aicrNAL AnrtcLE: G,q.

srnnlirgnLo*y

Diagnostic Workup of Paediatric Patients Wth I nfln rr] rnat*ry S*wel ise&s* in tursp*: R*sults *f fi s*Y*frr

Audit *f th*

AL,

RKI*S Rcgistry

'Charlatte I. de Bie, tstephan Buderus, tBhupinder K. Sandhu,.Irssy de Ridder, SAnders Paerregaard, tiGabar Veres,lJorg* Amil Dias, and "Jahanna C. Escher, and the EUROKIDS Porto IBD Working Growp af ESPGHAI',I

ANST${A{:T

tlj**fiv*: In:{i{}5,
af

th* infi;rn'lmat*ry $*u,*} s*as* lt}tiilV,-:rk;ng Gr*up the il*r*pe*n S*ciet,v t*r Fediafric a*ctr,,:enic*:iL:Sl''. f-tr*Fat,:il-19"v. ;:rrd

Crncl**i*ss: Tire qu*lir3 *f ,*iagn,:stic rv-*rkup in piled,iatric patients evith iBD in*r*xeri stcailil',r' bEtrv**n :il{i4 and :**9. Sr*ai1*b*rvcl
in"laging
Ciagn*sis
br3,'

,\.uiriti{:n pubiish*d rns*ns*s guid*iines *n ihe diagn*stic wr:rkili: i:ipa*diami* lBt]" th* P*rio crit*ria" Ai-,cr:rrlirlg tit thssc guirieii**s. chjirir*r
sh*ul*i uneierg* an r]r]iiphag*gastrr:iiix;ielir:scr-rp]' t,{JD,t, ii**r-rl*nt: $:i:}p!', ai:d icxcepr in case c t:f de finitive ill**rar.ir,'e "'*liilq i adequat* imaging *f thc smrii b**s!. Tr: ar:dit and evail:{e t1i* diagn**tic
har,'ing

hr]rvrl f*il*w-tl:ri:r:gh.

rxagnetir: rcs{.}nlne* imaging supers*d*C th* u$f, *i" sniaii{rI} and ileal intutraticn c**trib*tcl tr: a r"i*fuiitir.*

slrsp*[*d

*l

il]t]

*f CD.
3.,iefci.

he3" Word*r d.iagncrsti* w*rkup. ifiagn*stic

'Jis*ar*.

inflar*rnfitr]* b*w'*l

wi:rkup
createC

*f

pa*Cintri* p*tients u,"ith

IB in Eur*pc. tiru \\rr:rl**g r**p

r*sistry oi-*rwl.' riiirg*i.'s*d
vears} we rf, r*gisterrei in *4 ce ntrr} w*rku: r+'tr* anaiy'seC a*r'*rding io

{}{:D, p*ediatrics

ilLjR*KIDg"

a pr*srlectir..q, We b-bas*J
!S

pn*Ciatric pati*nts rvitft [*S. h't*thads: Patients with ISn {agrs *

{J,Pi\ 3{i 13t54: I ?4 38*

lli*i'nsiic vieiri

in lS c**niries. Dat.a *n di*gn*slic the vear *f"diagnosis. tvp* r:i illl]" *rid c**tre si;'-*.
antl ileal ini-;bati*n rverr er,'eiuatcd.

*-.f

***

R*ellts:

Beti,e*e*rl 2{J{14

irnd lfi9. :fl$? nerviv di*unr-:erj patienrs

Br-rth

wtrc ctl:r**tiY register**i.

perl*nxeri in

D *nd iiele*llltiis*F}''

herd Lr*r'ti

S-l!,r= *f atri t:f Lh* pati*nn anci incr*aseii sig;riiicanrik l'rr:r: yir i {5: %i tc: 51?i':}'i:, P<{},{i{}l;" 5n'la!l-bci"r."*i fcrll*:r"r'-thrr:ugh sr d*tr*aseei during [ht] 'r'ear$ t-Vear I n.::li, ,Vear 5 R-= l{ii: P<{}.i:}iili, whsr**s rnagnetic rrjs*re imaging $$r inr:rt--asec! i"ear i R == 15. ,v**r 5 n '- 1 7l; f { {}.{Xi i }. fiatients dia.{nase+l as iraving {.r*hr: iis*Ese lCD. 5$..ii

and }:ie',,* ief*nif;*d {hat r;ertain are unique tq: ehildren ss W*rking rt:rlp *:f the Eurcpean r,*rnpar*d with aduits {*}. Th* Sr:ci*t3; fbr Pediatrie {iastr*enter*l*gv. Hepat,*:1*gy, and hiurrition

ineid*ncs
features

il f-

bowci diselse

argc-$c*le nnrinal epiriemi*icg-v studi*s r:f ini'iamruai*ry ifB*i in children havs d*cun*ntcd a nsing

*f

paediatric iBD

cf I$ pres*nting in chiliihr:oci

il *:ii

t*il

anrJ ulc*rative

*aiiris i5$'l'*i were rn{:}rc iik*iy tr: Lave had a c<.i*:plct*

dia.F::l*stic w*rku: than paticnt* diagn*serl as hrl,ing

{45'1.'l'}. In Cl}. iirc 'Jiagn,;uti,: lcai inf rbali*n ra,as i 3''.

iBI]

r:nciassiii*rJ

yield ,f i}Cl}

v-uas

?.5'i'; anil rhe 'l*ld *l-

{[SFL{AN}r*cr:gnisecl l{},v*ars }gr} that a{ th* tim* th*re rvsr* n agre*d-up*n *rit*r:ia f*r diagn*sing pa*diatnc lBl). Th* trV*rking Grr:up agred that c*l1ab*ratier* n a multinati*nal icvel 14,,*as n**rled. T* hal'* cernsistrn{ a*d reiiable dara. th* *ssentiai first $tep was t* *nsure *n *ptin"ral and u*iir:nn w*rkup and u*e *fl elgr*cdupq3n cdtciia i* diagn*se ISD" Tl:s griiiip ih*: heiii a nrimb*r *f
me*tings in Prti:. ti:okeci atthe evid*nce, and in ?ti5 p*L,lish*d th* Pr:fi* diagn*stic crit*ria. a cRs*nsus guid*iine f*r the diag;n*sis *f lBlJ in ehiidren i?). lt v/as agre*d that clingnosis *iCr*hn discase iCl)), uic*raiiv* caiitis { UCi. and ll}l}-une iassif red ( f &D-ij} sh*u}d be bisEd *n cli*icai signs and s!'mpt*ms, endt:$eopy r'vith hist*lagy'. *xql radi*icg_v. Ev*rv *hiici suspecteei cf I13l] sh*ulrtr underg* a c*mpi*t* diagnastic pr{}grarn c*nsisting *f scl*nscp}* wit}.r ikal irtubati*n- *es*phagcgastrcdu{.}d*ni}scr}p} {*i, axd in ail i.-ases" except in rlet"initiv-e U{l- radiological contrast in-laging r-:f ths- small b*w*1. Adriitir::rally, n"ir:ltipie bir:psies fr*n all *l-the segmsnts *f the gasir*intestina] lI) treet are ne*il*d fl*r a c*rnpi*te histalcgi*al *valuation. It was nlsc agrc*d that a diagn*sis r:f 1B]-U is acc*ptabie *nly rvh*n ti:e diagn*stic prgrarr has been fi:tiy
i:*rnplcte
d"

R"scciv*d Juns 9- :{ii }; ace*pt*d Augusr ,*. i. F:ren-l the *f}epanmeni Plcdiarric Castr**nter{-\lg:y'. Erasrnus MC-

l{*spira}. Rr:ttsrdam" T}r* N*thcrlaneJs. rh* tllepartnrsnt *f, FagEliarri*s. St-fu[*rien Hi:spital. B:nli, Cerman}', the iDepartment *i Paccii;rtne {i;:strq:ent*r*lagy. R*3,'ai ll*i;piral tar Chilqfren. Briscqrl. LlK, ths ' sepilrtrnent oi Pardintri**" Flvidvre {-,niversir-v f {trxpil, C*pcnhas.cr}. L}ennrark, th* iiisr l}er:anffieni :f Fas{iiatrics" Ssrnmtlrveis {Jnirjrrsit.t, Sudap*st, l'lungar,,'. and tire t,ilepartmenr *l'
5r:pNria CFriiciren's
PacrJiatrics, Fli:spititi S. .f*;ii:" Fr.:nE:, F*rrugel. Address eorrrsp{}ndenc* and reprint requ*sts { ir:hanna C" [seh*r, h.{n" Ph" *parfr:r*nt r.:f Pa*diatri* asir*e nt*r*1i-rg;-" Erasmus ful{-.-Sr:phia {'l'ricJr*n's ll*spitai. l}r fu{ril*wat*r;:l*in f}" 3i5 i Reitrsrdam. Tli* Nethcrlands i *-nlaii: j.'seherlii erasnrusnrc.ni i. T"he fiUIq{}K{l}$ pr*i*ct re*siv*d finenciai supp*rt lr*m H;qP*I{AN. The a*tho;'$ rspclrt ni c*niJien *i i*tere*t. t'rpyrigl"rt . :1? b,v E*r*pean Sr:ci*ty lbr Pediafri* astrrent*rult:g.v, l{epilt*losr,.', anC F{r.rlriti** and Nr:rth American $t:*iei.v* for Pediatric asf r**nte ri: icg:' " l{ *part*J ugy, atrd I'i' ut nti r: n

*f

i*i

F*rts *riteria, tI:* gr*up start*d t* pr*sB**tiveiy c*ilect a*ny:m*us data *n ew p*cdiatri* patients rvith fSD fr*m &{ay :iX}4, r:sing *n agreed-upein ciatabass tf;UR*KII}S Registryi. -lhs
Tcr au*Jit the

prim:1ry:'aini

OI:

{}.

*Q?'il,{P*.*bi-l

tr

3e3 i 8:3 ?d984

*f th* nUR*KlnS r*gistry -tr'he ?4"*Aprii 2*91. see*ndary aim lvs t* evaluatc th* diagn*stic _vieid cf i) and il*al int*bati*r durir:g **lci*sc*py and the *dditi*nal vaiue $f snrall-b*w*l imaging.
tir*
P*rJq]

*f

the pr*sent srurlv wa$ tt: evaluats adhertncs ti:

erit*ria in tir* firsr 5 yer$

i$'1a,1"

:$1; #*pyright ?12 by SFGHAf{ nd NASPGHAN. Un*uthcrized r*prsducli*n #f this article is prohihit**.
3?-f,
54., Numher 3, March

jPt'i * Vclume

iPtN " V*lume 54, Number 3, fu{arch 20.l2

Diognosiic ?Vorkup

*f

Psediatric Patients Wittl tBD in furope

MITH*Ds
TUROKIDS Registry
The L,LIRKIS registry- is a pr*spective . We b-trascd rexistr,v an*,l lsrael. *staL:lished l:' the ISfi 1&rrking *roup ol' HSFGHAI'1. This prospective registryt wils initiated in fuI*.v* :{i4 by'2 p;icdiatric sentr*s in 1 I Europeen countries and Israel as a rnethod ta ;:u,.iit the diagnostic

*f CD. The diagnstic yield af' GD in tlie s\.'aluati{}n ol children susp*ct*d cf h;lving IBD rvas detennined b.v- the detecti*n r:f granul*mais i
pr*cedure ntributed t a definitivr diagnr:sis

*f'newlv diagnnsed pa*diannc prti*nrs with lBfi in fiurope

ilr lear ile*ci:lonnscopi* *vid*nce *f Ct)"

*=hc:rrs

isolated ia the Llpper Gl tract in patients with**i p*r:ianal tJisease

Pn-ianrl disease was

i1*fined as ttre prcsencr of perianal abscessies) ancjlor fistulais),

ct*ar ileocci*noscopic evid*ncc *f CD cilnsist*d of

w*rkup i:i'paediatric patients with 1S in the years foiiowing publicati*n {"rf the Fi:rt* *rireri; i?. end t$ acr:uratelv dsscrilre disease pheilty'pe in n*wiv Ciasned pa*diairic patients with lB D. During the first -i yers" the re gr str-r has been extende d ti: *]lor.v'inclusir:n of patienls liom -14 c*ntres in IS cauntries: Beigiurn, ilr*atia, C**h Repuhlir', Denrir*rk. Frunc*. Gcnnany', Grscce. HunSnry. l:;rael, [tal3'"" l-atviu. ti:r ]ietherlands" i\irrnvav. Pq.,]and,
Fcrtugal. SL:venia, Slveden, and th* United Kl*giJr.)m. The majority of participating hospitals prl.r-ide tertiarl. care, wirh 6 csntr*s providing L:r:th secaniJrry and lert;ary crre. Sc,.'eral centr*r fru:r hiorwa,v* provirJ* s*cr:nriary car* *nlv. Fiv* *ntre\ report that they'treat.*rr:11.'the mast severe paeiiratr-ic tBD +ases. Panicipating entr*s prospe{tivelv rerr:ril dara {ln *very nervly diaun*sed chiiil r adt:le sc*nt i irg**{ ll * S y*arr; with IBD. The nnin dntn ers r:r:llecled r:t diagn*sis ancl are g* at lirst sy-rnptoms anrJ final diagnasis. s*x. ethnicit,v-, ibmii! histr:r5, ,:f [BI]. ISpe of IBI). presenii*,s svmptcl.lls, ind height and wr:sht at prt:c*dures pertbrrned at and + ithin 'Siiignosis. Aii of'the diagnastic 3 mr,rnths r:f diagnosis ar* r*cordell, a.q weil as ilisease e,xt*nt and i*calisrticn i*ndoscopic. histologi*al, and rarlir:i*gical asp*ct *i each segmefit of the i tract). All *f ihe patr*nt dta fbr the present studv iinceptir:n *i:hort ,r'Ia.v' ?llf)4-Apnl 2[]9] wers acc-rsser"l from the i:nline registry {}n 24 Febru&ry:l" fixi:lusi*n criteria irrr the pr*s*nt st*<iJv wers age at diagnosis *lder than l8 type af tBD missing, ciata re**rde d "vears, rrtrospectiv*l,l'. IBD rJiagnosis date after April ?{XJ9, r:r incnrrect IBI) iiiagnosis tJate iie, dlag*:sis dute )1 mr:nth afier registratir:* date). Hthiss cqlrnmitte* per:missi*n ws cbrain*d in the L::ited Kingdom. Swede n, and Foland. ln the other cuntries, a Statrr.tent of No Obje ction was re leased by the iocal *thics c*mrnirtee s brcause r-iata ari: anonvmuslv cclllected.

isclated ierminai iieitis cir ileocaeqal disease. Because inforc'ration *n ganulamais) was nct available in the first year *f the registry, r,ve cle ternrined the diagnstic yieki *f {iD in patients diasn*sed ticm ysar J i:rrrvards. and cnly in cases that had bi*psies from all *f the segments af the GI trae t. In patients diagnosed as having Cl)- we used boih the isolated detection of granuloma{si in the uppcr fil trrt and tl"* presence *f rnrctrr)$i:plc abnnrrnaliti*s in the upp*r $l tract l'or eletrrmining th* diagnostic yieid ui SD. b{acrr:sc*pic abnormalities in the uppfr- CI tract that were consiciered signifieant l'*r tl-re diagncsis of Ct) *onsist*d al ulceraticn, cobbles{*ning,

*r

'fhe rliagnostic yi*id i:f ileal intubaticn during colt:n*$c{}pv I to 5 wh* had biopsies from the terrninal ileum a:ri all of tt're segments of the c*lcln. The presrnce of isr;latecl t*rrninal ileitis i without pe ri;inal disease r sranutrumas) in the cr:lon i and isolate* granulorna{s} in the terrninai ii*um iwith*ut perianal disese *r iie ol:$lonqlscopic evidence r:f Clf ) was used for dete:-mining the diagnosric ,v1*1d.
wis evaluaterl in all of the chii'iren resi*.tered in y'ears

stenr)sis.

Alternatively. we also 'J*termined the disconcordance between the enrioscopic and radiological aspect r:i'the tsrniinai ileum in pati*nts who uncJers,cnt both ileccalonoscpy and adequate irnaging *i the

rmali bowei.
Tl:e additional value of adequat* imaging cf tle smail b*wel using I dif*rent definili*ns: abnr:r:n;ll ilsp*ct r:f- the t*rminal ileum on sm*ll-b*rvel imaging in paediatric patients with tBD who had colonoscp,y witiraut iieal intubati*n" and n*rmai aspf ct ot'the ierminal ile um on endoscp,v but an abn*rmal aspect ol je-iunum andiur proxirnal iieurn on small Lruwe[ imaging in parierrts wi:* had h*th ilercol*l:r)scopy and adeqr"riit* in-l;lging r:f- th* small bowel.
lvis er,'aluateri

Statistical Analysis
Dnta w*re analys*d in SPSS iversion 15., SPSS Inc, Chicago. I[-i. Descriptiv* statistics were culcults'J es pe rer]fages. F*r comparisons of prop*fiir:ns, we usd the r test. All rep*rterl F value s are 3-sided. F < "5 was c*nsidrred Eisfiiflcant. RTSULTS
As of 3,i February 20 10, 2i)6 newly diagnosed paedi*trie patients with IISD were registered. Atter excluding 519 patients" a stud-l' coharl of 28? patients rsmained, of *'hom 59r'ir 14'ere riiagnosetl as having Cl], q% as hav-ing fBD-Li" and 3?% as havinq UC {FiS. 1). The mean ags at diagnosis lyas 12.1 }*cars irange .6*- 1?.q vears). with 56o'u being borr's. Fig*r* 2 sh*ws the

efiniti0ns
Acccreling t th* Porto crite ria. the workrrp rn patients diagnosed as having CD anri IBD-U was consider*d complete when D ( witl- bi*psies i, e*lanoscp;v with ile al intubari*n I wirh biopsie:), rnd adequat* i*raging o!- the srn:ril b,,lie.,el were peribr-nred i?.1" q colnnrscopy was defined irs a procedr-rre ieaching pr*ximai

t*

the splenic fl*xure. {maging

of the smali b*wel was ron-

sid*recl adeeuatr when *ne cJf the f.*ll*r,ving m*:dalities was us*d: crnventional raciiolug,v- isrnall bi:wel ftllor*,*thrtush [S*F'Tl, e*terociysis i= magnetic resonance irnaging { MRI- fuIR-enterocl vsis, c'r &f R-enter*graphl'), camputed rm*graphl" CTi scan. capsule *ndr:scopy, an#or *nter-stopy. [n p:rti*nts diagn*sed as hilving iiC, a cornplete w*rkr"rp w;rs d*fin*d as perfilrrrlanc* ol Ct] and ilr:ocolonosrpy {both with biopsies}.

Centre size t\.as drtennined

b_v-

eSiagn*seil p;r*di*rric pati*nts with tBS psr yrilr. A "snrall snlre-' F,'il$ eirrpiri*ally rief in*rJ as a hospitai recruiting < 15 ncr+'lv diagnrseri paerii;rtric patients rvith IIID p*r vear. a '-mcrJium cenlrs" 1s a

the number

ol

newly'

distributitn of the study cohart according tc f_vpe af ltsl]. A tatai of 424 patients i?i)?.,;i re'sre rep*rred f,rom srnall centres {n -- 25 i, I I 2rt patients { 54?+ frorn medium centres in - l6). anri 539 patients {i6}i} frcm large centres (n == i l. The
age

disrributir:n *i participating centre s and patients tlTrcugl"rout Eurup* and lsracl is rtsplaycd in Table l.

hr:spital recruiting l5 t 3 newlv diagnased paediatnc patients lvith IBD per y*ar, and a '"large cf,ntre'o as a h*spital recruitins >3{) **wly cliagnr:sed pnediatric patients wirh IBD per year. We de fined the diaenostic y'ield al OCD and ilerl inrub,ation during calon*scopy as tlie percentage of patients in wi:arn this
www"

tndoscopy
OCD was perforn"rerJ in 8?% { 181 li2$8? } of all of, t}re paediatric patients with [I]lJ, colanascop!' in 9% {1995i?.=?}. and ileocoicnoscopy in ?2,'o il495r'2*8?). Iliapsies were taken in

jpgn.#{g

3?5

Copyright 2012 by ESPGHAN and NASPGHAN. Unauthorized reproduction of this arti*le is pr*hibited.

Ce 9ie et ul

JFtrV " Vni*me 54, Number 3, March TABLI

:$l

Farticipants in the fURCKi*S registry

I'Jr:"

pa*icipirting
f etres

!
1

f *untn'
4 Aqe ai di#$ner*is -' 1 'lr 'l Typ+ ri 'H ."nrss;nr; ?.ii S*rr,*,gfiec,iiv* dnta *i$ ti*glr**i* > .Ar*i?ta* 3,{ iitci}rre*; dqt* *f rjia.Cn*s;s'

netl
I

*nts

xciusi**

[3eigiurn

f"t

llr*ati;i
zech R*prihiic *nmark
Francc *t*#y c*h**
a

Ti !J

2
f

I f)8

:
+
1
.4

llr
s? tS3

{ienrrai:y

il/ -. *na?
{,-1,/LJJ -

rssc*
Hring*ry

5*
4{]
9{}

I
L

lsri:*i
taly Latvia 5e th*riands
I

1
'

i*1
.T
4

I
1

I ri3
*t

tltllnt

1. Ffu:wchart

tUfi*Kl*5 r*gistry. ,* ate *f diagn*sis ->l manth after date *f registrati*n. C* * trchn disease; l**: infiamratry b*wel disea:e; t8*-t"J * lSil-unclassified; Uf _- ulferative e*litis.

*f thc :tudy p*pul*ti*n in th*

Nlr*"av

11

A1

P*land F*nugai Si * l'snia

Swed*n

J
I

]f:{r
JY
-r

:
{ +

JJ 1{{*l r -,'

.l?

i-riited K:ned:

5*4

i1?i 1i'XSl i)*f {}#D, r}S% {l$4,'1q5} *f'e*i*i:r:scop:ies, a*d g3'i'; {i-}:,'i.i*53 i:f iis***i*nr:*ci:pi*s. T}rr** p*ti*nrs w*-rr diagn*s*d as hsvii:g {lI} b'y sr":rg*rv" }l*dici:l r*asrins fi-:r n*t insperting the t*mrinill ileum ii*" nsk *f perf'oratir:n. pr*scncr at a sqen*sis. p*rf*,:rati*n. abs:*ru"ili cil**rir1 rvith a ps*ud*ciiv*rticuiurri r&irir
Q5,rr

insuf,flci*nt su^dati*n
aeurn ln

re*ist*reii in

pati*nts i 5% ), *th*r re*rt:ns wcre - 'tcchnie al pr*biem" {n *" {49. ?-t'!r3- insrffi*icnt bc,1.*i preparati*r': {n * 5. ?'r"iri- la*k r:f tiru* t"n: J4. ?q.;1. judgcd *nn*cssery b;* th* end*.:* sca:ist {n.,= ? 1- lu}'{,}" col*nosi:op}' di:nr *isswi:cr* in -_ 9, *.4o*,,
t

ii:

it

and binpsirs takrn in *rrrlr at th* remaining palients in .* 3 i9' i 1o,,:i. was ni:t elear rvhrr,,tl:* t*rmin*i iieun: $'ils Rt visuaiiserJ i:r-"*

i*

1" -.5qt':i.

fi.lo.ul. - 4. the

ln

*::rlosc**y.
Frig*r* 3 sh*ws th*
e *l*nosd.rlpy, anel iie*c*l*nscpy during tl-r* fji:st 5 3'ears cf th* registry. Setw,**n y*ar$ 1 a*d 5. perf*rn;an** *1" ${.s iF: #.ti3i. **l***sc*pics iF ,: {].fl I 3 i. and iiei:c*ic*t:si"'i:pie s {,e{.tlt} I } inrr*esed signiiic*ntl"v. A cr-:rnbinati*n cf *{lD and ii*:ccr:ianscr}pv rnr,*$ p*rtbnn*d

pati*nts

.,*

ith JBn r+'h* i:nden*.*nt *n-

pr*prfii*n *f

n*w13"

iiiagn*s*d p*erliatric

in 6.t% of ali *f th* paediarric patients

r.rithr

si

llU

.;
d

(:L;

z.

1{i

y*ars 1 and 5 {F { {}.{}i}} i. ** was perf*rm*d significanily fililrc *ften in pati*nt$ diagn*seJ es having C$ than in nati*nts diagnased *s hav'ing IS*-i., *nd L,iC iTabl* l]. Pati*nts diagn*s*d as hal'ing C als* u*denr*nt celcnosc*py and ii*r:c,:i*nospv signiti*anti3,' m$rc *it*n than patl*nts rii*gn***rJ as having UC. Thsr* e,,er* nt: signifieant rlifllerences i* perfi:E-nanc* *f e*d,*.ser:pic pr*r:*durer b*tw**n patier:ts diagn*sed as l:*r,'i*g U{ and IgI}-U. Cases tic:i larg* c*ntres 1l'ers ffiore likci,l' t$ havs h*j tXrl] iSSs, ) than th*ss firni srnali *entr*s { $5'*, P -, t}.*{,}i i r msdiuru e entr*s lS.3o, p *- l). I i }:ut were l*ss trikel-v r* i:avs had ils*c*i*nssrp,v {5*3o"r vs ??i4 and ?{:%,, bot!: F { .ii.
sigr-:iij*-,ent11' b,v 19% L'*tr.r,-*en

IBfi.

and incr*llscrl

* 1 2 3 .4 5 * 7 * S 1* t1 ie i3't4 1 1 r,? 1
Age *t *i*g*csis {ye*r*}

lrnaging of the Smaftr SaweN in Pa*diatric Patients Newly $iagn$sed as l{aving C and lBs-u
lnft:nxati** *n ii::*ging ef t!:e small b*rvri 1vi$ *vailahl*
i i4-114 t?) *f patienrs diagn*s*d as l-raving CI) and t$l)-U. Arl*quaf* imagi*g *f th,* sr"ral] b*rvel was pcrfbrmsd in 8?% il116i,' ii i) *f paediitri* patients at c:r witliin 3 nr:nths r:f tl] diasnrsis: SBFT in 58?i' {n * 7?}. futRl in ?*s'o {n * 353}, CT abqJ$men in ?4' i*:8), rapsul* *nd*-cc*py i& 4% {n * 52}, and entcr*s*py i:r ..+% af pati*nts {n * 5}" 'lhe smali bew*l was r,,is*rlised b"v > 1 imaging tech*iqu* in i#t'i i i ?5.1? 16) r-rfl pati*nts eiiagn*s*d as in
?9?'+

ffffi

Cr+l'rr- disease

l#l

"J

iit**rativ* *alitis
lS*-un*iassifi*d

FltUKr 2" ASe di:tributi*n ilf newly di*gn*sed p*ediatric pati*nts with infl*n:matnry L:*wrl disease in th* tUR*Kl*S
reqistry.

J /$

nf

wvvw jf*n.#rE

Copyright 2012 by ESPGHAN and NASPGHAN. Unauthorized repr#du*tisn *f this arti*ls is prchihit*d.

JPfiN * Valume 54, Number 3,, March 2S1;

:0
gr:

agnosflr kV*rkup sf Psedl*fric Psrrenfs

y1+fh

t& in Europe

1
I

1
I

*oj
l'o
70

*"

Coicnosccpl:

sD

bU

t {leoccl*nascspy + C - ileccolcr:c$copy

i ? I a

Adequate irnaging SBFT

MRI

CT abdorren Capsule +ndcscopy

40

*3
*att*\*

4t ...?ut -\" {'

'.-d_ ,"_ *#o

;}* *ao* *""
-iF*

ond'

FltURf 3. [ndoseopic procedures in paediatric patients with inflarnnratory bowel disease during the first 5 years of the
[URKIDS registry. OCD

+4/+ *-t "+} ?o .tf ,"3 ,* \ *\@' +P" -r9-

,-

.P,
,us .* -{e'"

.ft
*\4"
r$

","F

"to

* o*sophagogastrcduodenoscopy.

FlCUtt 4. Small bowel imaging in paediatric patients diagnosed with Crohn disease and IBD-unclassfied during the first

5 years of the IURK|DS registry. CT =. *rnputed tamcgraphy; MRI : rnagnetic ressilan{e imaging; SBFT : smsil
adequatc raelii:logv tr\'as p*rfcrmcd in 7l':'i, { 138'lS8. r.lhich was significanrl,*lorver than in patients diagnased as having Cil iP. 0.*l)" Fitt]'six p*rc*nt nf pat*nts diagnuscr.J as having IBD-U underwent SIIFT {n =* 1{}6}. l,3q"i il,{Rl {^n =.33}, 2t,; CT abdc}rnen in * 3i. and i?i: capsuie endr:scop)' tn 5.). In 4?'o {?,''188) of paiienis diagr"rr:sed as

hat'ing il [i"

ln patients diagni:sed i]s having IBD-Lj.

bowel fallow-through.

Carnplete Dlagnostic tfforkup According ta the Porto Criteria

In tomi ,, 57a ( I I q 1,'283 i:f paediatric patrents wjti: IBD hacl a diagnostic r+'orkup accarding ta the fuii Forto criteria. A cambination oiGD, ileacalon$scrlpy. anci adequate imaging of the smail

hnving flSl]-Ll' > i irnaging teL'hniqur was used tc visualis* th* srnail borvei. Radi*;lagical *xaminatii:n b1, abrJr-:rninai ultrasound alane rvas pertbrmed n 'lo of patients diagnased as l:aving Il] {n,*n9i and 11% cf'paticnt-* diasn*scd as having IBD-U {n -* 2i.
F =, .ii1)1.

b*wcl

\,'es

pertbrnled in

59..6

as har.ing

Ci) and

4i41: i86i

{?15,'1223} of pstients i9l r:f patients diagnosed

diagn*s*d
as having diagnostic

Variation
ss having

in

small-b*rvei imaging

of

patients diagnos*d

and IiSD-U is ilisplayed in Figure 4. ijse of smailb*wel in"raging incr*ased.*ig:rifieanti,v f-r*m S4ur in ycar i ta 92o'i in year 3 {F * tl.iX};li hut clccrcaseci significanti,v tn ,11;qrr in year 4 iP < .tX)1) and returned to 8-1", again in year 5. Between vears i and 5' usr of SISFT' decreilsed signiflcantlv b-v i14'),ro iF <. .01, rvht:r*ls use *f MRI anrl flT abdorncn increas*rl signilicantl;r by 12o,+ and 79, lbath P < .ill ). [,se oicapsul,e endoscop]'inc'reased significantly cluring the hrst t years {vear i: I }'a. .v-ear -i: 8",: f { ii.il I }, but d*creas*d significantiy in the lest vear i3n.n;
F

Cil

rvorkup, biopsies fiar:r the uppcr {ii tract and fr*m thc c*lan anrl terminal iieum were taken in fi9% i634'?15) *f patients Ciag*i:sed as having C anri 88% {7t,$6i r:f patients diagnosed as having IBt}U. 1f iess strict criteria w.re use*J ({iD, coionoscop-y' and either iieocclonoscopy or adequate irneging of tlie small b*wel), 8TQ;'*

IBD-U {P-il.0i}ll. ln tire patients with a compiete

* .{i31.

ltl6f i:23)r-;f paticnts rJiagnosedes having CIJ and 75% { l,lii lqfi} af patients diagnosectr as havine IBD-U had this combinatian of ciiagn*stic pracedures. ln patients diagnosed AS havtng UC. 58% {i9'67} undenvent a c*mbination rf f}CD and iler:c*k:n*scop,v" In the patients r,vith a cornpiete wcrkup, biapsies oiali *f th*
t

When sxaxrliried try centrc sizc, sigaiiicant variations in ust cf smail b*r'el imaging rv*re absen'ed. Ilatie*ts diagnosed Cf) and Its-U in large crntres lr'ere ffire like13'tr: har.e had SBI-T {829'i} than tl'rose in smrli ccntres {"i39,,h, P < .{}i } r:r
as ha';ing

rn*dium *ntr*s {5 tr t,r. P < .fxi 1 ) but lvcr* less likely' to have had MRI {l-q l's ;15'},ir and 34CI,''0, P < 0.l }, CT abdomen i3t},,: vs l*l"ii and 6?, P < .01i. or capsule endoscopy- {iY+ vs 5. and
9,,;" F

*. ,il

).

scgulents r.vere taken in 87'l'ir {338,'3q). Adherene to the full Porto criteria increased signifrcantly fiorn 45'i,i) in *eatr i t* 64?o in i,'car 5 {P -; {J.{J*li. lVhen cx}mined L'r1' type at IRD. ihere was a significant time trend in adherence tc the full P*rta criteria lbr parients diagnosed as having CD {year l: 498,b, year 5: 4?, P < 0.l}01) anC pationts cliagnos*d as h*ving UC i,vcar 1: ;ll0,6; )'ear 5: 6S?, P < .001i, but not fbr patients eliagn*sed as having IBD-U {,vear 1: 339/,r: year 5: -1}%. F * .35i.

TABLE

2. Endoscopic procedures in paediatric patients with

All {n =f0S7}

IBD according to type of IBD

CD

in*

l?2-i)

[3D-tr {n

190}

l,{l

{n * s7}

{}{iD {%i
Cr: I t:no sf {:}p,v
tr

lQ11 r.r-ri r?\ lC, l I

{
",.,?,

f I f { /1r* I I t *.r \*J I ) 111q4

eoco

no sc*prv* {o,ir i

le95 {q6 t4q5 i7:i

{'iri
111''

(97)""

CD + ilcr:c*ionosc*py

i JJJ

IO+

t/1t

9) ( 7 4)"' 8i I i68)OGD:

i6l {85} i8t 195i i:9 (b8)

i r2 (59)
oesophagogastroduodenoscopy;

535 {8j

6l

.tfli
UC:

{94} iS9)

3e{} {58}
ulcerative colitis.

CD: Crohn *_Significant

disease; IBD - inflammatory bowcl disease; IBD-U: ItsD-unclassied; difference compared wirh C {P<0.001) anrt IBD-U (P<0.03). Signifit:ant dilference compared with UC (P < 0.03).

vvww.jpgn.org

377

Copyright 2A12 by ESPGHAN and NASPGHAN. Unauthorized reproduction of this article is prohibited.

ds #i* ef

r/

JPtru " Vclxrne 54, Number 3, March

disease cer be ciassifieri acsr:rding

?*1:

Patrents wltil ltlt) **m larg* rnr$s w*rr lsss likel;; te have hsd a **rnpl*t* di*grrE:stic lv*rkup {5 i+"-:i than pati*nts f"r*nl smail ce*tr*s {59%' P -* .}lJ or medirm centrss i*ai4:. f -,{J"{i{il }"

#iagn$st*{ Yl*ld *f *t*, ltr*al Intubatisft, end Addltisnel Value $f SrnaH Bcwel lrnaging
In ytiirs

tr: 5

*f ELffi.KIIIS. tli*r* u/*rr ?4 pa*diairic

patients with fBI]',vith bi*psies fn:rn *il *ithe s*gmrnts *f ti:c i tra*f. l) r';ith bi*psies led tr: the iss:leted detee ti*n r:lgranui*ma{ s } in th* uppe r [ trn*t l lvith*ur ef*t*cticn *f granu]*ma{s j in il**c*ionic brir-rpsir:s) in ?.;t% { ifir74{}: CIt pa*eliati:i* patir*ts wit}: l$l}. Frve sf tl"r*s* patients -tis* haci pe*snal ,J{s*as* *r cl*ar iler:er:}*n*scr:pic

{* the b'k:ntreal c}assificati:n {S} Per-is ciassii"ictti*n i9)" in th* pr*s*nt stud,v, ths lB Wr:rking Grr:up *f ESPHA,'-* h;rs prribmled an audit tt: *valuat* diagn*stir" p*rformance in children suspecterJ *f having ISD annJ r* anai3rse thc -iseiuln*ss *fl a $ns**sus-hased g*ideiine duing a 5-y*ar p*riail. F*r tliis purpss, & W*b-hrased pr*sp*ctive rcgistry;, ilUR*KI*S, t&'as initiat*d in 2tJ*4. Aithaugh it is genrrall_v kx*wn tirat tli* incid*nce r:f iBI] increases witl: agc. the p*ak incidence *crurred at ar*und lrl t l5 -rr:ars *f agc in r:ur H*r*pe;:n **ht:rt. T}:is ra'fl*cfs daily pre*fic*. in rvhich acii:iescsnts are of'ter dingnr:seri b3t "a{iu[1" Si$rri:ent*rk:gists" nt r:f th* mclst int*resting resuits of nnr stucf3'" lxas ths *iear incrcas* in quaiiry" *f, diirgnastir r'{r-k*p during tfr*

*r tvtn th* ru*r+: r**enti3'puhriish*d

*r'id*nr* cf D iis*latrei tennin*i iicitis. ii***a**ai disess*i, r.vhicir d*crsas*ci th* diagn*stic yie id siigl,tts; to 1 .,,q'!cr { 13"?40}" unng
*{ti'"}n*s*py,* r:f thes* 13 pati*nts wer* ais* ts}und l* i:ave inflamm;iti*r: *f th* *i*n, wh*reas 5 paticnts had in*ammatii:n *f b*th th* c*lt;n nnal t*rrlinai il*un-r" The remainins: pa*ents haiJ smali bawel inv*ivem*nt and upp*r Gl inu*1l'ecle1, i*esp*ctivel-v. ln CI). '+:S pati*nts had i:ittpsi*s fr*m eli*f,tl:* srg:xrn{s *ithe [il tar-t in y*ears i t 5. Tf:e fr*quen*3r *f sati*nts dlasilas*{i }s hving {'* whr:se diagn*sis r*ilr:C *n is*lsr**i det**tir:n *f,granuiama{s} at O{;D was }"fi'l'; { 13'4:i$}. ln additiq:n. th*re wer* lq patient* diagnosed es having L-l) whr: had uic*rations in thc upp{ir {il tract i*'ithout penanal dis*as*, ilecct1*n*scr:pi* evidsn*c *f C. or det*ctir:n f srailui** r*a[s] in t]re GI lraet]. Ccbblrst**ing sr stent:sis with*ut the i:th*r

first 5 v*ars *f ELjRKI*S. Til* a*erptanu:e in Eur*pe af perfcrrning an Sl] dr"ring ih* iirst diagncstic w*rki-rp sf a paediatric patient with :B# wes high. stilrting with 8l% in 3"ear I anrJ reaching aim*st *{J*,6 in 3"car 5. In L'C anrl I$D-U, {he nxmber *f *CI}s lvas signific;*ntly 1**'rr
than in CD b*t stili

charectsr'istics {- t} did n:t *rcur. ln lt: pati*nis, the t_y-p cf" ffiittr*srspic abno.:rmaiitv was nrissing. l\,'hsn ineiuding ani_-v tir* g:;ili*nts with is*lat*cl granul*mats) *r u-ll*crations i* tirc *pp*r l tratt, tLre tctai diagn*sttc yield *f 1] was ?"5*,1: { 13r-1t}14:8}. The diagn*stic yreld *fliieai intr:biiti*n r*uid be d*tennined in e)62 patients *'ith lB i* years I t* 5 rh* hadbir:psies ft*n: ail *f ti:e s*srnents *f th* cq31n and tl'i* t mlinel ii*um. Fifu'-six patients
d

*i

paedietric lBn]. A';*r:rding t* the.\iarth Ameri*an S*ciery f*r F*diairic as{r*en{cr:i*S}:, Hrpetol*gy', and hirtriticin, fifi with bi*psies shr:uld b* "ei:nsiri*rsd" in e hildrsn susp*ct*d *f, h;rving il3lJ, Lrut mr* resear*"h r: the diagni:stic vah"re *f 0 is rnan*1at*ry ii1. in r:ur st*C.v" we evaluat*d the dragnastic yi*lcl *f i-iD. bas*d o* bu-th end*sc*pic and irist*ir:gicai c;'rit$ria. ririis aisa taking int* arlrlri thre *nd*tc*:pic irnd histalagical finCings

al diagn*sis is nt rcr{rrfiin*rrded in ali rf the guidelines t:rl

Lrerra"sgn 8o end

9*%. The r*utine use *f

**lJ

ir*m

iis*c*i*{l*scpv. Xllld.

n*nsp,*cif

i Hti"" t li*rJ is*lnt*rt t*rminai ileitis witir*ut penanai ilis*ase or

{*9, erytirer:r. r=rosions. itpht:r*} in th* xpp*r l tract iire crr}tn*n in b*ih CD and i-C. Ssr=ause uic*ratins. **bbbstr:*ing. and sen*sis arc rar*iv $een in t {l {qi, 1ve *sed cnl,v ihese spe*if,* irbncrnraliti*s f*r ci*t"rrx:ining th* riiagnt:stie ,v*id qlt GD. This yicl*j was ?,5**" inrlicating that GlJ c*ntributed t a diagn*sis ct tD in I r:f
i.-1

re

mucset changcs

*ai*n. 1r.: adcliticn. 19 pati*nts {2.1}s i had isaiated grenrii*rua{si in thc teminai iisum without thc other charactenstics *f {lI} {ie. p*rianal dis*ase l:r ile*c*i*n*sc*pic *vidsnce *f Ct}.i, resuiting in a tliagnr:sti* visiei *f ?.li-t l?5"9b: i. in Cl], 55? petie*rs in v*&r$ ? t* 5 had biopsi*s r*m all af the segrnents of the *clcr and the t*rminal iieurn. T"h* fr-*queti*,o* *f patis*ts diagn*sertr as having C wh*s* diagnosis r*li*cl *n iic*e*Ir:n*sc*r,v ii*, iscliated dctecti*n af greun*l*rxalsj in tht tenninnl i1*um cr isolaf*d terminal ileitisl waii i3?'L {?5i559}. Inf*rn*tirn ** br:th the enlcs**prc and radi*lcgical aspects *f tl"rr: t*nnnai ii*ufi1 rvas availabl* i* 875 paediatrir parie nts with lBD, In i5l { I7%i patienrs, ih* rqrmifiai itreunr l{,-'as cr"rnsidcred
grirnuh:ma{ si in the

pa*cii,:tric paticnts rSiagn*se

c1

as having

th* di*gncstic r*le *f flt] ]rave f*cused prin:arily *n the is*iat*d d*ttcti*n af granul*i$as i* th* upper GI tract. 'fh* r*sults *:i"ti:Ese sturli*s l&-"crc summarised in ;i}ti$: the fu*quen*y cif pe*diatnc patients diagnas*el as having CD whr:se rfiagr*sis relisd *n isr:iat*d

Cil. Prcvii:us studies *R

*ppr:sit* cqlmbinntir:n, a nfiTlftl end*scr:pic spprarancr rXeum in e r:mbinaticn with nn abn*rmal t*rminal il*um *:n eei*r1*ets srnali bt:w.*l imaxirig. rleutrred in 5S t7%i paltients. T'h*re w*r* 'l I S paedialri* pati*nts witli IBD rvh* und*rr,vent ci*n*s{J*F},v rvith*nt ileal intubatian b.t ivit}: adequate irnaging r:f li'l* st"nall b*w*l. The t*rminai iieum \t/as abn*nn:l in i?* patienrs i4;oi;ii) end n*rm*i in I94 p*ti*nts {4$$.,i, and data \\,ere rnlssing in S4 palients i l3-''r]. In S?? pa*diatri pati*ats rvith l$n, infqlrrn:rtian \'vas avaiiabl* n tli* aspect of th* terrninal il*um h:-v il**coi*nc* srlpv- as q'veil as $n th* asp*ct *f, j*-;uni:m and praximal ileurn h3,' adequar* srnail 'i:*r.a'*l ir:raging. Fif$-*nr Fetjrnts {tr.??a} had ll Irnfil t*n'rinal il*um r:n *nd*sop"v enel an abrn*rmai j*junter"l andl*r pr*xirn*l ileunr n srnall bcwrl irnagiag.

abnormal n end*scapy but nr:nnal n a+3equate *mall b*rv*l .Ihe

imaging.

*ftire t*rrninal

dstectir:n r:f, granulcmls at {iD s*em*d to rang* betwsen ?% an'J Z 1?i) i I 1; This iarge rarlgr cf granuiama $et**tion mfiy yary with nun:b*r and sitc of, bir:p$i*s- as r,vell as th* qu*iiry *{'hist*i*gical w*rkup in ti're pathol*gv l*boratcry" W* i:ave n data e*ne*rni*g tire nurnber *f bi*psies tak,en in aur patients, cther th;ln rhc gr:ideiin* arivict to take 2 *r ffisr* biopsies ti*r:r *ach seginrnt. Using anr:th*r dr:finiti*n fbr diagn*stic },'i*til *f tXiD, Lemberg rf ai t i 2} r*p***'3 tl-lat encl*sc*pic anci hist*logicri ass*ssmrnt *f thr ppa:r GI tract estabiish*d fr diagnrsis of CD in i3 af, 38 patirnls {34--*; rvith therrvise n*nspecific pitncalitis, Taken t*g*ther, ali *{'t}res* resulfs indicat* tlaat $D *cntrihxted t* a cliagn*sis *l'Cn in * s*bstarrtial number *f patients, justi$'int its uss in th* initiel assi-Fs;11**t *f chiidren suspcctetJ ot having IBI]" flesid*s ths diagncstic ir"::piicatiqlns nf l]. kn*r.viedge alr*ut inv*lvrment *f the u*sophagus,
slq-rinach, ;*nd du*tlcnurn rnay-

F*r cxampi*, it
r-r*s*phag*ai

have

th*rape

utic

GrIsr{ilncrs.

i-is been

tD,

shrrvn that in pa*r3iatrir- pati*nts witi:r dissase orlrsr has s high prcbability f*r *arlv

DI5dU55I*N
Th* Pi:rta criteria re*ffirrlcr:d a unif*nn diag**stic rvi:rkxp in *hiidrcn *nd ads:l*scrnts s*spccted sf'having ltlD to r*liabiy
*14*sifu diss:s* typ*, *xt*nt, anrj lcaiisatir:* t7}. Fri:n' ti:is w*rkup.

n*ed cf azaihicpr:nc { 13i. ln additir:n t* tir* incr*asing numbers rl {J{iDs, th* sucess r*tr of ileal intubati*n increas*d stesdil,v fr*m S l-tl in,v-*ar I t* ?Qi: in y*ar 5, This signifi*ant increes* m&*v bc the result cf rh* pres-g ai ,:cntinu*d registrati*n rvithin thc HU I{{}KILIS stud,v gr*uF. An ev*n rnr draniatie imprr:r.*m*nt in suc*css rate r:f iisal iniubatir:n !\,'*s rep*rted in a study fncm :{}*?" l}atres et al {itf} anal.vsecl paediatric c*l*ncscapi*s from I9q4 thr*ugh ?txli] and f*und an increase in ileai isrrubati*n fr*m 7]^,* betw"*** 1$94 and www"lpg$"#ry

3Tfi

t*pynght 2#1? by H$PSHAN nnd hASPGHAN" Unsuthcrieed r*prdu*tlsn f this articl* is prohib,ited"

lFtdv " V*lume 54, Nurxber 3,

fu1a'e

h 2*12

*i*gn*sfic

Wo rkup

*f P*sdisfre P*ficnfs

kffrffu JS#

in fur*p*

I $v* r* *S% in 2St]. Fo:ssible expianatians fi:r this innpr*v*ment wsrs th* t*ehniral d*v*l*pnrents :f *nCc*cops" vidc* in":*g*r, ancl scrfins in fh* l$S*s" es w*il *$ gr*wrng expcriencc rn*J skilis r:f p*cctiarric *ndose*pi sts, "fh* terfilinal ilcuru rvas n*t visualiseJ b_v endr:spy in 28'1,'n *f t.l* patinxis ivith ISI}. fuI*di*;li r*i:s*ns {*.risk *f perfr:ratit:n.

pr*$*nct *f a st*n*sis) wer* r*stlilnsible fr:r *xiir*inatjr:*s t*ry*i*at*d *utsiCe thr terrninal ils*nt in at ieast 5'f i: cl ali *f th*

pr*c*durss" TXr* ailult {Bi} iiterarure has r*p*rted iiral i*tui:ati*r rat*s *f 5'lt *f nii cr:i*n**rr:pies { l5}" whieh als* *mp}:asis*s that in appr*xiniatslv S5tl'o *f *ii pati*r:ts, the t*rmin;li ii.:uin sh*iid be

*f paticnts with CD {';5,?f:.. llespite its abvi*us adr,'anti:ges, CT' ais* rauses sigeifieanl radiati** cxp{rsurr i:5u-4.1n',; cf al1 ra<iiati*n *xprlsur* ir pci*<fiarric B}}
infiammation in the small int*sti*e

expedenced radi*ir-gists because inierpr*tation and sc*dng c'f futR{ iindings an sn:etimes be difTjcult i23]" Use *f CT aisr: incresed signiflcantl-v through th* yssrii, with 9'i.'.* *f patients di;ign*serl es having CL) and lB*-l- unJ*"gclng a CT in -"**r 5. {-T has i:rscn sir**.:r t* be sup*ri'.:r tr: SBtr-T in b*t}: se nsitivity" an<1 sp*cliicitv {15}. Atthi:ugh c{-}rnpararive ,Jate ern .\1Rl anl CT ar* lir*it*d, *"*id*nr* fi*m *n:all *ili-rlt:futJjs sugg*sts that b*th imaging iechniques sh*w similar ecruracy in d,*t**tu"lg sctivc

prssible t r***h. T**hniral prcblems sh*uid Lre vrrr*l# by t*eehi*g and tr*ining, ta,'hrrfl;:s lack r'if iirne- rnsufflei*nl c*i*n
prflparelti*n. and insrfTrci*nt sedatian cen b* av-*id*d by cptimising

il.

prr:f*c*is ir:r b*w*i pr*pnretir:n and ii^* tirning

*f

pr*e*r1:.res"

Ileal infubatiq:n with iieal bi*psles has br:en shqiwn tr: inrr*ase tl:e itiagnr:stic yi*ld al {lf} in adult pa{if;nts pres*nting
'"r.ith sympt*ms *f* ISD i l6). ln aur detas*t. th* diagasti _vi*ici *:i ii*n] in**batrq-rn in pati*nts diagn*sed as Xraving CD rvas 3ii"r. fi*ai intubatin rail sls* contdbutc tc a diiigncsis r:f CD in pati*ntx u..iih

A diagn*sis of f SD- u is usuall"v rrss-"'sd f*r p*tients with ltS l-] hav* fbatur*s that mak* the ciinician Rcortain as tc wirether the diag*r:sis is C* *r LiC. A*"r*r*ling {$ ihe Pq:rt* *teria.;* diagn*si* r:i' lB-U is oni_v ace*ptabie rvhcn e c*inpiete ili*gn*stit wr:rkup has L-et* perfl'rrar*e1. f * *ur snrd,r' cohr:rt, Stl'l; tf paedi*iic pati*nrs r+'ith Its* w*rr diagn*s*ri fis having IBD-U, w'trich is similar ti- th*
w}-ra

"1*l

pr*vale n*;*
{f:.1?

n#n$peilic panr-r:iitis rvh* her,'c distinci rriarosc*pic l*si*ns ir: lhe tsni:inal ilcilm, su*li as **bbie str:ning and li:*ar il**-ati**s. This infr:rn:ati*n wes ni r*gist*.r*d in q]r elatabase . Th* ratrs *i' iic*i infubatin w$s aisc hightrighi*d b_v- de h1;:ir-:s st al { 1?i. '.v}:* toli*d is*lat*d granul*mes in thc t*rn:inai iieum in ? *f 1i: {J3iii,J un{r:at*r"1 pa*di*trir prt;nts diagn*ls*il as }:al ing {.n.

*f an incsmplet* diag:l*stle w*rkup lin 55%:. diagn*srs cq:uld be changed t* CI) *r Ui- at?*r a fuil w*rkup haii be*n p*rir-,nr"r*cl. Estabiishing e d*finitie* diagrr*sis
liavr
been the result

}; hw*'"'er. that p*ents

r*pi:rt*d in *tir*r larg* p**tliatd* I B i:*hlrrt stucii*s 1+ ere labeiiflJ as {BI}-U #}lt'

It niao,'b* fhat this

L{ is *ssentiai. esp*c:al}y in the contsxt tf ch**sing therar:eutic clpti*ns and discussing 1*ng-teffir pr*Sn*sis ra'"ith
r:f C r
;r p}li*nl.

A c:mbinati*ri *f ilGll an"J i1**c*1:nsc$py ra''as perrfr:n:r*C in r:4?t ':f aii paticnts *"ith lili] snil in *vsn fl*w*r paii*nts di*gn*sed as heving IBII-{,* {5?'ti}. This lo',v pcr{llntlncr ra?r is prim*riiy itllscli b,t' lack *t ii*el in{rL:eti*n {in ?8*,',; r: pati*nts i. Hightv-f*ur percent of *ll paediatrir* pati*nts rvith IBlj und*n,"e:t

Fr*r,'i*us st*dies sn adhr:re*cc

guide

t* adult gastri:enterr:1i:gi*al

patir*ts with lB]. sun'eillane *al*nr:srp,v in UC. r*l*n p*i3?

surt'*ill*ne

iin*s if;g, *vaiuati*n and ir?anagement

*f

*stenp*r*sis in

ri

ha"''* d*morstrated ihat ai{h*renr:e is irequtxllv- sub-

b*th

*r*pi*

# nnd c*l'nscspir. thus rel'lecting a high graelt *t *cei**xt-ilinatirn *f ti:* upper *nd iE:w*r {ii trael. l}it?*reriees in
{}
and UC are prc'Lr;rbly ini:*ra:nt

*ptimai a:*ngst *iinician: {:8*:1i. This was als* sh*nn il *ur study, rvith ovcrali *dh*rence ral*s to tils fuii Pr:rt* cdteria varyir:g
beirvcen rl5?* an,J S??ir,nd*p*nding *n trte

nurnb*rs r:f {iDs and ii*r:**iq:n**cr:pi*s b*tiq'r*n paiients diagnr:sert as having

p*sitiv* anrl signifir"ani tirn* trsnd, shi:wing th* p*sitive *ff-**t

*pe *f IFD. Ther*

ws

t:f-

t* the difl*r*nc*s

tht guid*li*e audit *n cinii:al practic*. Th* evsil high*r rats Llf S?s,:

in dise*se distributinn" Althnugir n*t i* accnrdan*r with guideline s. paeriiatric *ndosc*pists ffi1ay decide t* rekain f-r*m ii*r:s**p;- anil {}{"rl} f* s&vt" tirule il,'}r*n the maLrsci:pic asp*ct r:f ti'l* cal*n is trlpicril fl*r iiil. Srnali nnri srr*dlun"i e*n{rss perfcrnn fbw*r *fi{}s but hal'e a higlier srircess raf* *f ikal i*nrb*tion thnn 1*rg* centrss. T"h* r*esrl*s fi"r tli*se diff*r*n*s are ni:f clear" bilt ths rcsults r*uld hiir''* b**n blassd b*caus* th*r* wsrLr *;li;* : iarge c'entres ir : r:*untri*s {L;K anci Pnlnnd. *th*r rcasons m*y b* dif?*renc*:s irr t**irnlcel *xp**ene*. llmited timc fl*r th* diagn*stie prcgrarri, en* clitferent in-i:*rs* strafegi*s, *tth*ugh th*s* n*t in ac**rdaRce rvith guid*lincs snd nr:t b*s*ci *r: anv rl.idenq-'. Railii:l*gical *xeminafi*n *f th* smril bcr'*i b,v SBFT, as prp*sfld in th* Pqiil* criteria, was highest in th* t'irst 2 vears cf Hi-R{}K}5, rvh*x 7T.r *f p*ti*nts diagn*s*d as having tt} anci IBI)-Lj i:nd*nryent SI3FT. As stat*d ir: the P-rrr: erit*ria" SSFT'r.viih b;irium r*ntr;:st prr":vi**s infbrn"rati*n *il the *xtent ar"tr pr:ssibl* c*mpii*nti*ns *f smali bcw*l inv*trvem*nf in C- inciuding st*nr:sis* st-icfur*, nr iniernai fisruias {?}. In ti:e .y*ars f*ll*wingth* us* *:f $*FT'cl*crri:s*d signifi*entl5r end was r*piaced by th* -i'!r; tus* rrf &{R1. Th is t*e hin ique has n* r aiieticn *xpr}5*ru, whe r*as S$i"'f is resp*nsibi* lor l$':.i to 3{i?i *i'ail radiatian *xpsurc in ehildr*n with lBll, ls wils denl*nstraf*d ln recrnt sfudies i18,11" In a.klitiq:n, acli.llt and pa*iliatric data have sl:*wn tirat \.{Rl i*'as rve rfirc sensitive tharr fl**r*sc$p3' in dcfr:cting ii*iris and inf;arnffia-

*f pati*nts

ciiagn*sed as havrng L=l] and

75?/r,

tr:rv changes in th* b*rvei rvail i-?*;31.[n

* reeent FSP*f-tAN-

and **i*n*se ilpv plr:s e ither ri*el intubatin *r snali b**'*l imaging, dr:curnents tl-lr high l*r,'*i *i'ace*ptan* *l the crit*ria *ntl s*ggcsts that othcr fuct*rs pi*.v* a rcle ln impiem*nteti*n. F*r *xampie, centr* size had nn i.irfluence *n pertb{rnancf; ratrs' indicaring that the results rnin-*r ilail,.., practicr in rJiag*r-rsine lBn iri*rc {han ke*ping t a st*dy prr:l*cr:l with **diti*nsi ci:*cks and r*utine:" In silffiffiary. tl"ris first anal_vsis *f the E{-,It*KIl}S r*gisfry sh*rvs that the :erf*rrn&nrf, *f {il} and il*r:col*nL}s{spy hns h*rn .+;nsfantlv rising sinc* thr: p*blic*ticn *f, the F*rt* criterin. Tlrking s*riai l':ii:psies fbr hisf*l*gy is an a*r*pted standard. Smali bcrv*tr im*ging b3,' h{t{J has increas*d ver the y**ars and }ras superserJsd the use *f SgFT. ln sme c*scs, T and capsule *ndss**py contribute ta th* ding*i:sis. Th* dragncstic: r,v$Fkup ean b* ftrther in:pr*v*d b_v incr*asing th*: sHCCfss rate *f-ileai inrubatir:n in aii pati*nts with lBl; and b-v stiniulating th* us* af small b*wei imaging, csp*ciail-v in patients di*gn*sed as having IB-ij. T.he diagnrstrc yield *f *{iL} tl.5%] *nd il**c*1qln*s$py { i ".1-%), conrbin*d with the ailditi**ai :,-'aJuc *f,sm*ii buwci in:aging. *mphasis*s ih* impr:rrtance *f a fui diagn*stic wcrkup in children and aii*lcscents wirh a s*spici*n *f having IBD. ln th* near *"rfur*, data from this S-y*ear HL,RKtIlS c*hort rl'i11 pr*viel* ac**rats anetr reiiabte inf*rmati*n an tli* untque phenr:t_vp* i:f pnediatri***ns*t I BD,

a: llal'ing tts)-U, wh* und*rrvent at ieast

*f patienfs

ciiagn*sr'.d

*nd*rsset Huri:p*an {-lr*hn's *nd {-r:litis rganisati*n guid*ili:e *n pii*datrie Cilj' klRl is r*{*mn:*rdrd as a prin:ary investigati*rr t**:1

imilging ir e hilciren with ttsD i j'li, but tri:cal expertis* sh*uld aisa be tek** intr: a*co*nt wl:cn chc*sing a small

f*r

RtrtRrN{*
1" Sawcenk*

s*"leil-b:*"v*1

bu'sl irnaging teci:niq*e

F*r insta*cs. evaiuatia* cf

'v-lRls

r*quires

ii" Pr*spee{ive slrrtr'*\' .:l' e hildhoscl i:flam*rat t:clw*l dis*;.rs* in the Sritirl" lsl*r'. lr,'ef ?tH] ;35?: i S$.i *4.
Sndhu

A.

8K, L*gan RF. ci

www.jpgn.rg

3?9

#*pyright 2*13 by ffi$FGhtAht and f{A$PGHAN. Una*th*rie*d r*pr*du*tinn sf this arti*le i* pr*hiblt*d"