Jejunoileal Atresia and Stenosis

Jason S. Frischer and Richard G. Azizkhan

History
Jejunoileal atresia and stenosis are the most common congenital anomalies of the small
intestine and are a major cause of intestinal obstruction in neonates.
Atresia (complete occlusion of the intestinal lumen) accounts for 95% of cases, whereas
stenosis (partial intraluminal occlusion) accounts for only 5%.
The first description of ileal atresia appeared in 1684 and is attributed to Goeller.1
Other early commentaries on this anomaly were recorded in the 1770s by Calder 2 and
Osiander.3
By the early 1800s, Voisin had performed an enterostomy for intestinal atresia and Meckel
had published a review of this condition and speculated on its etiology.
Decades later (1889), Bland-Sutton4 proposed a classification of the types of intestinal
atresia and postulated that these defects occurred at the sites of obliterative embryologic
events such as atrophy of the vitelline duct.
In 1894 Wanitschek performed the first, though unsuccessful, resection and anastomosis.3
In 1900Tandler5 theorized that intestinal atresia was secondary to a failure of recanalization
of the solid-cord stage of bowel development; this concept was reaffirmed a decade later by
Johnsons clinical observations.6
The first successful anastomosis for intestinal atresia was achieved by Fockens in 1911.7 A
year later, Spriggs8 suggested that causality for intestinal atresia could be attributed to
mechanical accidents including vascular occlusions.
This concept was later substantiated by the clinical observations of Davis and Poynter
(1922)9 and Webb andWangensteen (1931).2
In 1955 a landmark study conducted by Louw and Barnard10 documented that late
intrauterine mesenteric vascular accidents were responsible for most jejunoileal atresias.
Their findings laid the foundation for our contemporary understanding of the wide range of
intestinal defects seen in clinical practice.
As reported by Evans (1951),1 the survival of children with intestinal atresia in the first half
of the twentieth century
was dismal, with only 139 of 1498 cases having a successful outcome.
During the past 3 decades, a better understanding of etiologic factors and impaired
intestinal function, as well as
refinements in pediatric anesthesia, operative techniques, and preoperative and
postoperative care (especially in the
area of nutritional support), have led to a significant mprovement in survival.1113

Prevalence
Reports of the prevalence of jejunoileal atresia vary significantly across the globe, ranging
from as low as 1.3 to 2.25 cases per 10,000 live births in Spain, Latin America, and France to
as high as 2.9 cases per 10,000 live births in various regions of the United States.1216
Several authors1416 have reported a higher prevalence of small-bowel atresia in AfricanAmerican children and in twins, regardless of race.
Consistent with the latter finding, a population-based study conducted in the Netherlands
found a higher incidence of jejunal atresia in fraternal twins.17
A population-based study in Hawaii found a higher incidence of jejunoileal atresia in Far East
Asians compared with whites.16
Factors that increase the risk of developing small intestinal atresias include use of
pseudoephedrinen alone or in combination with acetaminophen and receiving ergotamine
tartrate and caffeine (Cafergot) for the management of migraine headaches during
pregnancy.1819
Nixon and Tawes20 reported a 2:1 ratio in the frequency of jejunoileal to duodenal atresias.
In contrast, a review of 387 patients with intestinal atresia and stenosis treated over a 32year period (1972 to 2004) at the Riley Childrens Hospital in Indianapolis revealed that 50%
of cases involved the jejunoileal region, 44%
were duodenal, and the remaining 6%were colonic.21
In a study of 619 patients representing the combined experience of members of the Surgical
Section of the American Academy of Pediatrics, de Lorimier and colleagues22 noted that boys
and girls were equally affected by intestinal atresia.
Although the mean birth weight of this cohort was 2.7 kg (range 0.9 to 4.8 kg), 33% of the
infants with jejunal atresia, 25% of those with ileal atresia, and more than 50% of those with
multiple atresias were infants with low birth weight. 22
Furthermore, infants with intestinal atresia detected on prenatal ultrasound (US) frequently
have low birth weight.23
Although trisomy 21 is seen in 30% of infants with duodenal atresia or stenosis, it is
uncommon in those with jejunoileal atresia.
More specifically, the previously cited studies conducted by Nixon and Tawes 20 and
deLorimier and colleagues22 noted that only 2 of 127 patients and 5 of 619 patients,
respectively, with jejunoileal atresia had trisomy 21.
In the study conducted at Riley Childrens Hospital only 1 of 194 infants with jejunoileal
atresia had this disorder