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Original article

- Clinical Relevance of Hirschsprung-Associated euronal


Intestinal Dysplasia (HA ID)

B. Hanimann, D. Inderbitzin,j. Briner, P. Sacher
Chirurgische Klinik, l'ni\'ersilätskinderspital Zürich, Sleinll iesstr. 75, Zürich, Schweiz

Summary 47 cas, dont 11 etaient associes a une D:'>ll: I d'entre eux

etaientlocalises dans le colon uniquement, alors que dans un
The rate of JIirschspnmg-associated cas seulement la totalite de I'intestin grele etait touchee. Dans
neuronal intestina] dysplasia (I 1A:'>l 10) irepolted to be as les 6 cas restant, nous n'a\'ons pas pu determiner l'etendue de
high as 20-75 % but no report deals with its sequeJae. The I'atleinte.

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aim of our study was to e\'aluate the influence of the retained
neuronal dysplastic segment in patients with ffirschspmng's .~Iots-cles
disease (I 10). \\'e report on 47 patients \\'ith 11 D including 11
cases \\ ith HA:\IO (23 %). All 11 children had a Duhalllel Dysplasie neuronale intestinale (D:\'l) -
procedure subsequent to colostomy and in none of the 11 .\ lalac! ie c!e Hirsc!zs!mtllg
case with HA. ID was the neuronal dysplastic segment
resected. There was no ignificant difference of early and late
complications in both groups and the results of the mean fol- Zusammenfassung
lo\\"up of 5 years after the Du/wlIlel procedure sho\\' that the
patients \\'ith HA='l1O did as \\'ell as the patients \\'ith isolated Die Assoziation der ='leuronalen Intestinalen
HD. It is suggested therefore that HA='l1O may be a distinct Dysplasie (='lID) bei .\Iorbus Hirsc!zsprung ist häufig. Eigene
disease compared to isolated _ 10 and that the. 10 attained Cntersuchungen haben gezeigt, daß das \'on der :-\10 betrof-
segment may be retained \\ithout increased risks 01' morbid i- fene Darmsegment eine \\'eitgehend normale Darmmotilität
ty. hat. Au diesen Gründen haben \\'ir anläßlich der Durchzugs-
operation den. 1O-Darm prinzipiell nie reseziert.
Key words Ziel dieser Arbeit ist e aufzuzeigen, ob das
Bela en des dysplastischen Anteils einen Einfluß auf den kli-
Hirschsprung-associated neuronal dysplasia nischen Langzeitverlauf hat. Wir haben deshalb unser Kran-
(HA 10) -
Neuronal intestinal dysplasia (N1O) kengut retrospektiv analysiert.
Hirschsprung's disease (HD) - Long-term results
Unter 47 Fällen von Morbus Hirschsprung
waren 11 Fälle as oziiert mit einer ID. Davon waren 4 le-
diglich im Kolon lokalisiert, in einem FaJ] war der gesamte
Dünndarm betroffen, und in 6 Fällen kannten wir die Aus-
dehnung nicht.
L'association d'une dyspla ie neuronale in-
Es zeigten sich keine Unterschiede in bezug
testinale (DNl) a une maladie de Hirschsprung est chose fre-
auf Früh- und Spätkomplikationen sowohl hinsichtlich
quente. 1 os propres investigations ont demontre que le seg-
Schweregrad als auch hinsichtlich Frequenz, ebenso schnit-
ment d'intestin atteint de DNI possede une motiJite tout a fait
ten bei der Spätkontrolle die _ IO-Patienten gleich gut ab \\ie
normale, raison pour laquelle nous n 'a\'on pa, par princi-
die Patienten mit isoliertem Hirschspnmg.
pe, reseque le egment portion d'une D='ll 101' de l'opera-
Le but de cet ex pose e t de montrer si oui ou
non le fait de lai seI' en place la partie dyspla tique de l'intes-
tin influence le prono tique clinique a long terme.
Hirsc!zspntllg-a oziierte neuronale intesti-
C'est pourquoi nou a\'on retrospecti\'e- nale Dy plasie (HA:\IO) - :-\euronale inte tinale Dysplasie
ment analyse l'ensemble de nos patients. Celui-ci comprenait (:-\10) - .\ lorbu flirsc!zsPnlllg

Heerl\ erl ,\", l'm1>er 2\J. 1\1\11

Eur (Pl'rllalr "urg 2 (1 \J\J21 11"7-1 IV

© Hlppokrall" \ edag Slutlgali \\a"ol1 Erliteur Par"
148 Eur J Pediatr Surg 2 (J 992) B. Hanimann et al

lnlroduction males) lhere are 11 patients wilh HA~IO. , 0 hislochemical differentia_

lion has been made for lype A and type B _ lD. The exlenl of lhe I lD
~euronal intestinal dyspla ia (NID) is a newly was lhe entire colon in 4 and lhe whole smal1 OOwel in I case. In 6 cases
lhe exacl exlenl of lhe neuronal dysplastic segmenl is unknO\\R The
de eribed disea e that presents similarly to Hirschsprung's dis-
chalts of all children ha"e been analyzed wilh regard 10 presenting
ease (HD) (5). In contrast to Hirschspntng's disease NID is
symploms a well as early and laie complicalions. 33 of lhe ·17 patients
characterised pathologically by hyperplasia of the submueous are regularly een in our outpalienl c1inic. 3 addilional patients were
plexus with an inerease of acelylcholine terase acli\'ity and lhe seen for a follow-up conlrol. 5 palients could only be reached by phone-
formation of giant ganglia. There seems to be a localized and call and anolher 5 palients li,'ing abroad were lost for follow-up. One
disseminated form of the di ease. Faddah (3) differentialed lype palienl died due 10 Jlirschsprung unrelated eondilion . Thus follolV-up
A and B dysplasia. Type A \\·ith an early aeule onsel of se\'ere results of 11 palients including all II palients \\ilh IIA '\10 wilh a mean
eonstipalion, diarrhea and uJcerali\'e colitis is eharaelerised by follo\\ ,up of 5 years are presenled.
hypoplasia 01' e\'en aplasia of lhe sympalhetie inner\'alion, \\'hile
group B presents with a late ehronic onset usually ehronie eon-
stipation and has anormal sympathetie inne['\·alion. FUliher- Results
more se\'ere complieations of lhis disease, e. g. inleslinal perfo-
ration, ileus and \'oh'ulus (8, 10) ha\'e been reporled. Due lo lhe Figure I pre,ents lhe \,'mptom, in 36 patients wilh
widely \'ar}ing presenling symptoms, no elear eut therapeulie isolaled 111) and 11 palients with HA'\II)' There is no signifieanl differ-
enee in Ihe paUern of presenling ,ymptOim bel\\eenlhe 1\\0 groups. 15
eoneepl may be ad\·oealed.
palienls hacllale eompliealions (Fig. 2) bul onl.,' 3 patients wilh IIAI JD
In 19/3 LaSSlllanll and H'ztrllig (4) repOIied 'ho\\ed laIe eompliealions. One ehild hacllo be reoperalecl for adhesiye

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loca] hyperplasia of ganglia proximal lo lhe aganglionic seg- ileus and :2 palienl~ had persi,lent posloperaliye eonstipalion. I)osloper-
ment of palients '\'ilh Hirschspnl/lg's disease. Subsequenlly ali"e enteroeolilis ami inlrapeilie absC'es, \\ ere founrl only in palienls
many reports eonfirmed HA\lID wilh a rate \'arying from 20 up \\ ilh isolatecl IID.
lo/5%(I,/). Figure 3 summarizes Ihe finding, al follo\\ up of 41
\\'hen \\'e sa\\' our first patienl \\'ith HA\:ID lhe patienh including all I1 palienls \\ilh HA '\ID.
question arose \\'helher lhe \lID allained segment had lo be
3 palienls \\'ilh II:\'\ID had mme lhan 3 ,10015 per
resected 01' not. Personal im'esligalions sho\\'ed lhal lhe da~· C'ompared 10 one patienl \\ ilh isolateclll D. \\ herea, only one palienl
neuronal dysplaslie segmenl showed a rather normal molilily
(9). Furlhermore, ,\Iunakala el al (6) reporled a functional im-
pro\'emenl, hO\\'e\'er, withoul any signifieanl morphologieal
changes in patients wilh \lID. For lhose reasons ,,'e did not ileus
resect lhe neuronal dysplastie bowel segment at lhe time of pull-
lhrough procedure. Our palient did very weil postoperatively
enterocolltiS pos top
and no eomplications were encounlered. Therefore, we did not
resectlhe neuronal dysplastic segment in a consecuti\'e series of
11 patients with HANID. abscess

The aim of lhis repoli is to elucidate whether

retainment of the neuronal dysplastic segment influences the
long-term results of these patients. with HANID (n-ll)
o no HANID (n-36)

Patients and methods o 2 3 5

N petients

Fig. 2 Late compilcatlons In 36 patlents wlth Isolated Hirschsprung dis-

From Oecember 1971 10 Februal)' 1989 ·17 palients ease and 11 patlents wlth Hirschsprung assoclated neuronal Intestinal
had been lrealed allhe surgicaJ deparlrnenl of lhe L:ni"ersily Children' dysplasla (HANID).
Hospital, Zurich, for HD. Among lhose 47 chiJdren (39 males, 8 fe-

abdominalDistension abdominal diSlenslon



CJ Wirr HAN Ci 11) sncopess,s h CJ .",lIth HANID (n'11)

ns! pat on
rt HA~ :tj LJ no HAN n 16

'1 ( 4 61 8 1 )
percerl N pa' en'S

Fig. 1 Symptoms In 36 patlen S WI h Isolated Hlrscnsprungdlsease (HD) Fig. 3 F,nd,ngs a lollow-up 01 41 patlents Includlng a I 11 patlents wlth
and 11 patlents with Hirschsprung assoclated neuronal Intestinal dysplasla Hirschsprung assoclated neuronal Illtestlnal dysplasla (HANID)
Clinical Relevance oiHirschsprung-Associated Neuronal Intestinal Dysplasia (HANID) Eur J Pediatr Surg 2 (1992) 149

wilh t IA:--IID suffered from persistent constipation or encopresis com- References

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persistent eonstipation had eeased at follow-up. On the eon- dysplasia. Pediatr Surg Int 6 (lll91) 225-226
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compared to those wilh isolated HD. We eould nol find an ex- neuronal intestinal dysplasia mimicking Ilirschsprung's disease. J
Pediatr Sur'g 16 (I moll) 16~1-1/0
planation for lhis fact. The risk of a higher rale of eomplicalions
like posloperati\'e adhesi\'e ileus, enleroeolilis and persislent
constipalion seems lherefore to be only lheorelieal. Addilional-
Iy, there \\'as no signifieanl difference bel\\'een lhe l\m groups
regarding complaints and findings at follow-up. Although lhere
are only t\\'o eases of confirrned disseminaled HA="lID in lhis se-
ries of 11 eases, no differenee for lhe lwo forms eould be found
either. We lherefore suggest that HANJD may be a distincl dis-
ease eornpared 10 isolated NID wilh all ils well-known eompli- Dr. B. Hanimann
ealions and lhal HANJD does nol influence lhe long-lerm oul-
Chirurgische Klinik
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dysplastic segmenl may be retained without inereased risks or Steim\·iesstr. 75
rnorbidity. CI-I-8032 Zürich