CHAPTER 22 Jejuno-ileal Atresia
Heinz Rode, Alastair J.W. Millar
INTRODUCTION
Defects in the continuity of the small bowel can mor-
phologically be divided into either stenosis or atresia
and represent one of the most common causes of
neonatal intestinal obstruction. Jejuno-ileal atresia
has a prevalence rate of approximately 1:1000 live
births, with a third of the infants being either born
prematurely or small-for-date. The anomaly is rarely
genetically determined and <1% of babies will have
chromosomal or other associated anomalies.
Most jejuno-ileal atresias or stenoses result from a
localized intra-uterine vascular insult to the develop-
ing bowel with ischaemic necrosis and subsequent
reabsorption of the affected segment(s). Additional
pathology in the form of intra-uterine fetal intussus-
ception, malrotation and midgut volvulus, throm-
boembolic occlusions, transmesenteric internal her-
nias and incarceration or snaring of fetal bowel in a
gastroschisis or exomphalos further supports the
ischaemic hypothesis. The ischaemic insult also ad-
versely influences the structure and subsequent
function of the remaining bowel. Histological and
histochemical abnormalities can be observed up to
20 cm cephalad from the end of the atretic proximal
segment. The distal bowel is unused and potentially
normal in function.
Prompt recognition of intestinal atresia is essen-
tial for the early institution of management. A prena-
tal history of polyhydramnios and ultrasonography
showing dilated and obstructed fetal intestine are
strong indicators of congenital intestinal atresia. A
positive family history will help to identify heredi-
tary forms. Postnatally, intestinal atresia or stenosis
may present initially with large intragastric volumes
at birth (>20 ml gastric aspirate) followed by persis-
tent bile-stained vomiting. In 20% of children symp-
toms may be delayed for more than 24 h. Abdominal
distension is frequently present from birth and the
more distal the obstruction the more generalized the
abdominal distension. Proximal jejunal atresia often
presents with gastric distension and one or two loops
of visible bowel in the upper abdomen relieved by
nasogastric tube aspiration. With delay in presenta-
tion increasing intraluminal pressure and/or secon-
dary torsion of the proximal atretic distended bowel
may lead to ischaemia, perforation and peritonitis.
The differential diagnosis includes midgut volvu-
lus, intestinal stenosis, meconium ileus, duplication
cyst, internal hernia, strangulated inguinal hernia,
Hirschsprung’s disease and ileus due to sepsis, birth
trauma, maternal medications, prematurity or hypo-
thyroidism.
The diagnosis of jejuno-ileal atresia can be estab-
lished in most cases by an abdominal roentgenogram
with air as contrast medium. Erect and supine ab-
dominal radiographs show distended air and fluid
filled loops of bowel.
The lower the obstruction the greater the number
of distended loops of bowel and fluid levels that will
be observed. Occasionally intraperitoneal calcifica-
tion may be seen on the plain radiographs signifying
intrauterine bowel perforation, meconium spill and
dystrophic calcification. In the presence of complete
obstruction a contrast enema is usually performed to
confirm the level of obstruction and document the
calibre of the colon, exclude colonic atresia, and to lo-
cate the position of the caecum as an indication of
malrotation. With incomplete upper small bowel ob-
struction an upper gastrointestinal contrast study is
indicated to demonstrate the site and nature of the
obstruction and to exclude midgut volvulus.
The clinical and radiological presentation of jeju-
no-ileal stenosis will be determined by the level and
degree of stenosis. The diagnosis is often delayed for
years because of subclinical symptoms and findings.

214
Figure 22.1–22.3
The morphological classification of jejuno-ileal atre-
sia into types I–IV has significant prognostic and
therapeutic implications. The most proximal atresia
determines whether it is classified as jejunal or ileal.
Although single atresias are most commonly en-
countered, 6–12% of infants will have multiple atretic
segments and up to 5% may have a second colonic
atresia. The appearance of the atretic segment is de-
termined by the type of occlusion, but in all cases the
maximum dilatation of the proximal bowel occurs at
the site of the obstruction and is often hypoperistal-
tic and of questionable viability when presentation is
delayed.
쐽 Stenosis (12%) is characterised by a short local-
ized narrowing of the bowel without discontinuity
or a mesenteric defect. The bowel is of normal
length.
쐽 Atresia type I (23%) is represented by a translumi-
nal membrane or short atretic segment causing
complete intestinal obstruction. The bowel re-
mains in continuity, has no mesenteric defect, and
is of normal length.
쐽 Atresia type II (10%) has the blind-ending proxi-
mal bowel attached to the collapsed and underde-
22
Heinz Rode, Alastair J.W. Millar
veloped distal bowel by a fibrous cord along the
edge of the mesentery. The proximal bowel is dis-
tended and hypertrophied for several centimetres.
There is no mesenteric defect and the bowel
length is not foreshortened.
쐽 Atresia type III(a) (16%) is similar to type II ex-
cept that the fibrous connecting cord is absent and
there is a V-shaped mesenteric defect. The bowel
length may be foreshortened.
쐽 Atresia type III(b) (apple peel) (19%) consists of a
proximal jejunal atresia often with associated
malrotation, absence of most of the superior mes-
enteric artery and a large mesenteric defect. The
distal bowel is coiled in a helical configuration
around a single perfusing artery arising from the
right colic arcades. There is always a significant
reduction in intestinal length. The infants are usu-
ally of low birth weight and may have associated
anomalies.
쐽 Atresia type IV (20%) represents multiple seg-
ment atresias like a string of sausages or a combi-
nation of types I–III. Bowel length is always re-
duced.
Chapter 22 Jejuno-ileal Atresia
215

Figure 22.1

Stenosis Type I

Figure 22.2

Type II Type III(a)

Figure 22.3

Type IV

Type III(b)

216
Figure 22.4
The infant is placed supine on a warming blanket and
the exposed abdomen cleaned and sealed with plas-
tic adherent drapes. Access to the abdominal cavity is
obtained through an adequate transverse supra-um-
bilical incision transecting the rectus abdominis
muscles. The ligamentum teres is divided between
ligatures.
Exploration and basic surgical considerations: the
small intestine can easily be exteriorised from the ab-
dominal cavity by gentle pressure on the wound edg-
es and manual delivery of the intestine. Anatomic-
pathological findings will determine the operative
procedure.
Steps in the operative procedures are:
쐽 Identification of pathological type and possible
aetiology.
쐽 Confirmation of patency of distal small and large
bowel with saline injection (patency of the colon
could have been demonstrated by contrast enema
prior to surgery).
쐽 Resection of the proximal bulbous atretic segment.
쐽 Volvulated bowel must be untwisted carefully, es-
pecially in type III(b) atresia.
쐽 Limited distal bowel resection.
쐽 Accurate measurement of residual bowel length
proximal and distal to the anastomosis.
Figure 22.5
쐽 Detection of Distal Atretic Areas. It is imperative
to exclude distal atresia, which have a prevalence rate
of 6–21%. This is best achieved with a preoperative
barium enema to exclude an associated colonic atre-
sia and by injecting saline into the distal collapsed
small bowel and following the fluid column distally
until it reaches the caecum.
22
Heinz Rode, Alastair J.W. Millar
쐽 Single-layer end-to-end or end-to-back anastom-
osis.
쐽 Bowel lengthening procedures have no place dur-
ing the initial surgery.
쐽 Gastric decompression post-operatively is best
achieved with a Replogle tube on low continuous
suctioning. Neither decompression Stamm gas-
trostomy nor transanastomotic feeding tubes are
recommended.
쐽 The fashioning of proximal or distal stomas are
only indicated in the presence of established peri-
tonitis, or with compromised vascularity of the re-
maining intestine.
쐽 Additional steps may include derotation of a prox-
imal jejunal atresia, back resection to the distal
second part of the duodenum and excision or in-
version tapering of the duodenum if very dilated.
Where total bowel length is significantly reduced
(type III and type IV), the bulbous dilated seg-
ment proximal to the atresia is conserved. As pro-
grade peristalsis of this bowel is deficient the lu-
men calibre should be reduced. Maximum muco-
sal conservation is achieved by inversion plication
prior to anastomosis to the distal bowel.
쐽 Bowel Length Measurement. The total length of
the small bowel is measured along the antimesenter-
ic border. Once bowel resection has been completed,
residual bowel length is of prognostic significance
and may determine the method of reconstruction es-
pecially in types III and IV atresia. The normal bow-
el length at full term is approximately 250 cm and in
the premature infant it is 115–170 cm.
Chapter 22 Jejuno-ileal Atresia
217

Figure 22.4

Ligament of Treitz

Figure 22.5

218
Figure 22.6
The atretic region and the adjacent distended proxi-
mal and collapsed distal bowel are isolated with ster-
ile moist swabs. The intestinal content is milked
backwards into the stomach from where it is aspirat-
ed and a bacteriology swab is sent for culture and
sensitivity. Alternatively, proximal bowel contents are
milked into the bulbous blind end if it is to be resect-
ed. An atraumatic bowel clamp is then placed across
the bowel a few centimetres proximal to the elected
site for transection.
If total bowel length is deemed of adequate length
(>80 cm + ileocaecal valve) the bulbous hypertro-
phied proximal bowel is resected (5–15 cm) alongside
the mesenteric bowel border in order to preserve
maximal mesentery for later use, until normal diam-
eter bowel has been reached. The bowel should then
be divided at right angles leaving an opening of ap-
proximately 0.5–1.5 cm in width. The blood supply
should be adequate to ensure a safe anastomosis.
This is followed by very limited distal small bowel re-
section over a length of 2–3 cm. The resection line
should be slightly oblique towards the antimesenter-
ic border to ensure that the openings of the proximal
Figure 22.7, 22.8
The anastomosis is either end-to-end or end-to-back
(Denis-Browne method); 5/0 or 6/0 absorbable sutu-
res stitches are used. The mesenteric border of the di-
vided ends is united with a stay suture and a match-
ing stitch is placed at corresponding points of the
22
Heinz Rode, Alastair J.W. Millar
and distal bowels are of approximately equal size to
facilitate easy axial or end-to-back (Denis-Browne)
single-layer anastomosis. However, the discrepancy
in luminal width of the proximal and distal bowel
may vary from 2–5 cm depending on the distance
from the stomach.
With type III(b) or high jejunal atresia the proxi-
mal bowel should be derotated and resection of the
bulbous portion may be extended into the third or
second part of the duodenum without jeopardizing
the ampulla of Vater. The distal “apple peel” compo-
nent of Type III(b) atresia may require release of re-
stricting bands along the free edge of the distally
coiled and narrow mesentery to avoid kinking and
interference with the blood supply. The large mesen-
teric defect is usually left open but with proximal
bowel resection the residual mesentery can be used
to obliterate the defect. Furthermore, to prevent
kinking of the marginal artery after completion of
the anastomosis, the bowel needs to be replaced very
carefully into the peritoneal cavity in a position of
non-rotation.
anti-mesenteric borders of the divided ends. The “an-
terior” edges of the bowel are then united with inter-
rupted through-and-through extramucosal stitches,
which are tied on the serosal surface.
Chapter 22 Jejuno-ileal Atresia
219

Figure 22.6

Figure 22.7 Figure 22.8


220
Figure 22.9
After completion of one-half of the anastomosis the
bowel is rotated through 180° and the “posterior”
anastomosis completed. Alternatively the posterior
edge of the bowel is anastomosed with the stitches
tied on the mucosal surface followed by anastomosis
of the “anterior” edges with interrupted stitches tied
on the serosal surface. The suture lines are inspected
for anastomotic integrity or tested with saline injec-
tion on completion.
Although isolated type I atresia is best dealt with
by primary resection and anastomosis, multiple dia-
phragms have been successfully perforated with
transluminal bougies being passed along the entire
length of the affected small bowel.
Figure 22.10
The defect in the mesentery is repaired by approxi-
mating or overlapping the divided edges with inter-
rupted sutures taking great care not to incorporate
blood vessels or kinking the anastomosis. Closure of
the large mesenteric defect can be facilitated by using
the preserved mesentery of the resected proximal
bowel.
22
Heinz Rode, Alastair J.W. Millar
Multiple type IV atresias, present in 18% of in-
fants, are often localized necessitating en-bloc resec-
tion and a single anastomosis, rather than multiple
anastomosis. It is important, however, to maintain
maximum bowel length to avoid the short bowel syn-
drome.
Similar techniques are used for intestinal stenosis
and type I atresias. Procedures such as simple trans-
verse enteroplasties, excision of membranes, bypass-
ing techniques or side-to-side anastomosis are no
longer utilized. They fail to remove the abnormal
dysfunctional segments of intestine, thus increasing
the risk of the blind loop syndrome.
쐽 Wound Closure. The peritoneal cavity is thorough-
ly irrigated with warm saline to remove all macro-
scopic debris and the bowel then returned to the ab-
dominal cavity. Care is taken not to kink the anas-
tomosis. The abdomen is closed by approximating en
mass all the layers of the abdominal wall, excluding
Scarpa’s fascia, with a single continuous 4/0 monofil-
ament absorbable suture, followed by subcutaneous
and subcuticular absorbable stitches. No drains or
trans-anastomotic tubes are used.
Chapter 22 Jejuno-ileal Atresia
221

Figure 22.9

Figure 22.10

222
Figure 22.11, 22.12
Alterative surgical techniques may be required if the
ischaemic insult has resulted in an atresia with mark-
edly reduced intestinal length, where large resections
of abnormal or multiple atretic segments are re-
quired or if the measured residual small intestinal
length is <80 cm.
Indications for tapering are:
쐽 As part of bowel length preservation where the
proximal atretric segment is grossly dilated and
hypertrophied over an extended distance – typical
in type III(b) atresia and high jejunal atresia
쐽 To equalize disparity in anastomotic lumen size
쐽 For the correction of a failed inversion plication
procedure
쐽 To improve function in a persistently dilated non-
functioning mega-duodenum following surgery
for upper jejunal atresia
22
Heinz Rode, Alastair J.W. Millar
쐽 Tapering duodeno-jejunoplasty. This method is
primarily indicated to conserve bowel length (high
jejunal atresia, type III(b) atresia) and to reduce dis-
parity in anastomotic diameter size. The atretic jeju-
noduodenum is derotated and the antimesenteric
segment of the dilated proximal segment is resected
over a 22–24F catheter. The resection may extend
cephalad to the second part of the duodenum. An in-
testinal auto-stapling device may be used to facilitate
the resection and anastomosis. The longitudinal
anastomotic line is reinforced with an absorbable 5/0
or 6/0 Lambert suture. Tapering can safely be done
over a length of 20–35 cm. The tapered bowel is then
primarily anastomosed to the distal bowel (with or
without equal bowel diameters) and left in a depen-
dant position as for corrected malrotation with the
caecum in the left hypochondrium.
Chapter 22 Jejuno-ileal Atresia
223

Figure 22.11

Figure 22.12

224
Figure 22.13, 22.14
쐽 Plication and Folding. The basic derotation and
back resection methods are used as previously de-
scribed for tapering procedures. The plication meth-
od has the advantage of reducing the risk of leaking
from the antimesenteric suture line, conserves mu-
cosal surface area and may even facilitate return of
bowel peristalsis. More than half of the antimesen-
teric bowel circumference may be infolded into the
lumen over an extended length without causing an
22
Heinz Rode, Alastair J.W. Millar
obstruction with care being taken not to narrow the
lumen excessively. The “keel” must be trimmed and
closed with interrupted sutures. The main drawback
of this method is unravelling of the suture line with-
in a few months, necessitating revision. The bowel is
left in a position of derotation with the duodenojej-
unum dependant, the mesentery broad based and the
caecum in the left hypochondrium.
Chapter 22 Jejuno-ileal Atresia
225

Figure 22.13

Figure 22.14

226
Figure 22.15–22.17
쐽 Antimesenteric Seromuscular Stripping and In-
version Plication. This technique prevents unravell-
ing of the plication method and preserves maximal
mucosal surface for absorption. A single or two anti-
mesenteric converging seromuscular strips ±2 mm
in width are resected. This is easily performed by sta-
bilizing the proposed line of resection with straight
non-toothed forceps. A seromuscular strip is then
22
Heinz Rode, Alastair J.W. Millar
excised taking care not to damage the mucosa. The
two denuded mucosal strips are then approximated
with a running monofilament suture. The keel of the
inverted bowel should be trimmed and bowel edges
approximated with interrupted sutures prior to
anastomosis to the distal bowel. Proximal and distal
luminal size can be approximated to facilitate the
anastomosis.
Chapter 22 Jejuno-ileal Atresia
227

Figure 22.15

Figure 22.16 Figure 22.17


228
CONCLUSION
The overall survival amongst newborn babies with
intestinal atresia has increased from a dismal 10% in
1952 to 90% at present. This came about primarily
from a change in the surgical procedure from pri-
mary anastomosis without resection to liberal resec-
tion of the blind proximal and distal ends followed by
end-to-end anastomosis.
Understanding the pathogenesis of atresia and
adapting surgical procedures to minimize loss and
conserve bowel length ensured that most infants will
have sufficient bowel length for normal alimentary
tract function and overall growth and development.
Despite improvement in surgery, anaesthesia and
peri-intra- and post-operative care, type III(b) atre-
sia still carries a mortality of 19% predominantly due
to gangrene of the proximal end of the distal segment
(7%), anastomotic leak (15%) and stricture formation
(15%). The prognosis of intestinal atresia is further
determined by genetic factors, prematurity (30%),
delay in presentation, associated diseases, i.e., cystic
fibrosis, malrotation (45%), exomphalos, gastroschi-
sis and Hirschsprung’s disease, together with other
gastro-intestinal atresias, infarction of the proximal
atresia with peritonitis, sepsis, pneumonia and the
complications of prolonged parenteral nutrition.
The incidence of post-surgical complications such
as anastomotic leaks, stricture formation, transient
SELECTED BIBLIOGRAPHY
Kling K, Applebaum H, Dunn J, Buchmiller T, Atkinson J
(2000) A novel technique for correction of intestinal atresia
at the ligament of Treitz. J Pediatr Surg 35 : 353–356
Louw JH, Barnard CM (1955) Congenital intestinal atresia: ob-
servations on its origin. Lancet 2 : 1065–1067
Malcynski J T, Shorter N A, Mooney D P (1994) The proximal
mesenteric flap: a method for closing large mesenteric de-
fects in jejunal atresia. J Pediatr Surg 29 : 1607–1608
22
Heinz Rode, Alastair J.W. Millar
intestinal dysfunction, blind loop syndrome and the
short bowel syndrome can be minimized by careful
attention to the presenting anatomical detail, meticu-
lous surgical technique and maximal bowel length
conservation methods. Because of the high incidence
of unravelling, the plication technique is rarely used.
The short bowel syndrome is a major factor influ-
encing outcome. It may be due to predisposing fac-
tors such as extensive intra-uterine bowel loss, oper-
ative factors, i.e., over-zealous bowel resection and is-
chaemic injury to the bowel or post-operative com-
plications. Under ideal circumstances a survival rate
of 46–70% can be expected in most infants with less
than 25 cm jejuno-ielum.
Several surgical procedures have been identified
to improve the outcome of the short bowel syndrome
including reversal of segments of bowel, interposi-
tion of colonic segments and methods to increase
mucosal surface area for absorption purposes. Most
are of experimental value only except for bowel-
lengthening procedures. The latter should not be
performed initially until conservative methods to
stimulate and allow maximum bowel adaptation to
occur, have failed. Full bowel adaptation may require
6–18 months to become achieved.
Rode H, Millar AJW (2003) Intestinal atresia and stenosis: In:
Puri P (ed) Newborn surgery. Arnold, London, pp 445–456
Weber TR, Wane DW, Grosfeld JL (1982) Tapering enteroplasty
in infants with bowel atresia and short bowel syndrome.
Arch Surg 117 : 684–688