Intestinal Atresia and Stenosis
INTESTINAL ATRESIA
AND STENOSIS
Etiology
Goeller is credited with the first description of an ileal atresia in
1684. In 1889, Bland-Sutton proposed a classification of the
types of atresia and suggested that they occurred at the site of
obliterative embryonic events. In 1900, Tandler proposed the
theory that atresia was caused by a lack of recanalization of the
solid cord stage of the bowel Although this theory seemed valid
for atresia of the duodenum, most jejunoileal atresias could not
be explained by epithelial plugging. In 1912, Spriggs suggested
that mechanical accidents, including vascular occlusions, might
be responsible for intestinal atresia. The classic experiments
reported by Louw and Barnard in 1955 confirmed the role of late
prepartum mesenteric vascular accidents as the cause of most
intestinal atresias in puppies. These findings were confirmed by
several subsequent investigators. Clinical instances of intestinal
atresia as a result of intrauterine vascular insults, such as
volvulus, internal hernia, intussusception, or constriction of the
bowel in a tight gastroschisis defect or omphalocele, supported
these experimental studies. In a large series of atresias, 40% to
50% of patients had evidence of either intrauterine bowel
necrosis or peritonitis.
Incidence
The incidence of intestinal atresia has been reported to be 1 in
4000 to 5000 live births. Boys and girls are affected equally.
Although the mean birth weight in most reports is about 2.7 kg,
at least 33% of patients with jejunal atresia, 25% with ileal
atresias, and 50% of patients with multiple atresias are of low
birth weight. In contrast to infants with duodenal atresia, Down
syndrome is uncommon in infants with jejunoileal atresia.
Similarly, there is a much lower incidence of associated
congenital anomalies. There is a familial pattern of multiple
atresias affecting the stomach, duodenum, small bowel, and
colon occurring in individuals of French-Canadian background
that probably represents a rare autosomal recessive gene
inheritance. Other reports of familial atresias involve only the
jejunum.
Clinical Presentation
Neonatal intestinal obstruction often manifests with many
cardinal signs, including maternal polyhydramnios,
bilious vomiting, abdominal distention, and failure to pass
normal amounts of meconium in the first 24 to 48 hours of life.
Although none of these signs are pathognomonic of a specific
obstruction, all are consistent with an obstructive phenomenon
and indicate the performance of diagnostic studies.
Polyhydramnios refers to the presence of excess amniotic fluid
in the amniotic sac (>2000 mL). Of amniotic fluid, 25% to 40%
is swallowed by the fetus (usually in the fourth or fifth fetal
month) and is reabsorbed in the first 25 to 35 cm of jejunum.
Jejunal atresia is associated with maternal polyhydramnios in
24% of cases. Although there are other fetal causes of polyhy-
dramnios, any pregnant woman with abnormalities of amniotic
fluid should have a prenatal ultrasound examination. Presently,
most pregnant women have routine ultrasound screening. The
prenatal ultrasound can identify small bowel obstruction
associated with atresia, volvulus, and meconium peritonitis. The
lesions can be anticipated and an organized management plan
developed for delivery and treatment of the infant.
Bilious vomiting is another cardinal sign of intestinal
obstruction and is always pathologic. The presence of bile in a
gastric aspirate at birth should be investigated carefully. The
newborn's stomach usually contains less than 15 mL of clear
gastric juice at birth. Greater than 20 to 25 mL of clear gastric
juice or any bile may signify the presence of intestinal
obstruction. Bilious vomiting also may be seen in instances of
neonatal sepsis with adynamic ileus. When mechanical
obstruction is present, bile indicates that the level of obstruction
is distal to the ampulla of Vater. Bilious vomiting occurs in 85%
of infants with jejunal atresia and a lesser number of infants with
ileal atresia.
Jaundice occurs in more than 30% of infants with jejunal
atresia and 20% with ileal atresia and usually is associated with
elevation of the unconjugated fraction of bilirubin. Abdominal
distention is a sign of a more distal intestinal obstruction. The
normal contour of the newborn abdomen is round, in contrast to
the usual scaphoid appearance of the adult. Physical findings
associated with distention include visible veins from attenuation
of the abdominal wall, visible loops of intestine (intestinal
patterning) with or without noticeable peristalsis, and
occasionally respiratory distress caused by elevation of the
diaphragm.
When obstruction is suspected, recumbent and erect
abdominal radiographs must be obtained to evaluate the
491
492 CHAPTER 49 Intestinal Atresia and Stenosis
Distal small intestine or colon atresia Meconium ileus
Hirschsprung's disease
Meconium plug syndrome
Small left colon syndrome
Infantile neuronal dysplasia
Infant of a diabetic mother
Maternal medications given during
labor/delivery (e.g., magnesium sulfate)
Sepsis
Hypothyroidism
nature of the distention. Some surgeons use a left lateral
decubitus view of the abdomen, particularly in sick, weak
infants. Distention may be a result of free air from a per-
forated viscus, fluid (hemoperitoneum from birth injury to
liver, chyloperitoneum), or distended bowel from intestinal
obstruction or adynamic ileus. Although distention usually
develops 12 to 24 hours after birth, abdominal distention
noted immediately at birth suggests the presence of giant
cystic meconium peritonitis.
An additional sign of alimentary tract obstruction is failure
to pass meconium spontaneously in the first 24 to 48 hours of
life. Normal meconium is composed of
amniotic fluid and debris (squames, lanugo hairs), succus
entericus, and intestinal mucus. Meconium is dark green or
black and sticky, and 250 g may be passed rectally. Failure to
pass this material the first day of life is often pathologic
(Table 49-1). In addition to infants with distal small bowel
obstruction, patients with imperforate anus, Hirschsprung's
disease, meconium plug syndrome, small left colon
syndrome, and colonic neuronal dysplasia may present with
failure to pass meconium. Other causes include sepsis with
adynamic ileus, hypothyroidism, and narcotic addiction in the
mother.
Diagnosis
The diagnosis of jejunoileal atresia usually is confirmed by
abdominal x-ray. High jejunal atresia may present with a few
air-fluid levels and no further gas beyond that point (Fig. 49-
1). The more distal the atresia, the more apparent the clinical
abdominal distention and the greater the number of distended
intestinal loops and air-fluid levels observed. The atretic loop
may be much larger than the other loops of bowel.
Occasionally, areas of calcification are seen on plain
abdominal radiographs and signify the presence of meconium
peritonitis, a sign of intrauterine bowel perforation (12% of
patients). The sterile fetal meconium causes a local
inflammatory reaction, which includes saponification
eventually becoming calcified. In addition, instances of
intraluminal calcification ("mummification") may be
observed, indicating an antenatal volvulus. In patients with
giant cystic meconium peritonitis, plain abdominal
radiographs may show an excessively large air-fluid level in a
meconium pseudocyst. This type of occurrence is related to a
late intrauterine perforation, resulting in an encapsulated mass
of perforated bowel and meconium. Because haustral
markings rarely are shown on
the abdominal radiograph of a neonate, a contrast enema study must
be performed in each case of distal intestinal obstruction. The first
enema a newborn should receive is a contrast x-ray enema
administered through a soft catheter. Preparation of the colon for
this study is unnecessary. The barium or soluble contrast enema
study can be used to differentiate small intestine from colonic
distention, to determine if the colon is used or unused
("microcolon"), to identify the level of obstruction (e.g., small
bowel or colon), and to evaluate the position of the cecum in regard
to possible anomalies of intestinal rotation and fixation (Fig. 49-2).
Most infants with jejunoileal atresia have a microcolon (unused) on
contrast enema study, limiting the obstruction to the small bowel.
Microcolon is the result of a lack of distention of the colon because
succus entericus does not pass beyond the area of obstruction in the
fetal bowel. Malrotation may be observed in 10% of patients with
intestinal atresia. There is usually no indication to perform an upper
gastrointestinal contrast study in patients with radiographic
evidence of complete obstruction; however, in patients with
intestinal stenosis who have an incomplete obstruction, this study
may be useful.
Differential Diagnosis
Neonates with intestinal obstruction from other causes may present
with a clinical picture similar to infants with jejunoileal atresia.
These cases include instances of
malrotation with or without volvulus, bowel duplication, internal
hernia, adynamic ileus with sepsis, meconium ileus, colonic atresia,
and total colonic aganglionosis. The two most common causes of
distal small bowel obstruction in the newborn are ileal atresia and
meconium ileus. The contrast barium enema often yields valuable
information that rules out certain causes of obstruction, particularly
colon atresia and small bowel aganglionosis. Jejunoileal atresia may
coexist with malrotation (10%), meconium peritonitis (12 %),
meconium ileus (9%), and rarely aganglionosis so that a precise
diagnosis beyond obstruction is not always possible. Certain
abdominal radiographic findings may help distinguish between
instances of ileal atresia and meconium ileus. Infants with
uncomplicated meconium ileus often show significant dilation of
similar-sized bowel loops with few if any air-fluid levels. A ground-
glass appearance in the right lower quadrant (Neuhauser's or "soap
bubble" sign) may be observed and represents viscid meconium
mixed with air. Although this is not pathognomonic of meconium
ileus (e.g., seen in some cases of colon atresia), it is a frequent
finding. An enema study commonly shows an unused microcolon
similar to cases of ileal atresia; however, reflux of contrast material
into the distal ileum may identify the small obstructive concretions
of meconium characteristic of this hereditary disorder.
Careful evaluation may avoid unnecessary surgery because at
least 50% of these patients with uncomplicated meconium ileus
may respond to nonoperative therapy using a hypertonic contrast
washout with meglumine diatrizoate (Gastrografin) or other soluble
contrast material. Meconium ileus may be complicated by
jejunoileal atresia, volvulus, perforation, or giant cystic meconium
peritonitis and require operative intervention; the appropriate
diagnosis is confirmed at the time of laparotomy. Because 10% of
cases of jejunoileal atresia
 PART VII Stomach, Duodenum, and Small Intestine 493

FIGURE 49-1 A, Abdominal radiograph in a neonate with bilious

vomiting shows a few loops of dilated intestine with air-fluid levels. B, At
laparotomy, a type I (mucosal) jejunal atresia was observed.

A B

.
FIGURE 49-2 A, Abdominal radiograph in a newborn infant with bilious vomiting and abdominal distention shows multiple dilated
bowel loops with air-fluid levels. B, Barium enema study shows a microcolon, indicating the obstruction is in the distal small bowel.
The infant had ileal atresia.
 494 CHAPTER 49 Intestinal Atresia and Stenosis

accomplished. The umbilical vein should be avoided as a site

occur in infants with cystic fibrosis, a sweat cWoride
determination should be obtained in each instance of atresia
before hospital discharge.
Pathologic Findings and Classification
Atresias occur slightly more often in the jejunum than in the
ileum. Most instances are single (>90%); however, in 10% to
15% of these patients, multiple atresias are observed. The
pathologic findings are classified by type (Fig. 49-3). Type I
atresias have a mucosal web ot diaphragm (possibly from
epithelial plugging) with an intact bowel wall and mesentery.
Type il atresias have a fibrous cord between two blind ends of
the atretic bowel but have an intact mesentery. In type ilIa
atresias, there is a complete separation of the blind ends of the
atretic bowel by a V-shaped mesenteric gap defect. Type TIIb
atresias have an "apple peel" or "Christmas tree" deformity in
which fetal occlusion of the proximal superior mesenteric
artery occurred with the distal bowel receiving a retrograde
blood supply from the ileocolic or right colic artery. Finally,
infants with type IV atresias have multiple atresias that often
are characterized by a "string-of-sausage" or "string-of-beads"
appearance.
Treatment
During the initial evaluation, an orogastric tube is inserted into
the stomach, and fluid and electrolyte repletion is
for fluid administration. Fluid resuscitation is accomplished
using 10% dextrose in lactated Ringer's solution given at a rate
of 10 mL/kg over 30 minutes while laboratory data are being
acquired. Additional fluids may be required to maintain the
infant's mean arterial pressure greater than 50 mm Hg and to
establish urine flow. A solution of 10% dextrose in 0.25%
normal saline is employed for maintenance with potassium
cWoride added as needed. In most cases, resuscitation and
stabilization can be accomplished within a short time, and
exploratory laparotomy can be carried out in an expeditious
fashion. Perioperative antibiotics, usually ampicillin and genta-
micin, are initiated 30 minutes before the procedure.
The infant is taken to the operating room in a thermally neutral
environment in an isolette. The operation is done with the
patient under well-monitored and controlled general
endotracheal anesthesia supplemented with infrared heating
lamps and other heating measures. A right upper quadrant
transverse supraumbilical incision is ideal in most cases. The
abdomen is explored, and the level of obstruction and the type
of atresia are determined. In patients with high jejunal atresia,
the obstructed proximal dilated atretic segment is often atonic
and should be resected back to the ligament of Treitz, provided
that there is near-normal bowel length, and an end-to-oblique
anastomosis is performed (Fig. 49-4). The distal bowel should
be evaluated for additional atresias or stenosis by passage of a
soft red rubber catheter or by injection of saline solution. In
patients with short-bowel syndrome caused by volvulus, the
proximal atretic segment is the only remaining proximal
intestine and must be preserved. This can be accomplished by
performing an antimesenteric tapering

IV

FIGURE 49-3 Classification of intestinal atresia. Type 1mucosal atresia with

intact muscularis. Type ll-the atretic ends are separated by a fibrous band.
Type IDa-atretic ends are separated by a V-shaped gap defect. Type IIIb-
apple peel deformity of the distal atretic segment with retrograde blood
supply from the ileocolic or right colic artery. Type IV-multiple atresias
(string-of-sausage effect).
FIGURE 49-4 In patients with relatively normal bowel lengths, the
dilated proximal atresia is resected, and an end-to-oblique
anastomosis is performed.
 PART VII . Stomach, Duodenum, and Small Intestine 495
FIGURE 49-5 . In infants with short bowel length, the proximal bowel
may be preserved by performing a tapering enteroplasty by resection of
the antimesenteric border of the bowel using a stapling device.
enteroplasty (Fig. 49-5). An alternative procedure is intestinal
imbrication, which also effectively reduces the caliber of
distended bowel and allows effective intestinal transit to resume.
For the rare infant with very short bowel syndrome and a dilated
proximal jejunum, the bowel lengthening procedure described by
Bianchi occasionally has been used. Disparity in the size of the
dilated proximal bowel and the smaller distal end may be allevi-
ated by an end-to-oblique anastomosis with interrupted 5-0
nonabsorbable sutures.
Most infants with ileal atresia usually have type IIIa
pathology, and when reasonably normal bowel length is
preserved, operative management includes resection of the
dilated atretic loop and an end-to-oblique anastomosis. At the
time of the procedure, the distal bowel should be checked for
additional areas of atresia or stenosis. In patients with short-
bowel syndrome, a tapering ileostomy is performed to preserve
an intestinal length compatible with survival. In the presence of
severe peritonitis, or when bowel viability is in question, a
temporary enterostomy may be required. Infants with ileal
atresia who have an intact ileocecal valve have better absorptive
capability and survival than infants in whom the ileocecal valve
is resected, so if possible, the ileocecal valve should be
preserved. This is probably related to prevention of colonization
with colonic flora or to improved fat and vitamin B12 absorption
and an improved enterohepatic circulation of bile from the distal
ileum rather than the valve itself (see Chapter 53). In instances of
meconium ileus complicated by atresia, bowel resection and
anastomosis or temporary enterostomy is required. Anastomotic
function may take 7 to 10 days, so the stomach is kept
decompressed by an orogastric tube.
Nutritional support is accomplished with postoperative
parenteral nutrition until bowel function is restored. When
stooling occurs and the gastric volume decreases
and clears, the orogastric tube can be removed and feedings
instituted. In infants with a temporary enterostomy, feedings
can be initiated when stomal function is observed. A low-
osmolar, small-curd, easily absorbed formula, such as
Pregestimil, or a soybean-based formula can be used. F
eedings may be initiated as dilute formulas, then increased in
density and volume as tolerated. Most infant formulas contain
adequate vitamin and iron supplements. In some formulas that
do not contain supplements, iron must be administered on a
daily basis for the first year of life: 0.6 mL of ferrous sulfate
daily. In instances of shortbowel syndrome, special formulas
may be required in addition to long-term total parenteral
nutrition to ensure adequate caloric intake for growth. After
correction of the obstruction in infants with meconium ileus
complicated by atresia, careful family counseling and parental
instruction regarding diet, enzyme replacement, and pul-
monary toilet using percussion and postural drainage must be
done. The parents also must be made aware that these
children are at risk of developing meconium ileus equivalent
even after several years.
Another less common cause of small bowel obstruction in
newborns is intrinsic small bowel stenosis. This condition
probably is related to an ischemic injury. These patients
present with abdominal distention and bilious vomiting but
have a normal contrast enema study and pass meconium
within the first 24 hours of life (Fig. 49-6). The indication for
operation is continued evidence of partial intestinal
obstruction and failure to thrive.
Morbidity and Mortality
The most common cause of death in patients with jejunoileal
atresia is infection related to pneumonia, peritonitis, or sepsis.
The most significant postoperative complications include
functional intestinal obstruction at the site of the anastomosis
and anastomotic leak. Other factors that affect morbidity and
mortality include respiratory distress, prematurity, short-
bowel syndrome, and postoperative volvulus with infarction.
In the past, the survival of patients with jejunal atresia was
lower than patients with ileal atresia. An increased mortality
also was seen in patients with multiple atresias (57%); apple
peel atresias (71 %); and when atresia was associated with
meconium ileus, meconium peritonitis, and gastroschisis. In
more recent years, because of improved neonatal intensive
care, pediatric anesthesia, total parenteral nutrition, and a
better understanding of the overall management of these
patients, the survival rate has increased dramatically to 85%
to 90% for infants with jejunal and ileal atresia treated at
major pediatric surgical.centers.
COLON ATRESIA AND STENOSIS
Colon atresia and stenosis as an isolated entity (unassociated
with imperforate anus or cloacal exstrophy) is a relatively
infrequent cause of neonatal intestinal obstruction. Bininger
described the first case in 1673. In 1922, Gaub reported
survival of an infant with atresia of the colon after performing
a colostomy. The first successful anastomosis for an infant
with colon atresia was reported by Potts in 1947.
 496 CHAPTER 49 Intestinal Atresia and Stenosis
FIGURE 49-& . Abdominal radiograph in an infant with abdominal distention
and bilious vomiting. A meconium-filled mass fills the right lower quadrant.
Incidence
The incidence of colon atresia in reported series ranges from 1 in
15,000 to 20,000 live births. The latter figure is probably more
correct because most major children's surgical centers see
approximately one case per year. In regard to other alimentary
tract atresias, only pyloric atresia occurs with less regularity.
Some reports suggest that associated anomalies are common. If
cases of colon atresia in association with cloacal exstrophy with
vesicointestinal fissure and abdominal wall defects (gastroschisis
and omphalocele) are excluded, however, the incidence of
associated anomalies in infants with colon atresia as an isolated
event is low. Colon atresia has been noted in infants with
Hirschsprung's disease, jejunal atresia, and some skeletal
anom.alies including syndactyly and polydactyly on rare
occasions.
Etiology
Similar to instances of jejunoileal atresia and stenosis, most
cases of colonic atresia and stenosis are the result of in utero
vascular compromise of the mesentery to the large bowel. The
areas that are most affected are the transverse and sigmoid colon,
which have a floppy mesentery and may be prone to volvulus in
utero. Atresia also may occur in the right colon.
Classification
The classification of colon atresia is the same as the
classification given atresias of the small bowel, but apple peel
deformity does not occur in the colon.
Clinical Presentation and Diagnosis
Most infants with isolated colon atresia are usually term and rarely
have associated anomalies. Failure to pass meconium in the first
24 hours of life, abdominal distention, and bilious vomiting are
the usual clinical manifestations. Erect and recumbent abdominal
radiographs show dilated intestine with air-fluid levels and a
particularly dilated segment at the point of obstruction. The atretic
loop may have a soap bubble appearance from the admixture of
meconium and air. Some cases are complicated by perforation and
have massive pneumoperitoneum. The diagnosis is confirmed by a
contrast enema study that shows a blind distal end of a micro-
colon. Most instances of colon atresia are type IDa with a V-
shaped gap between the atretic ends (see Fig. 49-6). The sigmoid
colon is the second most common site of colon atresia. Rare type I
mucosal atresia and type n atresia are more common in the distal
colon.
Treatment
The initial resuscitation and management of infants with colon
atresia is essentially the same as infants with jejunoileal atresia. At
the time of laparotomy, the atretic segment is delivered and
inspected. The presence of proximal intestinal (jejunal) and other
colon atresias should be ruled out. We, as most, have treated all
infants with colon atresia with a preliminary colostomy and
subsequent closure with an end-tooblique ileocolic or colocolic
anastomosis at age 3 to 6 months. Some surgeons recommend
performing a primary anastomosis for atresia occurring in the
rightsided or transverse colon and a temporary colostomy for
instances of atresia affecting the sigmoid colon. Surgeons in favor
of primary anastomosis in the neonatal period for right-sided
atresias recommend resecting the entire atretic segment including
the ileocecal valve and performing an ileocolic anastomosis.
Complicated cases, such as colon atresia in association with
proximal jejunal atresia, are managed best by jejunojejunostomy
and distal colostomy. Initial colostomy also is recommended for
instances of colon atresia occurring in a patient with gastroschisis.
Results
The current survival for infants with isolated colon atresia is 90%
to 100%. Mortality usually is limited to the occasional infant with
associated anomalies or when there is a long delay in diagnosis
complicated by perforation and peritonitis.
SUGGESTED READINGS
Louw JR, Barnard CN: Congenital intestinal atresia: Observations on its
origin. Lancet 2:1065,1955.
This classic report describes the experiments that documented
intrauterine vascular compromise as the cause of most
intestinal atresias.
Newman K: Jejunoileal atresia. In Oldham KT, et al (eds): Surgery of
Infants and Children: Scientific Principles and Practice.
Philadelphia, Lippincott-Raven, 1997, P 1193.
 PART VII. Stomach, Duodenum, and Small Intestine 497

This chapter provides an excellent up-to-date review of intestinal atresia

and postoperative care considerations.
This report concerning a large group of infants with intestinal atresia,
Rescorla FJ, Grosfeld JL: Intestinal atresia and stenosis: including colon atresia, documents the currently improved survival
Analysis of survival in 120 cases. Surgery 98:668, 1985. data.