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- incidence of jejunoileal atresia and stenosis is one in
1000 live births.
Etiology :
-Tandler suggested that intestinal atresia was related
to a lack of recanalization of the solid stage of the
intestine, while others have questioned these theories
- Louw suggested that jejuno-ileal atresia was probably
due to a vascular accident rather than the result of
inadequate recanalization
- A localized intrauterine vascular accident can
cause ischemic necrosis, liquefaction of tissues and
subsequent resorption of the affected devitalized

Stenosis (11%) is characterized by a short
localized narrowing of the bowel without
discontinuity or a mesenteric defect. The bowel is of
normal length.

Atresia type I (23%) is represented by a

Septum, membrane or web formed by both mucosa
and submucosa while the muscularis and serosa
remain intact. The dilated proximal bowel remains in
continuity with the collapsed distal bowel, there is no
mesenteric defect and the bowel is of normal length.

Atresia type II (10%) has two blind-ending

atretic ends connected by a fibrous cord along the
edge of the mesentery. There is no mesenteric
defect and the bowel length is not foreshortened.

Atresia type III(a) (15%) is similar to type II

except that the fibrous connecting cord is absent and
there is a V-shaped mesenteric defect. The bowel
length may be foreshortened. Cystic fibrosis is
commonly associated with this variety.

Atresia type III(b) (19%) (Apple peel or Christmas

tree) consists of a proximal jejunal atresia often with
associated malrotation, absence of most of the superior
mesenteric 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. Occasionally, additional
type I or type II atresias are found in the distal bowel.
There is always a significant reduction in intestinal
length. A familial incidence and atresias amongst
siblings and identical twins point to a more
complex genetic transmission with an overall recurrence
rate of 18%

Atresia type IV (22%) represents multiple

atresias or a combination of types IIII. Bowel length
is always reduced. A rare autosomal recessive pattern
of transmission has been documented and
pathological findings could support the concept that a
developmental process early on, could have affected
the whole bowel.

Clinical Manifestations

present as neonatal intestinal obstruction

Abdominal distension and bilious vomiting is
observed within 24 hours of birth. The degree of
abdominal distension is related directly to the site
of obstruction. Abdominal distension is more
pronounced with distal small-bowel obstruction.
Most infants fail to pass meconium. Although the
classic first stools passed by these patients are
small, gray and mucoid, normal meconium can
occasionally be passed
Jaundice is present occasionally


radiological examination of the abdomen and chest distended air-filled small intestinal loops proximal to
an obstruction in a gasless distal abdomen. In some
instances, the first abdominal radiograph can reveal a
completely opaque contrastless abdomen due to
fluid-filled obstructed bowel.
The more distal the obstruction the greater the
number of air fluid filled and distended loops of
The bowel proximal to the site of obstruction may
have the appearance of a large air-fluid filled loop.
a contrast enema is performed to exclude colonic
atresia, distinguish between small and large bowel
distension, determine whether the colon has the
typical microcolon appearance,



and intravenous hydration are essential. Dehydration
and acidbase and electrolyte imbalances should be
The aim is to establish intestinal continuity and preserve
as much normal bowel as possible. The proximal bowel
is dilated and a limited resection is usually needed. This
resection also facilitates an end-to-end or end-to-oblique
anastomosis. A single-layer, end-to-oblique anastomosis
is simple and efficacious. If proximal resection is not
possible, then tapering or plication of the dilated bowel
is indicated. The distal bowel should be evaluated for
additional atresias or stenosis by an intraluminal
injection of 0.9% saline. If multiple atresias are present,
then many anastomoses may be required