eMedicine Specialties > Pediatrics: Surgery > General Surgery

Small Intestinal Atresia and Stenosis

Author: Jaime Shalkow, MD, Head of Surgical Oncology, Division of Surgery, National Institute of
Pediatrics, Mexico; Head-Professor of Pediatric Surgical Oncology, Universidad Nacional Autonoa de
Mexico
Coauthor(s): J Antonio Quiros, Assistant Professor of !linical Pediatrics, University of !alifornia-Davis
Sc"ool of Medicine; !onsulting Staff, De#artent of Pediatric $astroenterology and Nutrition, University
of !alifornia-Davis Medical !enter; Nicholas A Shorter, MD, Professor of !linical Surgery and !linical
Pediatrics, State University of Ne% &or'-Do%nstate University; Division !"ief, De#artent of Surgery,
Division of Pediatric Surgery, State University of Ne% &or'-Do%nstate Medical !enter
!ontri(utor Inforation and Disclosures
U#dated) Mar *, +,-,
Introduction
."e %ord atresia etyologically coes fro t"e $ree' a, %"ic" eans no or %it"out, and tresis, %"ic"
eans orifice/
0e1unoileal atresias and stenoses are a1or causes of neonatal intestinal o(struction/ Atresia refers to
a congenital o(struction %it" co#lete occlusion of t"e intestinal luen/ It accounts for 234 of
o(structions/ Stenosis, on t"e ot"er "and, refers to a #artial occlusion %it" inco#lete o(struction and
accounts for t"e reaining 34 of cases/
-
Intestinal atresia or stenosis can occur any%"ere along t"e $I tract, and t"e anatoic location of t"e
o(struction deterines t"e clinical #resentation/ Most ne%(orns %it" intestinal o(struction #resent %it"
(ilious eesis/ 5ilious voiting in t"e neonate s"ould (e considered secondary to a ec"anical
o(struction until #roven ot"er%ise, and eergency surgical evaluation is %arranted in every ne%(orn
%it" t"is sy#to/
."e survival of #atients %it" intestinal o(struction "as ar'edly i#roved over t"e last +, years
(ecause of an i#roved understanding of intestinal #"ysiology and t"e etiologic factors of t"e
condition, refineents in #ediatric anest"esia, and advances in surgical and #erio#erative care of
ne%(orns/
-
History of the Procedure
Ileal atresia "as long (een recogni6ed/ ."e first descri#tion of ileal atresia %as credited to $oeller in
-7*8/
-
In -2--, 9oc'ens re#orted t"e first successful surgical re#air of a #atient %it" sall intestinal
atresia/
+,-
Ho%ever, t"e ortality rate for t"e surgical correction of t"is condition reained "ig" for
any years, even in t"e (est #ediatric surgical centers/
+
In -233, :ou% and 5arnard deonstrated t"e role of late intrauterine esenteric vascular accidents as
t"e li'ely cause of 1e1unoileal atresias, rat"er t"an t"e #reviously acce#ted t"eory of inade;uate
recanali6ation of t"e intestinal tract/
<
Since t"at tie, ot"er factors suc" as in utero intussusce#tion,
intestinal #erforation, segental volvulus, and t"ro(oe(olis "ave also (een s"o%n to cause
1e1unoileal atresia/
8
Atresias can also develo# in #atients %it" gastrosc"isis and in t"ose %it" econiu
ileus/
Frequency
."e #revalence of 1e1unoileal atresia %idely varies aong different re#orts/ In 9rance, t"e #revalence
is +/+3 cases #er -,,,,, live (irt"s/
3
."e overall #revalence of sall intestinal atresia in S#ain and
:atin Aerica is -/< cases #er -,,,,, live (irt"s/
7
Intestinal atresia accounts for a(out one t"ird of all cases of neonatal intestinal o(struction/
=
In >est
Africa, intestinal atresia is t"e fourt" ost coon cause of neonatal intestinal o(struction after
anorectal alforations, Hirsc"s#rung disease, and strangulated inguinal "ernias/
*
In an ---year
retros#ective revie% of 3,, c"ildren in India, ?anan et al found intestinal atresias to (e t"e ost
coon cause of intestinal o(struction in ne%(orns and t"e second ost coon cause @--/*4A
after intussusce#tion @+,/*4A in all age grou#s/
2
5oys and girls are e;ually affected/
=
In ost studies, 1e1unoileal atresias see to (e ore coon
t"an duodenal atresias, and colonic atresias account for t"e fe%est nu(er of cases/
-,
Unli'e duodenal atresia, 1e1unoileal atresia associated %it" Do%n syndroe is uncoon/ Patients
%it" intestinal atresia are e#ideiologically c"aracteri6ed (y young gestational age and lo% (irt"
%eig"t, t"e atresia is associated %it" t%inning, t"e #arents are ore often consanguineous co#ared
%it" #arents of "ealt"y neonates, and vaginal (leeding fre;uently co#licates t"e #regnancies/ No
correlation (et%een 1e1unoileal atresia and #arental age or disease "as (een #roven/
3,7,8
Ho%ever, one
study in 9rance s"o%ed an increased #revalence of intestinal atresias in infants (orn to teenagers/
3
Soe aternal infections ay (e associated %it" ileal atresia/
7
Etiology
Segental differentiation of t"e e(ryonic gut starts as early as < %ee'sB gestation/ 9etal (ile
secretion and t"e s%allo%ing of aniotic fluid (egin in at ----+ %ee'sB gestation, %ell after
revacuoli6ation of t"e solid cord stage/
-
."e sall intestinal e#it"eliu in "uans is ature at (irt"/
:uinal co#onents suc" as aniotic fluid @(efore (irt"A and enteral nutrition @after (irt"A are not
essential for e#it"elial aturation/ ?at"er, e#it"elial #rotein ex#ression @genetically i#rintedA is crucial
for nutrient a(sor#tion, e#it"elial defense, and re#air of t"e sall intestine/
--
."e "ig"er #revalence of associated congenital alforations %it" duodenal atresia co#ared %it"
1e1unoileal atresia suggests t"at #roxial o(structions occur earlier in fetal life/
-+,-<
Unli'e duodenal atresia, any 1e1unoileal atresias are se#arated (y a cordli'e segent or a C-s"a#ed
esenteric ga#/ ."is finding and t"e usual finding of (ile #igents and lanugo "airs distal to t"e atretic
segent indicate t"at an in utero vascular accident t"at occurs relatively late in gestation @D----+
%ee'sB gestationA is li'ely t"e origin of t"ese atresias, rat"er t"an failure of $I tract recanali6ation/ A
locali6ed intrauterine vascular accident %it" isc"eic necrosis of t"e (o%el and su(se;uent
rea(sor#tion of t"e affected segent is t"e favored t"eory/
-8,<,+,-,-3
de !"adarevian et al @+,,2A re#orted
on an infant %it" in"erited t"ro(o#"ilia creating a "y#ercoagula(le state, favoring a segental
intestinal t"ro(osis and resulting in terinal ileal atresia/ ."is #atient %as also found to "ave
Hirsc"s#rung disease, %"ic" is rarely associated %it" intestinal atresias/
-7
."e locali6ed nature of a vascular insult ex#lains t"e lo% #revalence @-,4A of coexisting conditions/
Intestinal atresia associated %it" in utero intussusce#tion or #erforation, alrotation, volvulus, internal
"ernias, gastrosc"isis, and o#"alocele furt"er corro(orates a vascular event as t"e etiology of ost
1e1unoileal atresias/
-=,-3,-*,-2
S%eeney et al exained <* #atients %it" 1e1unal atresia and 83 #atients %it" ileal atresia at t"e
!"ildrenEs ?esearc" !enter in Du(lin, Ireland/
-<
!o#ared %it" #atients %it" ileal atresia, #atients %it"
"ig" 1e1unal atresia "ad a "ig"er rate of associated congenital alforations @8+4 vs +4A, "ad a
"ig"er rate of ulti#le or a##le-#eel @ty#e III(A atresias @3<4 vs 24A, and "ad a "ig"er ortality rate/
."ese results suggest t"at 1e1unal atresia ay also develo# fro a alforative #rocess/
In a colla(orative study in 9rance, $aillard et al revie%ed -,+ cases fro 8+ induced a(ortions and ++
still(orns, as %ell as surgical findings in <* neonates/
+,
A(noralities suc" as econiu ileus
@associated %it" cystic fi(rosisA and c"roosoal a(errations @eg, Do%n syndroeA %ere #resent
during t"e second triester of gestation/ Intestinal atresia and stenosis %ere detected in t"e t"ird
triester of #regnancy and %ere associated %it" isc"eic conditions/
Most infants %it" t"is condition "ave only a single atretic segent/ Ho%ever, ulti#le atresias "ave
(een descri(ed in infants of ot"ers %"o ingested ergotaine and caffeine, or #seudoe#"edrine alone
or in co(ination %it" acetaino#"en during #regnancy/
+-,++
Ot"er vasoconstrictive factors suc" as
cocaine a(use and so'ing during #regnancy "ave also (een associated %it" increased ris' for t"e
develo#ent of intestinal atresia/
++
Also, t"e ris' is "ig"er in #atients %it" graft versus "ost disease and
iunosu##ression and in t"ose %it" alforative #rocesses t"at are li'ely due to autosoal
recessive transission/
+<,+8
Multi#le intestinal atresias "ave (een re#orted in rare association %it" #yloric atresia and
#yloroc"oledoc"al fistula/
+3
In a study of --8 cases of 1e1unoileal atresia in t"e Net"erlands, Stollan et al found ot"er
gastrointestinal anoalies in +84 of #atients, genitourinary alforations in 24, cystic fi(rosis in 24,
neurologic anoalies in 74, and congenital "eart disease in 84/
+7
9ailial cases of various ty#es of atresia "ave (een %ell descri(ed/
+=
9ailial ty#e I 1e1unal atresia
affected < e(ers fro + generations in one faily/ Proxial atresia %as associated %it" renal
dys#lasia/ Fno%ledge of t"e failial for of t"e disease indicates t"at ost cases of 1e1unoileal atresia
actually result fro disru#tion of a noral e(ryologic #at"%ay, ost li'ely t"e develo#ent of t"e
su#erior esenteric artery and its (ranc"es/ ."ey s"ould (e considered to (e true e(ryologic
alforations rat"er t"an ac;uired lesions/
+=
."is association is #resua(ly an autosoal doinant condition/
+*
Matsuoto et al re#orted a case in
0a#an and revie%ed t"e literature, finding 7 ot"er cases of sall intestinal atresia occurring in t%ins/
+2
All #u(lis"ed cases exce#t one involved identical t%ins/ ."ree #airs of t%ins "ad different ty#es of
atresia, and 8 #airs did not "ave any ot"er anoalies/ ."e ot"er e(ers of t"ese failies %ere not
affected; t"is finding suggested t"at suc" cases ay (e due to environental influences during
gestation/
Anot"er re#ort of different intestinal atresias in identical t%ins #ro#oses t"e to (e eit"er t"e
conse;uence of lin'age of t%o genes or a #leiotro#ic ex#ression of a single gene/
<,
Pathophysiology
Dalla Cecc"ia et al #erfored a +3-year retros#ective revie% and found +== neonates %it" intestinal
atresia/
-+
."e level of o(struction %as duodenal in -<* #atients, 1e1unoileal in -+*, and colonic in +-/ Of
t"e +== neonates, -, "ad o(struction at ore t"an one site/ 0e1unoileal atresia %as associated %it"
intrauterine volvulus @+=4A, gastrosc"isis @-74A, and econiu ileus @--/=4A/
In atresias of t"e sall intestine, t"e 1e1unu and ileu are e;ually affected/
=,<-,-
."e #roxial 1e1unu
is t"e site of atresia in <-4 of cases, t"e distal 1e1unu in +,4, t"e #roxial ileu in -<4, and t"e
distal ileu in <74/
-
In ore t"an 2,4 of #atients, t"e atresia is single; "o%ever, ulti#le atresias are
re#orted in 7-+,4 of cases/
<+,-
Stollan et al @+,,2A #u(lis"ed one of t"e largest series of 1e1unoileal atresias as a retros#ective
revie% at a large #ediatric referral center in t"e Net"erlands/ 5et%een -2=8 and +,,8, t"ey found --8
infants %it" 1e1unoileal atresia/ Sixty-t%o #ercent of atresia and stenosis cases %ere noted in t"e
1e1unu, <,4 in t"e ileu, and *4 in (ot" t"e 1e1unu and t"e ileu/ Seven #ercent of #atients "ad
intestinal stenosis, -74 "ad ty#e I atresia, +-4 "ad ty#e II, +84 "ad ty#e IIIa, -,4 "ad ty#e III(, and
++4 "ad ty#e IC/
+7
Hei1 et al #erfored a retros#ective analysis of +- #atients %it" 1e1unal atresia and +8 %it" ileal atresia
and found ore differences t"an siilarities (et%een t"e grou#s @see .a(leA/
<<
."e ean (irt" %eig"t
and gestational age %ere significantly lo%er in #atients %it" 1e1unal atresia t"an in t"ose %it" ileal
atresia/ Most 1e1unal atresias %ere ulti#le, %"ereas ost ileal atresias %ere single/ Antenatal
#erforation occurred fre;uently @-, casesA in ileal atresia (ut infre;uently @+ casesA in 1e1unal atresia/
."e #osto#erative course %as often #rolonged, and t"e ortality rate increased in #atients %it" 1e1unal
atresia, aong %"o t"ree deat"s occurred @all in #atients %it" a##le-#eel deforityA/ 5y co#arison,
one #atient %it" ileal atresia died/
Hei1 et al suggested t"at a difference in co#liance of t"e (o%el %all (et%een t"e 1e1unu and ileu
ay ex#lain soe of t"eir findings/
<<
."e co#liant 1e1unal %all allo%s for assive dilatation %it"
su(se;uent loss of #eristalsis, accounting for t"e #rolonged #osto#erative course and t"e relatively
"ig" rate of #erforation in ileal atresias/
Differences 5et%een 0e1unal and Ileal Atresia
<<,3,-,7,8,<8
O#en ta(le in ne% %indo%
Characteristic Jejunal Atresia Ileal Atresia
$estational age :o%er t"an t"at of ileal atresia :o%
5irt" %eig"t :o%er t"an t"at of ileal atresia :o%
Atresias May (e ulti#le Si#le
Antenatal #erforation Uncoon !oon
Associated alforations Soe ?are
Posto#erative course Prolonged S"ort
Mortality Hig"er t"an t"at of ileal atresia :o%
Characteristic Jejunal Atresia Ileal Atresia
$estational age :o%er t"an t"at of ileal atresia :o%
5irt" %eig"t :o%er t"an t"at of ileal atresia :o%
Atresias May (e ulti#le Si#le
Antenatal #erforation Uncoon !oon
Associated alforations Soe ?are
Posto#erative course Prolonged S"ort
Mortality Hig"er t"an t"at of ileal atresia :o%
."e axial dilatation of t"e #roxial segent occurs at t"e #oint of o(struction/ ."is segent is
coonly a#eristaltic, of ;uestiona(le via(ility, or (ot"/
+
$rosfeld et al "ave odified :ou%Bs original
classification into t"e follo%ing ost coonly used descri#tion of intestinal atresia)
<-
.y#e I G Me(rane
.y#e II G 5lind ends 1oined (y fi(rous cord
.y#e IIIa G Disconnected (lind end
.y#e III( G A##le-#eel defority
.y#e IC G Multi#le, string of sausages
Stenosis
."e #roxial dilated intestine is in continuity %it" t"e distal nondilated (o%el, and t"e esentery is
intact/ 5et%een t"ese #ortions, a narro%, seirigid segent %it" a inute luen is #resent/ ."e sall
(o%el lengt" is noral/ ."is lesion ig"t siulate an atresia ty#e I @see Media file 8A/
Intestinal stenosis. Dilated prestenotic bowel is in continuity with the distal
intestine. No mesenteric gap is present. Bowel length is normal.
Atresia tye I (mem!rane)
."is ty#e is a ucosal @se#talA atresia %it" an intact (o%el %all/ ."e #roxial dilated intestine is
continuous %it" t"e distal narro% one/ ."e esentery is intact, and t"e intestinal lengt" is noral/ ."e
#ressure generated on t"e internal e(rane ay elongate it as a %indsoc', giving a conical
a##earance to t"e transition/ ."e distal intestine is colla#sed @see Media file 3A (ut ay contain
econiu/
he transition area has a conical appearance due to windsoc! elongation of
the membrane in atresia type I. No mesenteric gap is present. Bowel length
is normal.
Atresia tye II (!lin" en"s joine" !y a #i!rous cor")
In t"is ty#e, a fi(rous cord se#arates t"e #roxial (o%el fro t"e distal segent/ ."e esentery is
usually intact, (ut a sall, C-s"a#ed defect ay (e #resent/ ."e lengt" of t"e intestine is noral/ ."e
#roxial (lind #ouc" is grossly dilated, often a#eristaltic and cyanotic/ In addition, #erforations "ave
(een encountered in #atients %"o #resent late/ Dilatation usually extends #roxially -,--3 c, after
%"ic" t"e intestine assues a relatively noral a##earance/ ."e distal (lind #ouc" ay (e ildly
distended (ecause of retained cellular de(ris @as in fetal intussusce#tionA @see Media file 7A/
Intestinal atresia type II. he pro"imal dilated bowel is separated from the
distal narrow one by a fibrous cord# in this case# without a mesenteric gap.
Bowel length is normal.
Atresia tye III
.y#e III atresias see to (e t"e ost coon/
=,-,
Intrauterine resor#tion of fetal gut su(1ected to a
vascular insult ex#lains t"e reduced (o%el lengt" coonly seen in t"is ty#e of atresia/ ."e distal
(o%el is sall and deco#ressed/
Atresia tye IIIa ("isconnecte" !lin" en"s)
In t"is ty#e of atresia, (ot" (lind ends are co#letely se#arated %it"out a fi(rous cord (et%een t"e/
."e atresia "as a C-s"a#ed esenteric ga#, and t"e intestine is s"ortened @see Media file =A/ ."e
#roxial dilated #ouc" ay "ave ;uestiona(le via(ility and undergo torsion/
Intestinal atresia type IIIa. Both blind ends are separated completely. $ %&
shaped mesenteric gap is present. Intestinal length is shortened.
Atresia tye III! (ale$eel "e#ormity)
."is ty#e of atresia is also called t"e !"ristas-tree defority/ 5ot" intestinal segents are se#arated
as in ty#e IIIa, and t"e esenteric defect is large/ ."e #roxial atretic segent is in t"e u##er
1e1unu, near t"e ligaent of .reit6, and t"e #ouc" is distended and lac's dorsal esentery/ ."e
su#erior esenteric artery distal to t"e iddle colic (ranc" is a(sent/ ."e colla#sed distal intestine
"elically encircles a sall vessel @arginal arteryA t"at arises fro t"e ileocolic or rig"t colic arcades,
or t"e inferior esenteric artery, and its vascularity ay (e i#aired/
.y#e I or ty#e II atresias ay coexist in t"e distal segent/ ."e intestine is al%ays su(stantially
s"ortened @see Media file *A/ Many #atients %it" t"is variant "ave lo% (irt" %eig"t @=,4A and %ere
(orn #reature @=,4A; t"ey ay also "ave alrotation @384A, ulti#le atresias, and an increased
nu(er of ot"er associated anoalies t"at increase t"e #revalence of co#lications @7<4A and
ortality rate @38-=-4A/
<3,-,<7
Intestinal atresia type IIIB 'apple&peel or (hristmas&tree deformity). he
pro"imal pouch is dilated. he collapsed distal intestine encircles the
marginal artery helically. Intestinal length is substantially reduced.
Atresia tye I% (multile atresia)
.y#e IC atresia refers to any nu(er and co(ination of atresias ty#e I to III t"at #resent
siultaneously, creating a string-of-sausages a##earance @see Media file 2A/ A #ossi(le cause is
intrauterine inflaation/ Ho%ever, findings of t"is ty#e of atresia in faily e(ers suggest #ossi(le
autosoal recessive transission/
<=,<+,+8
Intestinal atresia type I%. *ultiple atresias appear simultaneously as a
string of sausages. he intestinal length is in+ariably and considerably
shortened.
."e #resence of ulti#le $I atresias %it" cystic dilatation of t"e (ile duct is rare; t"e association "as
(een descri(ed in <= #atients, %it" no recorded survivors in t"e %orld literature/
+*,<*
."e dilatation of t"e
(ile duct sees to (e due to noral drainage of (ile into a closed-loo# duodenal o(struction/ Patients
#resent %it" ulti#le atresias and die fro s"ort-(o%el syndroe and co#lications related to total
#arenteral nutrition @.PNA/
Presentation
0e1unoileal atresias can (e identified on t"e (asis of #oly"ydranios #resent during #renatal
ultrasonogra#"ic evaluation, (ilious voiting, a(doinal distension, and 1aundice/ Soe #atients ay
not #ass econiu in t"e first day of life/
!linical #resentation of #atients %it" 1e1unoileal atresia is as follo%s)
!oon c"aracteristics
o Poly"ydranios on #renatal ultrasound @+*4A
o Preaturity @<34A
o :o% (irt" %eig"t @+3-3,4A
!lassic signs
o 5ilious eesis t"at %arrants eergent surgical evaluation @ost #atientsA
o A(doinal distention @in distal atresiasA
o 0aundice @<+4A
o 9ailure to #ass econiu in t"e first +8 "ours @?ule out Hirsc"s#rung disease/
Passage of econiu does not rule out intestinal atresia/A
Signs of continuous fluid loss
o De"ydration, anifested (y sun'en fontanel and dry e(ranes
o Decreased urine out#ut @(est clinical indication of tissue #erfusionA
o .ac"ycardia
o Decreased #ulse #ressure
o :o%-grade fever
o Neurological involveent, anifested (y irrita(ility, let"argy, or coa
Patients are fre;uently #reature @<34A/
3
One t"ird of infants %it" 1e1unal atresia, one fourt" of t"ose
%it" ileal atresia, and ore t"an one "alf of t"ose %it" ulti#le atresias "ave lo% (irt" %eig"t/
-
Most #atients #resent %it" (ilious eesis, %"ic" indicates t"at t"e o(struction is distal to t"e a#ulla
of Cater/
."e #atientEs #ulse, res#iratory rate, (lood #ressure, and te#erature are usually initially %it"in t"e
reference range/ As t"e #atient loses fluid into t"e (o%el and (y voiting, diinis"ed #lasa volue is
reflected as tac"ycardia, decreased #ulse #ressure, and, soeties, lo%-grade fever/
Iediately after delivery, t"e #atient a##ears relatively "ealt"y/ Over tie, t"e #atient develo#s signs
of "y#ovoleia @sun'en eyes, sun'en fontanel, dry s'in and ucous e(ranes, and #rolonged
ca#illary refill tieA, %"ic" are due to voiting and intra-a(doinal t"ird-s#ace loss secondary to t"e
o(struction/ ."ese #atients are "ungry and #ro#erly suc' il'; "o%ever, t"ey cannot tolerate feedings
and continue to voit #rofusely/ ."ey eventually (ecoe let"argic and "y#oreactive, %it" uscle
flaccidity/ ."ey can develo# s'in ottling, cardiovascular insta(ility, and neurological involveent
@irrita(ility or coaA/
A #roxial sall-(o%el o(struction results in loss of fluids t"at rese(le gastric 1uice and t"us
#roduces "y#o'aleic and "y#oc"loreic eta(olic al'alosis/ >it" distal sall (o%el o(struction,
fluid losses are usually isotonic, so seru electrolytes are noral until sufficient de"ydration results in
eta(olic acidosis, as deonstrated (y tac"y#nea, lo% seru (icar(onate levels, and elevated seru
c"loride values/
Ade;uate tissue #erfusion is evaluated (y o(serving t"e #atientEs ca#illary refill tie, #ulse, (lood
#ressure, and urine out#ut/ If t"e #atient is severely de"ydrated, tenting of t"e s'in can (e noted/
A(out <+4 of infants %it" 1e1unal atresia and +,4 of t"ose %it" ileal atresia "ave 1aundice, %"ic" is
c"aracteristically due to indirect "y#er(iliru(ineia/
-,
A(doinal distension is ost evident in cases of ileal atresias, in %"ic" it is diffuse, as o##osed to
#roxial 1e1unal atresias, in %"ic" t"e u##er a(doen is distended and t"e lo%er a(doen is
sca#"oid/
Intestinal loo#s and t"eir #eristalsis ay (e seen t"roug" t"e t"in a(doinal %all of ne%(orns/
."ese (a(iesB a(doens are usually soft, %it"out signs of #eritonitis/ In utero #erforations usually seal
(efore delivery/ Ho%ever, an excessively dilated #roxial segent ay undergo torsion, necrosis,
andHor #erforation/ In t"ese cases, t"e #atient a##ears se#tic and de"ydrated, and t"e a(doinal %all
ay (e discolored/
."e #atientEs a(ility to #ass soe econiu does not exclude intestinal atresia/ !ellular de(ris and
s%allo%ed aniotic fluid and lanugo "airs for econiu, ex#laining t"is finding; t"is foration occurs
earlier in gestation t"an t"e insult t"at #roduces t"e atresia/
U#on la(oratory exaination, an elevated "eatocrit level secondary to "eoconcentration due to
reduced #lasa and extracellular fluid volue loss ay (e detected/ ."e >5! count ay (e eit"er
noral or elevated/ Patients ay #resent %it" indirect "y#er(iliru(ineia and t"e electrolyte
distur(ances entioned a(ove/
Di##erential "ia&nosis
Atresias s"ould (e distinguis"ed fro ot"er causes of neonatal intestinal o(struction/ Meticulous
"istory ta'ing and #"ysical exaination are t"e ost useful eleents in differentiating t"ese
conditions/ !linical settings and #araclinical studies to su##ort t"e decision-a'ing #rocess are
entioned/ ."e follo%ing are differential diagnoses of 1e1unoileal atresia and t"e indicated study
associated %it" t"e)
Malrotation %it" or %it"out idgut volvulus G !ontrast-en"anced u##er-$I study
Meconiu ileus G !ontrast-en"anced enea
Intestinal du#lication G !ontrast-en"anced study, ultrasonogra#"y
Internal "ernia G Intrao#erative exaination
!olonic atresia G Cisual ins#ection @%"ic" reveals diffuse distensionA
!olonic aganglionosis G ?ectal 5io#sy
Adynaic ileus G Ixaination @%"ic" reveals se#sisA and electrolyte distur(ances
In #atients %it" econiu ileus, a faily "istory and a c"loride s%eat test are i#ortant/ In t"is
su(grou# of #atients, t"e viscosity of t"e econiu #recludes t"e o(servation of air-fluid levels, and
t"e Neu"auser sign can (e revealed using radiogra#"y as a ground-glass a##earance in t"e rig"t
lo%er ;uadrant/ A contrast-en"anced enea t"at reveals reflux into t"e ileu is diagnostic/
Meconiu #eritonitis, due to in utero intestinal #erforation, can (e revealed using #lain a(doinal
iaging/ It a##ears as calcifications t"roug"out t"e #eritoneal cavity/
Patients %it" Hirsc"s#rung disease are not usually #reature or sall for gestational age/ A transition
6one can (e revealed using a (ariu enea study if enoug" tie to develo# "ig" intraluinal
#ressure and #roxial dilatation "as #assed/ ?ectal (io#sy findings are diagnostic
Imaging ,tudies
Prenatal ultrasonogra#"y
o Intestinal atresias can (e #renatally diagnosed using ultrasonogra#"y/
Poly"ydranios and ec"ogenic distended (o%el loo#s are t"e c"aracteristic findings/
Poly"ydranios is #resent in one fourt" of #atients overall, (eing ore coon in
cases of "ig" atresia @<*4A/
<2,8,,<7
o ."e s#ecificity of ultrasonogra#"y "as ar'edly i#roved over t"e last years/
Persistently dilated (o%el loo#s on serial sonogras "ave s"o%n a 77/=4 #ositive
correlation %it" intestinal atresia diagnosed after (irt"/
8-
."e fetus s%allo%s a(out +3-
8,4 of t"e aniotic fluid in t"e fourt" or fift" ont", and t"e fluid is rea(sor(ed in t"e
first +3-<, c of t"e 1e1unu/
8+
."erefore, #roxial atresias are easier to diagnose
t"an ileal or colonic atresias (ecause t"e distal lesions ay not (e associated %it"
#oly"ydranios/
o Unex#lained u(ilical cord ulceration and "eorr"age on #renatal ultrasonogra#"y
"as (een re#orted in fetuses sus#ected to "ave intestinal atresia/
8<
Plain a(doinal radiogra#"y of t"e 'idneys, ureters, and (ladder @FU5A
o A #lain a(doinal fil s"ould (e o(tained in every ne%(orn %it" an intestinal
o(struction/
o Alt"oug" considered #at"ognoonic of duodenal atresia, t"e dou(le-(u((le sign can
(e seen in very #roxial 1e1unal atresias/ ."e radiogra#"ic findings c"aracteristic of
1e1unoileal atresia are distended (o%el loo#s %it" air-fluid levels #roxial to t"e level
of t"e o(struction @see Media file -A/ ."e lo%er t"e atresia is in t"e $I tract, t"e
greater t"e nu(er of intestinal loo#s t"at a##ear distended on t"e radiogra#"/
Peritoneal calcifications, seen in -+4 of #atients, suggest econiu #eritonitis, a
sign of in utero intestinal #erforation/
Plain abdominal radiograph of a newborn re+eals a dilated
gastric bubble and a massi+ely dilated duodenum and pro"imal
-e-unum with a gasless abdomen distal to the le+el of the
obstruction. these findings are consistent with -e-unal atresia.
U##er $I series
o A contrast-en"anced u##er-$I is seldo re;uired/ ."is study is ty#ically #erfored to
rule out #artial o(struction or alrotation, %"ic" is #resent in -,4 of #atients %it"
1e1unoileal atresia/
-,
o >"en #erfored, t"e study s"o%s gastric dilatation and an enlarged sall (o%el u#
to t"e level of t"e atresia, %"ere a (lind #ouc" can (e seen @see Media file +A/ In
cases of intestinal stenosis, t"e #restenotic segent a##ears dilated, a transition
#oint is evident, and contrast aterial can (e seen in t"e distal (o%el/
/pper&0I contrast study demonstrates a dilated stomach and
duodenum# with an enlarged upper -e-unum and a lac! of
passage of contrast agent to the distal small bowel. these
findings are consistent with high -e-unal atresia.
5ariu enea study
o ."e radiogra#"ic iage of t"e colon of t"e ne%(orn lac's t"e c"aracteristic "austrae
seen in older c"ildren/ A contrast enea can (e useful to distinguis" large-(o%el
distension fro sall-(o%el distension, to identify a site of colonic or distal ileal
o(struction, to deterine t"e #resence of a icrocolon, and to identify t"e #osition of
t"e cecu; t"is last finding is useful in cases of a(noral rotation and fixation of t"e
intestine @see Media file <A/ Most neonates %it" 1e1unoileal atresia "ave an unused
icrocolon, exce#t %"en t"e vascular accident leading to atresia occurs late in
gestation, as in t"e case of idio#at"ic in utero intussusce#tion/
-=
Barium enema study re+eals a microcolon in an infant with
long&standing ileal atresia. (ourtesy of 1ebecca ,tein&2e"ler#
*D.
o ?ee(er t"at #atients %it" econiu ileus also #resent %it" a icrocolon/
Diagnostic Procedures
>"en a neonate does not #ass econiu in t"e first +8 "ours of life, Hirsc"s#rung disease
s"ould (e a concern/
In t"e a(sence of radiologic findings suggestive of atresia, suction rectal (io#sy s"ould (e
#erfored/ ."is is done at t"e (edside and does not re;uire anest"esia/
Histologic Findings
If t"e #atient "as Hirsc"s#rung disease, rectal (io#sy reveals an a(sence of ganglion cells and nerve
(undles "y#ertro#"y/
-3
reatment
,urgical herapy
."e 'ey for successful treatent of neonates %it" intestinal atresia is co#re"ensive #erio#erative
care/ ."is is usually (est acco#lis"ed (y a tea t"at includes ex#erienced surgeons, neonatologists,
and nutritional su##ort teas/ Iarly diagnosis, #ro#er #reo#erative sta(ili6ation, t"e rig"t c"oice of
surgical #rocedure, and good #osto#erative neonatal care are t"e ost i#ortant considerations/
Preoperati+e Details
Neonates tolerate surgical #rocedures (est %"en t"ey are eta(olically and "eodynaically sta(le/
Attention s"ould (e directed to #revent or correct "y#ot"eria, "y#ovoleia, "y#oglyceia, and
"y#oxeia/
."e #atient is aditted to t"e neonatal intensive care unit and restricted to not"ing (y out" @NPOA/
."e (a(y s"ould (e 'e#t in a %ar environent @eg, an incu(atorA %it" "uidified air, and t"e oxygen
saturation s"ould (e onitored/ Cital signs s"ould (e fre;uently assessed/ ."e air%ay is 'e#t clear
%it" fre;uent naso#"aryngeal as#iration/ Intu(ation for res#iratory su##ort in #atients %it" severe
a(doinal distension or se#sis ay (e necessary/ 5aseline la(oratory investigations are #erfored,
and (lood is cross-atc"ed/ Use of u(ilical lines s"ould (e avoided (ecause of t"e increased ris' of
infection and (ecause t"ey (ecoe su(o#tial for t"e transverse incision for la#arotoy/ S#ecific fluid
and electrolyte anageent of t"e ne%(orn #atient are (eyond t"e sco#e of t"is c"a#ter/ Acute
"y#ovoleia is anaged %it" -,- to +,-:H'g (oluses of lactated ?inger solution/
An orogastric tu(e s"ould (e #laced for gastric deco#ression and to avoid as#iration/ Neonates are
o(ligate nasal (reat"ers; (reat"ing t"roug" t"e out" is a learned reflex t"at occurs around age <
ont"s/ $astric out#ut ust (e re#laced as %ell/ 5ecause urine out#ut is t"e (est clinical indicator of
"eodynaic sta(ility, a (ladder cat"eter is used to ensure an out#ut of --+ :H'gH"/ 5efore surgery,
- g of vitain F is intrauscularly @IMA adinistered, and (road-s#ectru anti(iotics are
intravenously @ICA adinistered/
Neonates lose "eat ore ra#idly and "ave "ig"er eta(olic re;uireents t"an older #atients/ !are
ust (e ta'en in 'ee#ing t"e eta(olic deands satisfied and t"e (a(y %ar at all ties, including
during trans#ort to t"e surgical suite and during induction of anest"esia/
Intraoperati+e Details
During anest"esia, t"e #atient is carefully onitored/ ."e (lood loss is ;uantified and t"e intravascular
volue 'e#t ade;uate/
!lassically, t"e a(doen is entered t"roug" a su#rau(ilical transverse incision @see Media files -,-
--A/
he surgical approach for patients with -e-unoileal atresia is through an
upper&right quadrant trans+erse incision that can e"tend across the midline
if necessary.
,urgical image of a -e-unal atresia type IIIa# with a pro"imal dilated pouch#
completely separated from the distal narrow intestine# o+er a %&shaped gap
mesenteric defect.
.ransu(ilical and la#arosco#ic a##roac"es "ave (een re#orted lately/
88,83
Of course, advanced
la#arosco#ic s'ills are necessary/ ."e entire intestine is delivered t"roug" t"e incision to assess t"e
anatoy and ty#e of atresia and to rule out ot"er anoalies/ A #erforation, if #resent, s"ould (e
controlled at t"is stage (efore furt"er ex#loration is done/ ."e definitive %ay to exclude distal atresias,
%"ic" occur in 7-+-4 of #atients, is to irrigate noral saline solution into t"e distal #ouc" and to il' it
caudally/ If ot"er anoalies are ruled out, t"e intestine is returned to t"e a(doinal cavity %"ile
'ee#ing t"e atretic segent ex#osed/ >"en t"e intestinal lengt" is noral, t"e dilated #roxial #ouc"
can (e resected, (y reoving -,--3 c of dilated (o%el #roxial to t"e atresia, to avoid #osto#erative
#"ysiologic o(struction due to lac' of #eristalsis/
Instillation of noral sodiu c"loride solution %it" a +8-gauge needle t"roug" a #ursestring suture into
a cla#ed distal #ouc" ay (e useful to distend t"at segent and to reduce t"e si6e discre#ancy
(et%een t"e #roxial and distal intestine @see Media file -+A/ ."e #roxial intestine is transected at a
rig"t angle to axii6e its vascularity, %"ereas t"e distal (o%el is transected o(li;uely and t"e incision
is continued along t"e antiesenteric (order as a fis" out" to e;uali6e t"e si6e of t"e o#enings on
(ot" sides for t"e anastoosis @see Media file -<A/
87
Intestinal atresia type IIIa. $ clamp is applied on the distal bowel# and
sodium chloride solution is instilled through a purse&string suture to dilate
the intestine and diminish the si3e discrepancy between the two loops to
facilitate the anastomosis. he dotted line mar!s the area of the resection.
he pro"imal dilated pouch is transected in a 456 angle to ma"imi3e its
+ascularity# while the distal intestine is transected obliquely to diminish the
si3e discrepancy between the segments.
:ou% et al conducted ex#erients t"at #recluded t"e (lood su##ly to soe #arts of t"e intestine under
develo#ent/
<
."e aut"ors concluded t"at it %as li'ely t"at t"e (lood su##ly to #ortions of t"e (o%el
ad1acent to t"e atretic segent %ould (e co#roised, not enoug" to cause necrosis (ut sufficient to
cause a functional #ro(le %it" resultant defective #eristalsis/ ."us, t"ey recoend resection of t"e
(lind (ul(ous end of t"e #roxial intestine #rior to t"e anastoosis/ ."e ortality of intestinal atresia
at $reat Orond Street Hos#ital decreased fro 724 to <<4/
8=
A -- or +-layer, end-to-(ac' @end-to-o(li;ueA anastoosis is #erfored/ ."e esenteric ga# is t"en
a##roxiated %it" fine a(sor(a(le sutures, ta'ing care not to 'in' t"e anastoosis or to daage t"e
esenteric vessels/ Patency of t"e anastoosis can (e tested (y il'ing intestinal air t"roug" it/ ."e
intestinal segent is t"en oistened %it" %ar saline solution and returned to t"e a(doinal cavity/
."e a(doinal %all is closed in layers %it" a(sor(a(le sutures/
Intestinal stenosis and ty#e I atresias @e(ranesA s"ould (e treated in t"e sae %ay descri(ed
a(ove/ 5y#ass #rocedures are generally su(o#tial (ecause t"ey fail to reove t"e a(noral
intestine, and side-to-side anastooses "ave t"e ris' of creating (lind loo#s/ 9or e(ranous
atresias, e(rane excision %it" transverse entero#lasty is soeties a##ro#riate, #articularly %"en
e(ranes are #resent as #art of ulti#le atresias/
$astrostoy tu(es are not routinely used, and #osto#erative nasogastric suction suffices for gastric
deco#ression/ ."is is t"e aut"orsE #reference/ Ho%ever, soe aut"ors recoend using a
gastrostoy tu(e in a very "ig" 1e1unal atresia for stoac" deco#ression and to #ass a
transanastootic tu(e for early #osto#erative enteral dri# feeding @see Media file -8A/
+
Anot"er o#tion
for early #osto#erative enteral feeding is to #ass a naso1e1unal transanastootic tu(e; "o%ever, t"e
"ig" incidence of nasal sores %it" long-ter nasal tu(es ust (e 'e#t in ind/
0astrostomy used for stomach decompression and to pass a
transanastomotic tube for early postoperati+e enteral feeding.
>"en (o%el lengt" is reduced @for ty#e III or IC atresiasA and t"e #atient is in danger of "aving a
severely s"ortened intestine, an antiesenteric ta#ering 1e1uno#lasty ay (e #erfored over a +79
tu(e (y diinis"ing t"e diaeter of t"e #roxial (o%el and #reserving (o%el lengt"/ ."is can (e
anually #erfored or #erfored using a $IA sta#ler and overse%ing t"e sta#le line %it" :e(ert
sutures @see Media files -3--7A/ Soe #atients re;uire re#eated surgical #rocedures to anage
functional o(struction due to an a#eristaltic, dilated (o%el at t"e site of t"e anastoosis/
0astrostomy used for stomach decompression and to pass a
transanastomotic tube for early postoperati+e enteral feeding.
*anually sewn tapering -e-unoplasty.
If t"e need for long-ter .PN is antici#ated, a central venous cat"eter is #laced/ ."e aut"or #refers to
#lace t"e central cat"eter in t"e sae anest"etic #rocedure, after t"e la#arotoy/
In #atients %it" atresia associated %it" gastrosc"isis, closure of t"e a(doinal %all ta'es #riority over
t"e re#air of t"e atresia/
8*
Indications for #riary anastoosis versus stoa foration de#end on t"e
degree of daage, t"e dilatation of t"e #reatretic intestine at initial #resentation, and t"e overall
edical condition of t"e #atient/ Alt"oug" #riary anastoosis is #referred, it is not advisa(le %"en
t"e vascular integrity of t"e intestine is ;uestiona(le or %"en severe #eritonitis or co#licated
econiu ileus are #resent/ In suc" cases, resection of t"e atretic segent and enterostoy are
advisa(le/
>"en ulti#le atresias are #resent, a 1udgent ust (e ade a(out %"et"er all atresias can (e
anaged at t"e sae tie or %"et"er a staged re#air is necessary/ In t"e latter case, a te#orary
ostoy or ostoies are used at t"e a##ro#riate levels/ One-stage restoration of intestinal continuity
%it" #reservation of axial intestinal lengt" s"ould (e t"e (asic #rinci#le of any o#erative
anageent in cases of ulti#le intestinal atresia/
82
&ardley et al @+,,*A re#orted on a case of
co(ined ulti#le 1e1unoileal and colonic atresia anaged %it" 2 #riary anastooses over a
gastro#erineal transanastootic tu(e/
3,
."is sees to (e a good alternative in order to avoid t"e use of
stoas and t"eir attendant co#lications/
$rosfeld et al re#orted on t"e treatent of -+* #atients %it" 1e1unoileal atresia in t"eir +3-year revie%
of #atients at 0aes >"itco( ?iley Hos#ital for !"ildren in Indiana#olis, Indiana/
<-
?esections %ere
#erfored in 2= @=74A of -+* #atients @anastoosis in 83 J874K, ta#ering entero#lasty in +< J+84K,
te#orary ostoy in +2 J<,4KA, ostoy alone in +3 @+74A, %e( excision in 3 @34A, and t"e 5ianc"i
#rocedure in - @-4A/
Stollan et al re#orted t"at #riary anastoosis %as #erfored in 724 of --8 infants %it" 1e1unoileal
atresia, %"ile te#orary enterostoies %ere used in +74/
+7
Postoperati+e Details
After t"e #rocedure, t"e #atient is transferred to t"e neonatal I!U/ ."eroregulation %it" an incu(ator
is ost i#ortant/ Oxygen saturation s"ould (e onitored, and aintenance fluids are adinistered/
."e gastric out#ut is closely onitored and re#laced volue for volue/ Ixtra (oluses of -,-+, :H'g
of lactated ?inger solution ay (e necessary to aintain urine out#ut at --+ :H'gH"/ .ransfusion is
adinistered if indicated/
$lucose, "eoglo(in, electrolytes, and (iliru(in levels are fre;uently onitored during t"e first
#osto#erative days, and ad1ustents are ade accordingly/ P"otot"era#y to avoid 'ernicterus is
soeties necessary/
Alt"oug" t"e literature does not su##ort t"e use of #ro#"ylactic anti(iotics (eyond +8-8* "ours after
t"e #rocedure, t"e aut"ors continue anti(iotic coverage for 3 days after surgery/
Interal feedings are carefully started after signs of #ro#ulsive #eristalsis occur, as indicated (y clear,
lo%-volue nasogastric out#ut; a soft, nondistended a(doen; and evidence t"at t"e (a(y is #assing
flatus or stool/
Interal inta'e can (e started %it" ost oral re"ydration solutions at sall rates/ If tolerated, (reast
il' is #refera(le to any coercial forula/ If (reast il' is not availa(le eit"er fro t"e ot"er or
t"e il' (an', diluted or "alf-strengt", "alf-volue forula can (e used/ ."e concentration and volue
are #rogressively increased as t"e #atient tolerates t"e forula/ Hydroly6ed isotonic or lo%-osolality
forulas (ased on co%Es il' #rotein are recoended for t"is setting/
Ac"ieving full enteral nutritional su##ort ay ta'e several days @ean, 3-=A or longer, even ont"s
later/ In #atients in %"o return of intestinal function is #redicted to (e #rolonged, centrally or
#eri#"erally delivered .PN is of #rie i#ortance/
$uidelines for .PN are %ell esta(lis"ed, and t"e use of .PN s"ould (e 1udicious/ Istiated nutrition
goals in t"e first 7 ont"s of life are a##roxiately -,,---, 'calH'gHd and +-< g of #rotein/ A
co#licated #osto#erative #eriod increases t"e #atientEs caloric needs (y +,-<,4 and s"ould (e ta'en
into consideration/ .PN s"ould (e advanced over <-3 days to caloric goals, and fat inta'e s"ould not
(e neglected/ A general rule is t"at calories fro fat @enteral, IC as intrali#id forulations, or t"e li'eA
s"ould (e 8,4 of total calories/ ."e goal for IC fat inta'e for neonates is < gH'gHd/
Alt"oug" .PN is t"e ain ad1unctive treatent for t"ese cases, it delays intestinal ada#tation and ay
cause c"olestasis and su(se;uent liver daage/
<7
."erefore, .PN s"ould (e a (ridge to full enteral
nutrition, and a concerted effort s"ould (e ade to use it as suc"/ As intestinal function returns, t"e
#atient is #rogressively %eaned fro #arenteral to enteral nutrition until t"e full nutritional re;uireents
are enterally o(tained/
$raduated enteric feedings and gro%t" "orone, glutaine, and odified diets "ave (een used to
successfully diinis" .PN re;uireents and en"ance nutrient a(sor#tion/
-+
Alt"oug" t"is feeding is
(eing esta(lis"ed, eta(olic surveillance of #atients is i#ortant/ Daily la(oratory %or'u# s"ould
include a (asic eta(olic #anel and assessent of calciu, #"os#"orus, and agnesiu levels/
.riglyceride levels s"ould (e onitored %"ile IC fat forulations are advanced until t"e goal is
reac"ed/ Persistent "y#ertriglycerideia ay reflect eta(olic dysfunction and %arrants furt"er
evaluation/ Also, IC fat s"ould (e %it""eld fro se#tic or "eodynaically unsta(le #atients until t"is
#ro(le is solved/
A #"ysiologic #rinci#le to ree(er is t"at t"e longer t"e tie t"at ela#ses %it"out enteral feedings,
t"e ore severe t"e (rus" (order atro#"y and t"e longer t"e tie t"at %ill (e needed to restore
a(sor#tive ca#a(ilities of t"e intestine/ Interocytes are exclusively nouris"ed (y glutaine/ :ac' of
glutaine su##leentation reduces levels of iunoglo(ulin A and increases (acterial translocation/
."is #"otosensitive su(stance s"ould (e added to t"e #atientEs #arenteral nutrition/ Overall, t"e
#rinci#le is to start enteral feedings as soon as t"e #atientEs clinical condition #erits/
."e current definition of intestinal failure is no longer anatoic, (ut rat"er, functional; intestinal failure
is no% defined as t"e #resence of ala(sor#tion after clinically significant sall-(o%el resection/
3-
Alt"oug" t"e inial lengt" of intestine necessary to aintain nutritional status is not fixed, ne%(orns
are estiated to re;uire at least -,-+, c of #ostduodenal sall (o%el to avoid s"ort-(o%el syndroe
if t"e ileocecal valve and colon are #reserved/ >"en t"e ileocecal valve is resected, t"is re;uireent
increases to 8, c/
3+
."e loss of t"e ileu is t"e ost difficult to co#ensate/ ."e 1e1unu cannot
#erfor soe ileal functions, suc" as t"e a(sor#tion of (ile acid and vitain 5--+/ ."erefore, a #atient
%it" ileal loss needs nutritional su##leentation/ ."e final lengt" of t"e intestine is difficult to
deterine at t"e tie of t"e o#eration (ecause t"e lengt" of t"e #roxial dilated #ouc" ay (e
overestiated, t"e neonatal (o%el is undergoing gro%t", and t"e final lengt" ay (e su(stantially
larger t"an t"e lengt" seen at surgery/
Surgical (o%el-lengt"ening #rocedures and sall-(o%el trans#lantation are (eyond t"e sco#e of t"is
c"a#ter, (ut t"ey ay #rove (eneficial in #atients of s"ort-(o%el syndroe associated %it" ulti#le
atresias/ ."is is an exciting area, %it" ne% develo#ents and o##ortunities to i#rove t"e survival and
;uality of life in t"is #atient #o#ulation/
7utcome and Prognosis
5efore t"e id-+,t" century, t"e ortality rate associated %it" sall-(o%el atresias %as #ro"i(itive
@D2,4A/ ."e survival rate increased to =*4 (y t"e late -23,s/ Survival rates i#rove %it" distal
atresias, %"ereas ortality rates are "ig" in instances of ulti#le atresia @3=4A; a##le-#eel defority
@38-=-4A; and atresias associated %it" econiu ileus @734A, econiu #eritonitis @3,4A, or
gastrosc"isis @774A/
<3,-,<7,-3
Overall survival rates @including #reter (a(iesA "ave reac"ed 2,4, %it" a surgical ortality rate of
less t"an -4/
<3,-+,<7
Mortality is related to se#sis, associated anoalies, #reaturity, alrotation,
econiu #eritonitis, and long-ter .PN co#lications in #atients %it" s"ort-(o%el syndroe/
."e ost coon cause of deat" in infants %it" 1e1unoileal atresia is infection related to #neuonia,
#eritonitis, or se#sis/
=,-
Sato et al @+,,2A re#orted on an infant %it" ileal atresia and econiu
#eritonitis after a #erforation %"o #resented %it" #yle#"le(itis @air in t"e #ortal systeA and a
#ulonary gas e(olis/ ."e #atient "ad res#iratory distress, s"oc', disseinated intravascular
coagulation, and intracta(le diarr"ea (ut eventually recovered and %as disc"arged fro t"e "os#ital
after 8 ont"s/
3<
."e ost i#ortant surgical co#lications are anastootic lea's and functional o(struction at t"e level
of t"e anastoosis; t"ese occur in as any as -34 of #atients/
= -,
In *8 #atients %it" congenital
1e1unoileal or colonic atresia %"o %ere treated in Ne% Sout" >ales, Australia, t"e ortality rate %as
"ig"er in infants %"o under%ent stoa foration t"an in #atients %"o received #riary anastoosis/
38
S"ort-(o%el syndroe refers to a s#ectru of alnutrition #ro(les t"at result fro inade;uate (o%el
lengt", %"ic" ay occur in #atients (orn %it" ulti#le atresias or in t"ose %it" t"e a##le-#eel defority
ty#e/ It is a cause of intestinal failure, toget"er %it" ot"er congenital diseases of enterocyte
develo#ent, and severe otility disorders @total or su(total aganglionosis or c"ronic intestinal
#seudo-o(struction syndroeA/
33
Ot"er coon causes of s"ort-(o%el syndroe include necroti6ing
enterocolitis and idgut volvulus/
37
Of t"e --8 #atients involved in t"e study (y Stollan et al, +*4 develo#ed early #osto#erative
co#lications, %"ereas -=4 ex#erienced late #osto#erative co#lications/ ."ey noted a ortality rate
of --4/
+7
S"ort (o%el syndroe sees to (e t"e (iggest #ro(le, resulting in longer "os#ital stay,
ore feeding #ro(les, and "ig"er or(idity and ortality rates/
.oday, t"e survival rate for #atients %it" s"ort-(o%el syndroe is *,-284/ ."e #resence or a(sence of
t"e ileocecal valve does not a##ear to affect t"e ortality rate/ Ho%ever, it does nota(ly affect t"e
lengt" of tie t"at .PN is re;uired and, t"erefore, affects t"e co#lications related to its use @eg,
#redis#osition to infections, central line se#sis, .PN-related c"olestasisA/
-+
Mala(sor#tion and
steatorr"ea are ost severe in #atients %it" terinal ileal resection, #articularly t"ose in %"o t"e
ileocecal valve is excised/ Citain 5 su##leents are useful in t"is su(set of #atients/
Overall ortality due to intestinal atresia does not see to de#end on t"e location of o(struction/
Preaturity, (irt" %eig"t less t"an + 'g, and associated anoalies are inde#endent ris' factors for
#rolonged "os#ital stay and "ig"er ortality rate/
3=,3*
:ac' of sufficient residual (o%el is res#onsi(le for considera(le or(idity or a #oor ;uality of life/ In
ost instances, axial intestinal ada#tation occurs %it"in 7--+ ont"s (ut ay ta'e longer/
-3
."e
use of t"e :ongitudinal Intestinal :engt"ening and .ailoring @:I:.A #rocedure, #ro#osed (y 5ianc"i and
odified (y Aigrain, can allo% t"e c"ild to (e %eaned fro #arenteral nutrition/
32
Future and (ontro+ersies
."e use of gro%t" factors to facilitate intestinal ada#tation and advances in sall-(o%el trans#lantation
ay furt"er i#rove t"e long-ter outcoe of c"ildren %it" sall intestinal atresia and stenosis in t"e
future/ Alt"oug" ortality and or(idity rates are still "ig", even in large ex#erienced centers,
intestinal trans#lantation is eerging as a feasi(le anageent o#tion %"en intestinal failure is
irreversi(le and for c"ildren %it" serious .PN-related co#lications/