.] 0]AJ19, r:Jl AJ

111:I.ftl1 1i1 ~ J~].¥. Q.] ° ]AJ, AJ {l ~ J~].¥. -&J J,~ ° VJ (intestinal

neuronal dysplasia)%o]q20,

TREATMENT

~~1 ~ "T-~~~oJ] {l-lj]~o] ~J~a}oj 3::7]oJ] -i::- °1~~

%AJ~.£ ;..] ~rii}O:j B' Z4 a1 ;..} uJii}-i::- ~ .q-lJ}7.] .!i!.Jls:1 Oi Q.] 11 s:1-i::- ~.q- ~]li-i::- ~4fii}71] "']:Q-s:10i 0j: ~q, ~Al -s-~%

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% ~'idii}O:j :J. ~% -'1'-{l~1i AJloIJoJ] ~~];..1~~ uR 4-6;..1

:j o}q ~7cl'J~]3i (rectal washout)% "'1 i;~~q, ~AJ{!- Q.] 11-'id.g. ~~ -1ij-~0] o}yc} ~AJtl1 J~]3io1 ~.B.~ -1ij-:Ajo1q,

.!i!.~~ ~]li'£ §.~s:1A1 '?i'-i::- ~.q- -'1'-{l~1i ~~1f-oJ1 1J-if­~% ;..1 i;~ii]·71s:. ~q,

HDQ.] ~~]~ +~4-oJ] ~.-\~ii}-i::- ~.q-~ BJA]ii}7] ~ a}O:j

70

oj] BJ?;j.9.-S- 3j -"JA1]3l (rectal irrigation) 0] A] <';~ 51 7].5:. ~c}.

:C7,]?;j T-~+ ~~?;j~.£ ~,\~t}-i::- 73+-i::- {FJ£o8~, -% ~ ,\~7,:l% A] <,;~~+7 +"i5Jtr-:C*~7H~ (posterior anal my-

- 1 lOB B A~ ~] A ( t )21.22 0 lll :;rl-o]: otomy) .2f.g. ~ -"cr:C-rr iT '" AI "2 myec omy L '-fl '" --,

:c ~ 7H ~ (internal sphincterotomy) 0 ] A] t~ .£]7].5:. t } Y. J

~~-c ~Ailt}A] ~-J-q23,24

MORBIDITY AND MORTALITY

0] ~~~ ~,\~.g. ~~7].n% ~ -"JA] 7'1 ~ li73 11 ] ~ ~7}

% 7}Ajyu-fll HDQ} {1:i(!:<§. ;,,}uJ~ 9-Jl~'?l.9.-s-;..i 0-33%

~ %~~~ ~;\~% .!2.0]E-s- "i5J-AJ J ~;\~~ ~~oJ] r)1""'H ;..i

9-~% 7]%0i0F ~q18.

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dilatation and hypertrophie des colons. Jaharb Kinderchin

27:1-7, 1887

2. Swenson 0 , Fisher JH: Hirschsprung's disease during

infancy. Surg Clin North Am 36:115-122, 1956

3. Rourdelat 0 , Vrsansky P, Pages R, Duhamel B:

Duhamel operation 40 years after: a multicentric study.

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4. Marty TL, Matlak ME, Hendrickson M, Black RE,

Johnson DG: Unexpected death from enterocolitis after

surgery for Hirschsprungs disease. Pediatrics 96( I Pt I):

118-21, 1995

5. Weber Tr, Fortuna RS, Silen ML, Dillon PA:

Reoperation for Hirschsprung's disease. J Pediatr Surg

34(1):156-7, 1999

6. Surana R, Quinn F, Puri P: Evaluation of risk factors

in the development of enterocolitis complicating Hir­

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7. Lifschitz CH, Bloss R: Persistence of colitis in Hir­

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291-293, 1985

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postoperative complications of congenital megacolon: A 25

year follow-up. Ann Surg 182:266-273, 1975

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term jUnction in 260cases. Arch Surg 127;934-941, 1992

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of Hirschsprung's disease in children with trisomy 21.

AM J Surg 159:40-404, 1990

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Hirschsprung's disease. Pediatr Surg Int 7:356-360, 1992

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Am J Surg 103 :70-74, 1962

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Enterocoli tis associated with Hirschsprung's disease: A

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14. Kleinhaus S and others: Hirschsprung"s disease: a

survery of the Surgical Section of the American

Academy of Pediatrics. J Pediatr Surg 16:588-597, 1979

15. Berdon W, Baker D: The roentgenographic diagnosis of

Hirschsprung's disease. AJR 93 :432-443, 1965

16. Swenson 0: Partial internal sphincterotomy in the

treatment of Hirschsprung's disease. Ann Surg 160:-

540-550, 1964

17. Hardy SP, Bayston R, Spitz L: Prolonged carriage of

Clostridium difficile in Hirschsprung's disease. Arch Dis

Chid 69:221-224, 1993

18. Wilson-Storey D, Scobie WG, McGenity KG: Micro­

biological studies of the enterocolitis of Hirschsprung's

disease. Arch Dis Child 65:1338-1339, 1990

19. Golderman L, Kaplan B, Rubinstein E: Escherichia coli

adherence to the intestine of mice. Isr J Med Sci

21:410-41 4, 1985

20. Puri P, Lake BD, Nixon HH, et al: Neuronal colonic

dysplasia: An unusual association of Hirschsprung's

disease. J Pediatr Surg .12:681-685, 1977

21. Kobayashi H, Hiradawa H, Surana R, et al: Intestinal

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