ABSTRACT
Jejuno-ileal atresia, defined as a congenital defect in continuity
of the bowel, is a common cause of intestinal obstruction in
the newborn.13 The incidence of jejuno-ileal atresia varies
from 1 in 330 and 1 in 400 live births,4 to between 1 in 1500
and 1 in 3000 live births.5 Jejuno-ileal occlusions occur more
frequently than duodenal or colonic.1,6 With improved
neonatal and perioperative care, safe anesthesia, refined
surgical techniques and management of short bowel syn-
drome, a survival rate of greater than 90% can be expected
in well-resourced centers. At the Red Cross War Memorial
Children’s Hospital in Cape Town during the 51 years 1959 2009, 333 jejuno-ileal atresias, 251 (75%) jejunum, and 82
(25%) ileum were seen (Table 52.1). Down syndrome is
most uncommon in babies with jejuno-ileal atresia (only
one baby in the Red Cross Hospital series) compared with
duodenal atresias. The first successful surgical repair of an
intestinal atresia was in 1911.7 The mortality rate remained
high over the next four decades and it was only in the mid
1950s that an improved understanding of the pathogenesis
and pathology of the condition led to innovative surgical
techniques which resulted in greatly improved surgical
In 1889, Bland Sutton postulated that atresia occurred at the
site of ‘obliterative embryological events’ and he quoted
atrophy of the vitelline duct.8 In 1900, Tandler,9 supported by
embryonal studies, suggested that intestinal atresia was
related to a lack of recanalization of the solid stage of the
intestine, while others have questioned these theories.1012
In 1952, Louw published the results of an investigation of
79 patients treated at Great Ormond Street, London, and
suggested that jejuno-ileal atresia was probably due to a
vascular accident rather than the result of inadequate
recanalization.5 At his instigation, Barnard perfected the
experimental model in pregnant mongrel bitches. Mesenteric
vascular insults, such as volvulus, intussusception, and
interference with the blood supply to a segment of bowel
were created in the dog fetus.13 This not only confirmed the
hypothesis but led to a change in the surgical procedure for
correcting atresias and stenosis of the jejunum and ileum
with a marked improvement in outcome.1416 Subsequently,
these experimental findings were confirmed by others in
several different animal models and in clinical practice.1721
Evidence of bowel infarction was present in 42% of 449 cases
of jejuno-ileal atresia in a collected series which further
supported the vascular hypothesis.22 Furthermore, the loca-
lized nature of the vascular accident occurring late in fetal life
would explain the low incidence (less than 10%) of coexisting
abnormalities of extra-abdominal organs.
