Ileal atresia

Dr Pir Abdul Ahad Aziz and Dr Jeremy Jones◉ et al.

Ileal atresia is a congenital abnormality where there is significant stenosis or

complete absence of a portion of the ileum. There is an increased incidence in
those with chromosomal abnormalities.

This article will focus on ileal atresia alone but bear in mind that some cases
correspond to jejunoileal atresia and show a mixed pattern, including the ones
discussed in the jejunal atresia article.

Pathology
Ileal atresia results from a vascular accident in utero that leads to decreased
intestinal perfusion and subsequent ischaemia a segment of bowel. This leads
to narrowing, or in the most severe cases, complete obliteration of the
intestinal lumen.

Associations
 cystic fibrosis: ~25% of cases 1

Radiographic features
Plain radiograph
In the postnatal period, an abdominal radiograph will show air in the dilated
loops of proximal bowel.

Fluoroscopy
Enema shows microcolon.

Antenatal ultrasound
An ileal atresia is often discovered prenatally at a routine prenatal ultrasound
scan or following the development of polyhydramnios. On ultrasound, there is
frequently a proximal dilated intestinal segment.

Treatment and prognosis

Surgical treatment depends on the severity of obstruction and length of the
damaged bowel but is usually curative

https://radiopaedia.org/articles/ileal-atresia
Overview
Approximately 95% of intestinal obstructions diagnosed in the first 2 weeks of life are due to
atresia and/or stenosis of small intestine. [1] Atresia is more common than stenosis and
represents complete luminal obstruction of a hollow viscus, whereas stenosis involves partial
occlusion. The incidence of atresia of the small bowel ranges from 1 case in 332 live
births [2] to 1 case in 5000 live births. [3] No specific racial or sex predilection is known.
Most ileal atresias are diagnosed in the first 24 hours of life (range of age presentation, 1-30
d) [4] and represent 50% of small intestinal atresias (ileal atresia is more common than jejunal
atresia. Although jejunal and ileal atresias uncommonly coexist and are multiple, the entire
small bowel must be examined at surgery.). [5]Prematurity of the neonate is related to the
severity of the subtype of atresia. [6](See Anatomy, below.) At birth, complete obstruction is
present with atresia, whereas various degrees of stenosis manifest with mild to severe
sequelae. About 15% of ileal atresias occur proximally, and 30% occur distally. [7]
In proximal atresias, the colon is normal or nearly normal in size, because adequate succus
entericus reaches the colon from small bowel distal to the obstruction. However, distal
atresias are usually associated with an unused colon or microcolon less than 1 cm in diameter.
When the ischemic event occurs later in gestation, the colon may be normal in caliber, even if
the obstruction is distal.
Small bowel atresia, meconium ileus, volvulus, intussusception, and idiopathic causes can be
complicated by perforation resulting in meconium peritonitis, which occurs in 5.2% of
cases. [4] In utero perforation of bowel may allow spill of meconium into the peritoneal space.
Fibrosis ensues, and peritoneal calcification may occur (see the image below). Calcifications
may be irregular, linear, round, or generalized in appearance. Intraluminal calcifications may
also occur in cases of severe obstruction.
 Cystic meconium peritonitis. Plain radiograph of
the abdomen shows multiple air-filled loops of bowel, some dilated, and a large, rounded
structure with calcified wall in the mid abdomen.
View Media Gallery

Anatomy

There are 4 main types of ileal atresia with subtypes. [8, 9] Martin and Zerella described the
survival rate for each subtype. [6]
 Type I is characterized by a thin diaphragm that occludes the lumen. The survival rate is
85%. [6]
 In type II, 2 blind ends are connected with a fibrous cord of atretic bowel. The survival
rate is 66%. [6]
 In type IIIA, 2 blind ends terminate with a V-shaped mesenteric defect; this is the most
common type.
 Type IIIB, apple-peel or Christmas-tree atresia, involves a large, V-shaped mesenteric
defect in which the blind-ended bowel distal to the atresia is wrapped around its blood
supply. That is, the proximal superior mesenteric artery is occluded, and the distal small
intestine spirals around its vascular supply of collaterals from the ileocolic artery. This
is associated with an absent distal superior mesenteric artery, shortening of the small
intestine distal to the atresia, and absent dorsal mesentery. Prematurity, malrotation, and
short gut occurs in most patients. [10] The survival rate is 57%
  Type IV is defined as multiple atresias. The survival rate is 29%. [6]

There are 4 main types of ileal

atresia with subtypes. Type I is characterized by a thin diaphragm that occludes the
lumen. In type II, 2 blind ends are connected with a fibrous cord of atretic bowel. In
type IIIa, 2 blind ends terminate with a V-shaped mesenteric defect; this is the most
common type. Type IIIb, apple-peel or Christmas-tree atresia, involves a large, V-
shaped mesenteric defect in which the blind-ended bowel distal to the atresia is wrapped
around its collateral blood supply. Type IV is defined as multiple atresias.
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Preferred examination

In the newborn with suspected intestinal obstruction, the preferred initial examination is plain
radiography of the abdomen. This examination allows for the differentiation of high
(proximal) and low (distal) obstruction, and the results dictate the next appropriate step in
management.
In infants, small bowel is impossible to distinguish from large bowel, but newborns are
unique in that they are swallowing air for the first time. If bowel obstruction is complete, no
air is present distal to the obstruction. Therefore, the abdominal radiograph is like an upper-
gastrointestinal (UGI) series with air as the contrast medium. The number of loops of dilated,
air-filled bowel differentiates high from low obstruction.
If only a few loops of dilated bowel are seen, a high obstruction is present. The plain
radiograph is often diagnostic, as with the double-bubble sign of duodenal atresia. [11] All
causes of high intestinal obstruction in the newborn are surgical; therefore, further imaging is
not usually indicated. If surgery is to be delayed or if the plain radiograph is normal, a UGI
series must be performed to exclude malrotation and/or midgut volvulus. This condition
requires immediate surgery and is the diagnosis most likely to produce a normal radiograph
in a neonate.
If the abdominal radiograph shows many dilated loops of bowel, a low obstruction is present.
The differential diagnosis includes incarcerated inguinal or umbilical hernia, ileal atresia,
meconium ileus, ileal duplication cyst, [12, 13] colonic atresia, functional immaturity of the
colon, Hirschsprung disease, and imperforate anus. Hernia and imperforate anus are
diagnosed on the basis of clinical examination. Otherwise, a contrast enema study is indicated
to differentiate surgical from medical causes of distal bowel obstruction in the newborn.

Limitation of technique

Plain radiographs in the newborn are useful in determining the level of obstruction, but
atresias are occasionally multiple, and the plain radiographs demonstrate only the most
proximal obstruction. In addition, dilated loops of bowel filled with fluid may go undetected
or may be mistaken for a mass (see the image below). Cross-table radiographs showing air-
fluid levels are often helpful in such instances. Also, radiographs may be normal in cases of
malrotation and intermittent midgut volvulus.
Ileal atresia. Upright radiograph of the abdomen demonstrates many dilated loops of bowel
and air-fluid levels.
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The UGI series may add no information to a diagnostic plain radiograph showing a
pathognomonic finding, such as the double bubble of duodenal atresia.
If the referring physician requests both a UGI series to exclude midgut volvulus and a
contrast enema study, one examination can interfere with the other. If there is an indication
for both, the UGI series should be performed first. Contrast should be administered through
an enteric tube; the enteric tube is then used to aspirate as much of the contrast agent as
possible after the duodenal jejunal junction is evaluated.
Contrast enema is useful in demonstrating microcolon, but if contrast agent is inadequately
refluxed into the distal small bowel, meconium ileus may be mistaken for ileal atresia; this
mistaken finding may lead to unnecessary surgery.
Ultrasonography can demonstrate dilated fluid-filled loops of bowel, but this modality is of
limited utility in the neonatal period, because it does not show the site of obstruction.
Ultrasonography may be helpful for specific limited purposes, such as differentiating a
dilated fluid-filled loop of bowel from a mass, diagnosing enteric duplication cyst, or helping
to differentiate meconium ileus from ileal atresia (see Ultrasound).
Computed tomography (CT) scanning and magnetic resonance imaging (MRI) have no role
in the diagnosis of ileal atresia.

Differential diagnosis and other problems to be considered

In addition to Hirschsprung disease, imperforate anus, incarcerated inguinal or umbilical

hernia, ileal duplication cyst, and functional immaturity of the colon discussed in Preferred
examination above, the differential diagnosis, includes meconium ileus, as well as meconium
plug syndrome and colonic atresia. Furthermore, polyhydramnios occurs in one fourth of
cases and is more common in proximal jejunal atresia than in distal ileal atresia. [14] However,
extragastrointestinal anomalies are rare and occur in less than 7% of patients. [4]

Special concerns

Use of barium or of undiluted, hypertonic water-soluble contrast agents in the performance of

a diagnostic enema study can cause untoward complications. Barium should be avoided in the
newborn for a number of reasons. The main reason is the potential for spilling contrast
material into the peritoneal cavity. Bowel adjacent to the atretic bowel is at risk of perforation
and can be necrotic. On occasion, initial plain images do not demonstrate the perforation.
Barium in the peritoneal cavity is not absorbed. Ensuing inflammatory response can lead to
formation of granulomata and adhesions. In addition, barium can become inspissated in the
colon, leading to obstruction. Patients at risk are those with Hirschsprung disease, cystic
fibrosis, ileus, and blind loops of bowel. Use of undiluted, high-osmolality contrast agents in
infants can also cause fluid shifts resulting in life-threatening serum electrolyte imbalances.
In addition, it is important to minimize radiation exposure in children. Important steps to
include are effective immobilization, limiting the field size to the area of interest, and use of
gonadal shielding whenever possible. During fluoroscopic procedures, examination time
should be kept to a minimum; pulsed fluoroscopy further decreases radiation exposure to the
patient.
Radiography
Plain radiographic findings in ileal atresia include a dilated stomach (if no nasogastric [NG]
suction was used), numerous loops of dilated bowel, multiple air-fluid levels proximal to the
point of obstruction, and absent gas distal to the obstruction (see the following images). A
dilated loop of bowel may be observed immediately proximal to the site of atresia; this is a
common finding with atresias in general. This loop may be filled with fluid and resemble a
mass. Ultrasonography may be helpful to show that this is fluid-filled bowel. [14]

Ileal atresia. Upright radiograph of the

abdomen demonstrates many dilated loops of bowel and air-fluid levels.
View Media Gallery
Meconium peritonitis can occur when an atresia or other obstruction leads to an in utero
bowel perforation. It is most commonly seen in the setting of jejunal or ileal atresia.
Calcifications around bowel and in the peritoneum are evident in meconium peritonitis (see
the image below). These calcifications can be focal, cystic or generalized. In the male patient,
they may extend into the scrotum by means of a patent processus vaginalis.

Cystic meconium peritonitis. Plain radiograph of the abdomen shows multiple air-filled loops
of bowel, some dilated, and a large, rounded structure with calcified wall in the mid
abdomen.
View Media Gallery
Contrast enema study is indicated when plain radiographs demonstrate a distal obstruction
(see the following images). In ileal atresia, the colon is diffusely small (< 1 cm; ie,
microcolon) (see the images below). Filling of the distal small bowel with contrast agent is
abruptly cut off, because material cannot reflux past the atresia into the dilated ileum
proximal to the obstruction. It is this abrupt truncation of the contrast column that allows
differentiation of ileal atresia, a surgical lesion, from meconium ileus, a medical lesion when
uncomplicated.
 Ileal atresia. Upright radiograph of the
abdomen demonstrates many dilated loops of bowel and air-fluid levels.
View Media Gallery

Ileal atresia in the same patient as in the

previous image. Contrast enema study demonstrates microcolon with abrupt cut-off of
contrast material filling of the ileum. This image shows no filling of dilated bowel proximal
to the obstruction and a lack of filling defects in the small bowel.
View Media Gallery

Ileal atresia. Contrast enema study

demonstrates a small, unused colon with abrupt truncation of the column of contrast agent in
the distal ileum.
View Media Gallery
 Meconium ileus. Main differential
consideration in ileal atresia. Plain radiograph of the abdomen demonstrates dilated loops of
bowel with no rectal air and with a soap-bubble appearance in the right lower quadrant.
View Media Gallery

Meconium ileus in the same patient as in

the previous image. Contrast enema study demonstrates microcolon with reflux of contrast
agent into small bowel of a caliber similar to that of the colon. Round, tubular filling defects
that represent inspissated meconium are demonstrated in the small bowel.
View Media Gallery
Both meconium ileus and ileal atresia cause distal obstruction and microcolon. In contrast to
ileal atresia, meconium ileus classically shows few, if any, air-fluid levels. Plain radiographs
may show a characteristic bubbly appearance in the right lower quadrant, which represents
inspissated meconium mixed with air (see the image below).

Meconium ileus. Main differential

consideration in ileal atresia. Plain radiograph of the abdomen demonstrates dilated loops of
bowel with no rectal air and with a soap-bubble appearance in the right lower quadrant.
View Media Gallery
A definitive diagnosis of ileal atresia can usually be made by performing a contrast enema
study. In meconium ileus, the contrast agent can be refluxed past the obstructing, inspissated
meconium into the dilated proximal ileum. The inspissated meconium may appear as round
or tubular filling defects in the contrast material–filled small bowel (see the following image).
Furthermore, contact with the contrast material usually softens and loosens the inspissated
meconium, allowing it to pass. This feature makes contrast enema study the preferred initial
therapy for meconium ileus, whereas ileal atresia always requires surgical repair.
 Meconium ileus in the same patient as in
the previous image. Contrast enema study demonstrates microcolon with reflux of contrast
agent into small bowel of a caliber similar to that of the colon. Round, tubular filling defects
that represent inspissated meconium are demonstrated in the small bowel.
View Media Gallery

Technique

It is important to reflux contrast material as far into the small bowel as is necessary to allow
differentiation between ileal atresia and meconium ileus and to allow the material to contact
all of the inspissated meconium.

Choice of contrast agent

With regard to the type of contrast agent, dilute, water-soluble contrast material is preferred
for the diagnostic enema study in the neonate. The rationale for using water-soluble,
iodinated contrast instead of barium involves multiple factors. The first is the potential for
spilling contrast material into the peritoneal cavity in the clinical setting of necrosis and
perforation of the atretic bowel. On occasion, the perforation is not demonstrated on the
initial plain image. Barium in the peritoneal cavity is not absorbed and may induce the
development of adhesions. Even if no perforation is present, water-soluble contrast agent in
the colon is preferable to barium if the patient needs immediate surgery after the examination.
Furthermore, 2 entities in the differential diagnosis of low intestinal obstruction, meconium
ileus and functional immaturity of the colon, often clinically improve after an enema study
performed with water-soluble contrast. A follow-up therapeutic contrast enema procedure
increases the likelihood of successful treatment of meconium ileus. Therapeutic enema is
more likely to be successful if the preceding diagnostic enema was performed with water-
soluble contrast material rather than barium.
Another concern is that barium can become inspissated in the colon and may be difficult to
evacuate. Finally, the improved depiction of mucosal detail with barium is not important in
the newborn.
The iodinated contrast used for the enema study should be diluted to be nearly isosmolar to
serum, yet it should be dense (opaque) enough to be adequately visualized. Use of undiluted,
high-osmolality contrast agents in infants can cause fluid shifts from the intravascular space
to the lumen of the colon and result in life-threatening serum electrolyte imbalances.
High- and low-osmolality agents provide adequate contrast if iodine concentrations greater
than 180 mg iodine per milliliter are used. [15] Normal serum osmolality is 285 mOsm/kg of
water. High-osmolality, water-soluble agents include sodium and meglumine salts of
diatrizoate and iothalamate, and these range in osmolality from 400-2000 mOsm/kg of water.
If diluted to be nearly isosmolar to serum, these agents may be used instead of relatively
expensive, nonionic, low-osmolality contrast agents. The low-osmolality, water-soluble
agents range in osmolality from 290 (isosmolar) to 844 mOsm/kg of water.
The osmolality of most commercially available contrast agents is specified on the product
insert. If the package insert does not state the osmolarity of the product, a particular dilution
is recommended (eg, 1:3-5 for agents with high osmolality). As an alternative, this
information can be found in Appendix A: Contrast Media Specifications of the Manual on
Contrast Media (version 7) from the American College of Radiology. [16]

Degree of confidence

The degree of confidence in plain radiographic findings is high for determining the presence
of high versus low obstruction in ileal atresia. For low obstruction, further imaging is
necessary to localize the site and nature of the obstruction.
Contrast enema study offers a high degree of confidence in the diagnosis if good reflux of
contrast agent into the small bowel can be achieved.
Ultrasonography
Ultrasonography is valuable in the diagnosis of in utero bowel obstruction, as the dilated
bowel is filled with fluid rather than air. The number of dilated loops indicates proximal
versus distal obstruction, and findings may be specific in some causes of proximal
obstruction, such as the double bubble of duodenal atresia. [9, 17,18]
In the postnatal period, air is introduced into the gut, making ultrasonography less useful than
before this period, particularly in distal obstruction, in which a great deal of air absorbs the
ultrasound beam.
If the results of the contrast enema study are equivocal, ultrasonography may help distinguish
ileal atresia from ultrameconium ileus in select cases. Meconium ileus is characterized by
echogenic material within dilated loops of small bowel, whereas ileal atresia results in
intestinal dilatation with anechoic fluid. [19] The following are radiographs and an
ultrasonogram in the same patient with meconium ileus and cystic meconium peritonitis.

Meconium ileus. Main differential

consideration in ileal atresia. Plain radiograph of the abdomen demonstrates dilated loops of
bowel with no rectal air and with a soap-bubble appearance in the right lower quadrant.
View Media Gallery
 Meconium ileus in the same patient as in
the previous image. Contrast enema study demonstrates microcolon with reflux of contrast
agent into small bowel of a caliber similar to that of the colon. Round, tubular filling defects
that represent inspissated meconium are demonstrated in the small bowel.
View Media Gallery

Cystic meconium peritonitis in the

same patient as in the previous image. Ultrasonogram shows rounded, echogenic near-surface
and posterior acoustic shadowing. Distal ileal perforation was discovered at surgery.
View Media Gallery
Ultrasonography may elucidate associated findings, such as meconium peritonitis. Fibrotic
tissue with calcifications has an echogenic appearance with posterior acoustic shadowing.
When peritonitis is generalized throughout the peritoneum, a snowstorm appearance may be
demonstrated.
Although they typically do not cause obstruction in the newborn period, enteric duplication
cysts are well evaluated with ultrasonography. The wall of the cyst is characterized by a
layered appearance with an inner echogenic layer of mucosa, surrounded by a hypoechoic
layer of muscularis propria, the so-called gut signature. Peristalsis may also be observed in
the cyst.
In a systematic review of 16 studies including 640 fetuses, the accuracy of prenatal
ultrasound in detecting nonduodenal small bowel atresia was very variable, ranging from 10-
100%, with an overall prediction of 50.63%. The individual detection rates for jejunal and
ileal atresia were 66.30% and 25.87%, respectively. Both dilated bowel and polyhydramnios
had an overall low detection rate for these anomalies.[20]

Degree of confidence

In utero, the finding large number of dilated fluid-filled loops has a high positive predictive
value, but this finding does not differentiate one cause from another.
https://emedicine.medscape.com/article/409746-overview#showall
Background
Jejunoileal atresias and stenoses are major causes of neonatal intestinal obstruction. Atresia—
derived from the Greek components a- ("no" or "without") and tresis ("hole" or "orifice")—
refers to a congenital obstruction with complete occlusion of the intestinal lumen; it accounts
for 95% of obstructions. Stenosis—derived from the Greek components stenos ("narrow")
and -osis ("process") and denoting narrowing—refers to a partial occlusion with incomplete
obstruction and accounts for the remaining 5% of cases. [1]
In 1955, Louw and Barnard demonstrated the role of late intrauterine mesenteric vascular
accidents as the likely cause of jejunoileal atresias, rather than the previously accepted theory
of inadequate recanalization of the intestinal tract. [2] Since then, other factors (eg, in-utero
intussusception, intestinal perforation, segmental volvulus, and thromboembolism) have also
been shown to cause jejunoileal atresia. [3] Atresias can also develop in patients with
gastroschisis and in those with meconium ileus.
Intestinal atresia or stenosis can occur anywhere along the gastrointestinal (GI) tract, and the
anatomic location of the obstruction determines the clinical presentation. Most newborns with
intestinal obstruction present with abdominal distention and bilious emesis in the first 2 days
of life, though the presentation can be delayed for weeks in infants with stenosis. Bilious
vomiting in the neonate should be considered secondary to a mechanical obstruction until
proved otherwise, and emergency surgical evaluation is warranted in every newborn with this
symptom.
Hyperbilirubinemia is also common. Intestinal obstruction increases the enterohepatic
circulation of bilirubin and often results in jaundice. About 10% of infants with jejunal or
ileal atresia have cystic fibrosis and meconium ileus.
In 1911, Fockens reported the first successful surgical repair of a patient with small intestinal
atresia. [4, 1] However, the mortality associated with surgical correction of this condition
remained high for many years, even in the best pediatric surgical centers. [4] The survival of
patients with intestinal obstruction has markedly improved over the past 20 years as a result
of improved understanding of intestinal physiology and the etiologic factors of the condition,
refinements in pediatric anesthesia, and advances in surgical and perioperative care of
newborns. [1]
Morbidity and mortality are usually linked with other medical conditions, such as short-
bowel syndrome and cardiac anomalies, often associated with duodenal atresia
(predominantly in infants with Down syndrome), prematurity, respiratory distress syndrome
or cystic fibrosis, other congenital anomalies, the complexity of the lesion, and surgical
complications.
Pathophysiology
The pathophysiology of dudoenal stenosis and atresia differs from that of obstructions located
more distally in the jejunoileal area; the importance of this difference cannot be overstated. In
duodenal atresias, a failure of recanalization of intestinal tube occurs at 8-10 weeks' gestation
after obliteration of the lumen by epithelial proliferation at 6-7 weeks; it usually occurs in the
second part of the duodenum. Incomplete recanalization can lead to duodenal stenosis or the
presence of a duodenal web. [5]
In jejunoileal atresias, however, the underlying process is an ischemic injury to the gut,
usually secondary to malrotation with volvulus or intestinal strangulation with the umbilical
ring, intestinal perforations, or vasoconstrictive drugs (eg, cocaine, ephedrine, and nicotine).
Jejunoileal atresias occur after intestinal development because of the presence of bile
droplets, meconium, or lanugo distal to the atresia.[6]
Dalla Vecchia et al performed a 25-year retrospective review and found 277 neonates with
intestinal atresia. [7] The level of obstruction was duodenal in 138 patients, jejunoileal in 128,
and colonic in 21. Of the 277 neonates, 10 had obstruction at more than one site. Jejunoileal
atresia was associated with intrauterine volvulus (27%), gastroschisis (16%), and meconium
ileus (11.7%).
In atresias of the small intestine, the jejunum and ileum are equally affected. [8, 9, 1]The
proximal jejunum is the site of atresia in 31% of cases, the distal jejunum in 20%, the
proximal ileum in 13%, and the distal ileum in 36%. [1] In more than 90% of patients, the
atresia is single; however, multiple atresias are reported in 6-20% of cases. [10, 1]
Stollman et al published a large series of jejunoileal atresias as a retrospective review at a
large pediatric referral center in the Netherlands. [11] Between 1974 and 2004, they found 114
infants with jejunoileal atresia. In all, 62% of atresia and stenosis cases were noted in the
jejunum, 30% in the ileum, and 8% in both the jejunum and the ileum; 7% of patients had
intestinal stenosis, 16% had type I atresia, 21% had type II, 24% had type IIIa, 10% had type
IIIb, and 22% had type IV (see Classification below).
Heij et al performed a retrospective analysis of 21 patients with jejunal atresia and 24 with
ileal atresia and found more differences than similarities between the groups (see Table 1
below). [12, 13, 1, 14, 3, 15]
Table 1. Differences Between Jejunal and Ileal Atresia [12, 13, 1, 14, 3, 15] (Open Table in a new
window)
Characteristic Jejunal Atresia Ileal Atresia

Gestational age Lower than that of ileal atresia Low

Birth weight Lower than that of ileal atresia Low

Atresias May be multiple Simple

Antenatal perforation Uncommon Common

Associated malformations Some Rare

Postoperative course Prolonged Short

Mortality Higher than that of ileal atresia Low

In this analysis, mean birth weight and gestational age were significantly lower in patients
with jejunal atresia than in those with ileal atresia. [12] Most jejunal atresias were multiple,
whereas most ileal atresias were single. Antenatal perforation was frequent (10 cases) in ileal
atresia but infrequent (two cases) in jejunal atresia. The postoperative course was often
prolonged, and mortality increased in patients with jejunal atresia, three of whom died (all of
them patients with apple-peel deformity); by comparison, one patient with ileal atresia died.
Heij et al suggested that some of their findings may be explained by a difference in bowel-
wall compliance between the jejunum and the ileum. [12] The compliant jejunal wall allows
massive dilatation with subsequent loss of peristalsis, accounting for the prolonged
postoperative course and the relatively high rate of perforation in ileal atresia.

Classification

Duodenal atresias have several basic morphologies, as follows:

 Type I atresias constitute luminal webs or membranes, some of which contain a central
defect or fenestration of variable size, and result in a marked size discrepancy with
mural continuity
 Type II atresias have dilated proximal and diminutive distal segments connected by a
fibrous cord
  Type III atresias are characterized by a complete discontinuity between the segments
The relation between the point of obstruction and the ampulla of Vater is important. Most
series document a predominance of postampullary obstructions. Obstructions caused by type
I membranes are frequently associated with anomalies of the common bile duct in which the
common bile duct may terminate within the membrane itself. [16]
The maximal dilatation of the proximal segment occurs at the point of obstruction. This
segment is commonly aperistaltic, of questionable viability, or both. [4] Grosfeld et al
modified Louw’s original classification into the following description of intestinal atresia,
which is currently the most commonly used classification scheme[9] :
 Type I – Membrane
 Type II – Blind ends joined by fibrous cord
 Type IIIa – Disconnected blind end
 Type IIIb – Apple-peel deformity
 Type IV – Multiple, string of sausages
Stenosis
The proximal dilated intestine is in continuity with the distal nondilated bowel, and the
mesentery is intact. Between these portions, a narrow, semirigid segment with a minute
lumen is present. The small-bowel length is normal. This lesion might simulate a type I
atresia (see the image below).

Intestinal stenosis. Dilated

prestenotic bowel is in continuity with distal intestine. No mesenteric gap is present. Bowel
length is normal.
View Media Gallery
Atresia type I
Type I is a mucosal (septal) atresia with an intact bowel wall. The proximal dilated intestine
is continuous with the distal narrow one. The mesentery is intact, and the intestinal length is
normal. The pressure generated on the internal membrane may elongate it as a windsock,
giving a conical appearance to the transition. The distal intestine is collapsed (see the image
below) but may contain meconium.

Intestinal atresia type I. Transition

area has conical appearance due to windsock elongation of membrane. No mesenteric gap is
present. Bowel length is normal.
View Media Gallery
Atresia type II
In type II, a fibrous cord separates the proximal bowel from the distal segment. The
mesentery is usually intact, but a small V-shaped defect may be present. Intestinal length is
normal. The proximal blind pouch is grossly dilated, often aperistaltic and cyanotic.
Perforations may be noted in patients who present late. Dilatation usually extends 10-15 cm
proximally, after which point the intestine appears relatively normal. The distal blind pouch
may be mildly distended because of retained cellular debris (as in fetal intussusception) (see
the image below).
 Intestinal atresia type II. Proximal
dilated bowel is separated from distal narrow bowel by fibrous cord, in this case, without
mesenteric gap. Bowel length is normal.
View Media Gallery
Atresia type IIIa
Type III atresias seem to be the most common. [8, 17] Intrauterine resorption of fetal gut
subjected to a vascular insult explains the reduced bowel length commonly seen in this type
of atresia. The distal bowel is small and decompressed.
In type IIIa atresia, the two blind ends are completely separated without a fibrous cord
between them. The atresia has a V-shaped mesenteric gap, and the intestine is shortened (see
the images below). The proximal dilated pouch may have questionable viability and undergo
torsion.

Intestinal atresia type IIIa. Two

blind ends are separated completely. V-shaped mesenteric gap is present. Intestinal length is
reduced.
View Media Gallery
 Surgical image of jejunal atresia
type IIIa, with proximal dilated pouch, completely separated from the distal narrow intestine,
over V-shaped mesenteric defect.
View Media Gallery

Surgical image of newborn with

type IIIa ileal atresia shows two blind ends with dilated proximal segment and decompressed
distal segment, with V-shaped gap in mesentery. Overall intestinal length is normal. Image
courtesy of Rodrigo Díaz, MD.
View Media Gallery
Atresia type IIIb
In type IIIb atresia (Christmas-tree or apple-peel deformity), the two intestinal segments are
separated as in type IIIa, and the mesenteric defect is large. The proximal atretic segment is in
the upper jejunum, near the ligament of Treitz; the pouch is distended and lacks dorsal
mesentery. The superior mesenteric artery distal to the middle colic branch is absent. The
collapsed distal intestine helically encircles a small vessel (marginal artery) arising from the
ileocolic or right colic arcades, or the inferior mesenteric artery, and its vascularity may be
impaired.
Type I and type II atresias may coexist in the distal segment. The intestine is always
substantially shortened (see the image below). Many patients with this variant have low birth
weight (70%) and were born premature (70%); they may also have malrotation (54%),
multiple atresias, and an increased number of other associated anomalies that raise the
prevalence of complications (63%) and increase the mortality (54-71%). [18, 1, 19]

Intestinal atresia type IIIb (apple-peel or

Christmas-tree deformity). Proximal pouch is dilated. Collapsed distal intestine encircles
marginal artery helically. Intestinal length is substantially reduced.
View Media Gallery
Atresia type IV
Type IV atresia refers to any number and combination of type I, II, or III atresias that present
simultaneously, creating a string-of-sausages appearance (see the image below). A possible
cause is intrauterine inflammation. However, findings of this type of atresia in family
members suggest possible autosomal recessive transmission.[20, 10, 21]
 Intestinal atresia type IV. Multiple
atresias appear simultaneously as "string of sausages." Intestinal length is invariably and
considerably shortened.
View Media Gallery
The presence of multiple GI atresias with cystic dilatation of the bile duct is rare; the
association has been described in 37 patients, with no recorded survivors in the world
literature. [22, 23] The dilatation of the bile duct seems to be due to normal drainage of bile into
a closed-loop duodenal obstruction. Patients present with multiple atresias and die of short-
bowel syndrome and complications related to total parenteral nutrition (TPN).
Etiology
Intrinsic duodenal obstructions and annular pancreas result from events that occur during
early development of the foregut. Duodenal atresia and stenosis are believed to result from a
failure of recanalization of the embryonic duodenum, which becomes solid as a result of early
epithelial proliferation.
Annular pancreas occurs when the ventral pancreatic bud fails to rotate behind the duodenum,
leaving a nondistensible ring of pancreatic tissue fully encircling the second portion of the
duodenum. It frequently coexists with intrinsic duodenal anomalies and anomalies of the
pancreaticobiliary ductal system, suggesting closely linked mechanisms of pancreatic,
duodenal, and biliary development during this stage. [16]
That associated congenital malformations are more prevalent with duodenal atresia than with
jejunoileal atresia suggests that proximal obstructions occur earlier in fetal life. [7, 24]
Unlike duodenal atresias, many jejunoileal atresias are separated by a cordlike segment or a
V-shaped mesenteric gap. This finding and the usual finding of bile pigments and lanugo
distal to the atretic segment indicate that an in-utero vascular accident occurring relatively
late in gestation (>11-12 weeks) is the likely origin of these atresias, rather than failure of GI
tract recanalization. A localized intrauterine vascular accident with ischemic necrosis of the
bowel and subsequent reabsorption of the affected segment is the favored theory. [25, 2, 4, 1, 26]
De Chadarevian et al reported on an infant with inherited thrombophilia that created a
hypercoagulable state, favoring a segmental intestinal thrombosis and resulting in terminal
ileal atresia. [27] This patient was also found to have Hirschsprung disease, which is rarely
associated with intestinal atresia.
The localized nature of a vascular insult explains the low (10%) prevalence of coexisting
conditions. Intestinal atresia associated with in-utero intussusception or perforation,
malrotation, volvulus, internal hernias, gastroschisis, and omphalocele further corroborates a
vascular event as the etiology of most jejunoileal atresias. [28,26, 29, 30]
Only one case of a newborn patient has been reported to date with multiple intestinal atresias
associated with multifocal angiodysplasia of the intestinal wall. [31]
Sweeney et al examined 38 patients with jejunal atresia and 45 patients with ileal atresia at
the Children's Research Center in Dublin, Ireland. [24] Compared with patients with ileal
atresia, patients with high jejunal atresia had a higher rate of associated congenital
malformations (42% vs 2%), a higher rate of multiple or apple-peel (type IIIb) atresias (53%
vs 9%), and a higher mortality. These results suggest that jejunal atresia may also develop
from a malformative process.
In a collaborative study in France, Gaillard et al reviewed 102 cases from 42 induced
abortions and 22 stillborns, as well as surgical findings in 38 neonates. [32]Abnormalities such
as meconium ileus (associated with cystic fibrosis) and chromosomal aberrations (eg, Down
syndrome) were present during the second trimester of gestation. Intestinal atresia and
stenosis were detected in the third trimester of pregnancy and were associated with ischemic
conditions.
Although most infants have only one atretic segment, multiple atresias have been described in
infants of mothers who ingested ergotamine and caffeine or pseudoephedrine (alone or with
acetaminophen) during pregnancy. [33, 34] Other vasoconstrictive factors (eg, cocaine abuse
and smoking during pregnancy) have also been linked with increased risk for intestinal
atresia. [34] The risk is also higher in patients with graft-versus-host disease and
immunosuppression and those with malformative processes that are likely due to autosomal
recessive transmission. [35,21]
Multiple intestinal atresias have been reported in rare association with pyloric atresia and
pylorocholedochal fistula. [36]
In a study of 114 cases of jejunoileal atresia in the Netherlands, Stollman et al found other GI
anomalies in 24% of patients, genitourinary malformations in 9%, cystic fibrosis in 9%,
neurologic anomalies in 6%, and congenital heart disease in 4%. [11]
Duodenal obstructions of congenital origin are often associated with other congenital
anomalies, which account for most of the morbidity and mortality in these patients. Various
publications report a 50-80% incidence of associated conditions. Congenital heart disease and
trisomy 21 are the most common of these associated conditions, each occurring in about 30%
of cases. [37] All three conditions may coexist in the same patient. [38]
In a study of patients with trisomy 21 who underwent antenatal ultrasonography, about 4%
showed prenatal evidence of duodenal atresia. [39] Other associated anomalies include
intestinal malrotation (20%), esophageal atresia, imperforate anus (10-20%),
thoracoabdominal heterotaxia, and gallbladder agenesis.
One of the most important factors to keep in mind is that in duodenal atresia, as in other
neonatal diseases, the outcome for patients depends more on the severity of the associated
anomalies and the ease with which they can be corrected than on the surgical management of
the obstruction itself. [16]
Familial cases of various types of atresia have been described. [40] In one family, familial type
I jejunal atresia affected three members from two generations. Proximal atresia was
associated with renal dysplasia. Knowledge of the familial form of the disease indicates that
most cases of jejunoileal atresia actually result from disruption of a normal embryologic
pathway, most likely the development of the superior mesenteric artery and its branches.
They should be considered to be true embryologic malformations rather than acquired
lesions. [40]
This association is presumably an autosomal dominant condition. [22] Matsumoto et al
reported a case in Japan and reviewed the literature, finding six other cases of small intestinal
atresia occurring in twins. [41] All published cases except one involved identical twins. Three
pairs of twins had different types of atresia, and four pairs had no other anomalies. The other
members of these families were not affected; this finding suggested that such cases may be
due to environmental influences during gestation.
Another report of different intestinal atresias in identical twins suggested them to be either
the consequence of linkage of two genes or a pleiotropic expression of a single gene. [42]
Epidemiology
Congenital duodenal obstruction may be complete or partial, intrinsic or extrinsic. Intrinsic
obstruction occurs in about 1 of 7000 live births and accounts for about half of all small-
bowel atresias. Extrinsic obstruction has many causes, including malrotation with Ladd
bands, other congenital bands not associated with malrotation, [43] preduodenal portal vein,
gastroduodenal duplications, cysts or pseudocysts of the pancreas and biliary tree, and
annular pancreas (which is commonly associated with a duodenal web, an intrinsic cause of
duodenal obstruction [16] ).
In West Africa, intestinal atresia is the fourth most common cause of neonatal intestinal
obstruction, after anorectal malformations, Hirschsprung disease, and strangulated inguinal
hernias. [44] In an 11-year retrospective review of 500 children in India, Ranan et al found
intestinal atresias to be the most common cause of intestinal obstruction in newborns and the
second most common cause (11.8%) after intussusception (20.8%) in all age groups. [45]
Boys and girls are equally affected. [8] In most studies, jejunoileal atresias seem to be more
common than duodenal atresias, and colonic atresias account for the fewest cases.
Unlike duodenal atresia, jejunoileal atresia associated with Down syndrome is uncommon.
Patients with intestinal atresia are epidemiologically characterized by young gestational age
and low birth weight. In addition, the atresia is associated with twinning, the parents are more
often consanguineous compared with parents of healthy neonates, and vaginal bleeding
frequently complicates the pregnancies.
No correlation between jejunoileal atresia and parental age or disease has been
proved. [14, 13, 3] However, one study from France reported an increased prevalence of
intestinal atresias in infants born to teenagers. [14] Some maternal infections may be associated
with ileal atresia. [13]
Prognosis
Before the mid-20th century, the mortality associated with small-bowel atresias was
prohibitive (>90%). By the late 1950s, the survival rate had risen to 78%. Survival rates
improve with distal atresias, whereas mortality is high in instances of multiple atresia (57%);
apple-peel deformity (54-71%); and atresias associated with meconium ileus (65%),
meconium peritonitis (50%), or gastroschisis (66%). [18, 1, 19, 26]
Overall survival rates (including preterm babies) have reached 90%, with a surgical mortality
of less than 1%. [18, 7, 19] Mortality is related to sepsis, associated anomalies, prematurity,
malrotation, meconium peritonitis, and long-term TPN complications in patients with short-
bowel syndrome.
The most common cause of death in infants with jejunoileal atresia is infection related to
pneumonia, peritonitis, or sepsis. [8, 1] Sato et al reported on an infant with ileal atresia and
meconium peritonitis after a perforation who presented with pylephlebitis (air in the portal
system) and a pulmonary gas embolism. [46] The patient had respiratory distress, shock,
disseminated intravascular coagulation, and intractable diarrhea but eventually recovered and
was discharged from the hospital after 4 months.
The most important surgical complications are anastomotic leaks and functional obstruction
at the level of the anastomosis; these occur in as many as 15% of patients. [8, 17] In 84 patients
with congenital jejunoileal or colonic atresia who were treated in New South Wales,
Australia, mortality was higher in infants who underwent stoma formation than in those who
received a primary anastomosis. [47] Reoperation may be requried to prevent
complications. [48]
Short-bowel syndrome refers to a spectrum of malnutrition problems that result from
inadequate bowel length, which may occur in patients born with multiple atresias or in those
with the apple-peel deformity. It is a cause of intestinal failure, together with other congenital
diseases of enterocyte development, and severe motility disorders (total or subtotal
aganglionosis or chronic intestinal pseudo-obstruction syndrome). [49] Other common causes
of short-bowel syndrome include necrotizing enterocolitis and midgut volvulus. [50]
Of the 114 patients involved in the study by Stollman et al, 28% developed early
postoperative complications, whereas 17% experienced late postoperative complications;
mortality was 11%. [11] Short-bowel syndrome seems to be the biggest problem, resulting in
longer hospital stay, more feeding problems, and higher morbidity and mortality.
Today, the survival rate for patients with short-bowel syndrome is 80-94%. The presence or
absence of the ileocecal valve appears not to affect mortality but does affect the length of
time for which TPN is required and thus affects the complications related to its use (eg,
predisposition to infection, central line sepsis, and TPN-related cholestasis). [7] Malabsorption
and steatorrhea are most severe in patients with terminal ileal resection, particularly when the
ileocecal valve is excised. Vitamin B supplements are useful in such patients.
Overall mortality due to intestinal atresia does not seem to depend on the location of
obstruction. Prematurity, birth weight less than 2 kg, and associated anomalies are
independent risk factors for prolonged hospital stay and higher mortality. [51, 52]
Lack of sufficient residual bowel is responsible for considerable morbidity or a poor quality
of life. In most instances, maximal intestinal adaptation occurs within 6-12 months, but it
may take longer. [26] The use of the longitudinal intestinal lengthening and tailoring (LILT)
procedure, proposed by Bianchi and modified by Aigrain, can allow the child to be weaned
from parenteral nutrition. [53]
As a consequence of advances in medical care, such as improved surgical techniques and
parenteral nutrition, the survival of neonates with intestinal atresias improved dramatically in
the 20th century. [54]
The presence of multiple intestinal atresias necessitates repeated surgical interventions and
sometimes small-bowel transplantation; the development of short-bowel syndrome in these
patients is associated with increased risk of cholestasis, liver cirrhosis, and hepatic failure.
The prognosis is poor, with most infants dying early in life. [55] Patients with this condition
often have immunodeficiency. [56] Thymic dysplasia and lymphoid depletion are key findings
in patients with mutations in the TTC7A gene who have multiple intestinal atresias.
https://emedicine.medscape.com/article/939258-overview#showall
History and Physical Examination
Jejunoileal atresias can be identified on the basis of polyhydramnios present during prenatal
ultrasonographic evaluation, bilious vomiting, abdominal distention, and jaundice. Some
patients may not pass meconium in the first day of life.
The clinical presentation of the infant with congenital duodenal obstruction depends on the
presence or absence of a membranous aperture, its size, and the location of the obstruction
relative to the ampulla. The classic presentation of a complete postampullary obstruction
includes bilious vomiting within 24 hours of birth in an otherwise stable infant with a
nondistended abdomen.
Findings on physical examination frequently are not particularly revealing. Most patients
present with some degree of abdominal distention, which varies according to the level of
obstruction. Tenderness or abdominal mass is mostly absent. Therefore, the presence of these
findings suggests that there may be a complicated obstruction associated with ischemia or
prenatal perforation or that the cause of obstruction may be malrotation with midgut volvulus.
Plain radiographs of the abdomen typically show the classic double-bubble sign: two distinct
gas collections or air-fluid levels in the upper abdomen, resulting from the markedly dilated
stomach and proximal duodenal bulb. If the infant’s stomach has been decompressed by
vomiting or previous nasogastric aspiration, 30-60 mL of air may be carefully injected
through the nasogastric tube and the double-bubble sign reproduced. Air makes an excellent
contrast agent, obviating a barium or water-soluble contrast study in routine cases.
The distal intestinal tract may be gasless or may contain a small amount of intraluminal air
deriving from a membranous aperture or perforation, or there may be an anomalous bile duct
with openings on both sides of the obstructing diaphragm. [57]
Common clinical characteristics of patients with jejunoileal atresia include the following:
 Polyhydramnios on antenatal ultrasonography (28%)
 Prematurity (35%)
 Low birth weight (25-50%)
Classic signs include the following:
 Bilious emesis that warrants emergency surgical evaluation (most patients)
 Abdominal distention (in distal atresia)
 Jaundice (32%)
 Failure to pass meconium in the first 24 hours (rule out Hirschsprung disease; passage
of meconium does not rule out intestinal atresia)
Signs of continuous fluid loss include the following:
  Dehydration, manifested by sunken fontanel and dry membranes
 Decreased urine output (the best clinical indication of tissue perfusion)
 Tachycardia
 Decreased pulse pressure
 Low-grade fever
 Neurologic involvement, manifested by irritability, lethargy, or coma
Patients are frequently premature (35%). [13] One third of infants with jejunal atresia, one
fourth of those with ileal atresia, and more than one half of those with multiple atresias have
low birth weight. [1]
Most patients present with bilious emesis, which indicates that the obstruction is distal to the
ampulla of Vater.
Initially, the patient's pulse rate, respiratory rate, blood pressure, and temperature are usually
within the reference range. As the patient loses fluid into the bowel and experiences
vomiting, diminished plasma volume is reflected as tachycardia, decreased pulse pressure,
and sometimes low-grade fever.
Immediately after delivery, the patient appears relatively healthy. Over time, the patient
develops signs of hypovolemia (sunken eyes, sunken fontanel, dry skin and mucous
membranes, and prolonged capillary refill time), which are due to vomiting and intra-
abdominal third-space loss secondary to the obstruction.
These patients are hungry and properly suck milk; however, they cannot tolerate feedings and
continue to vomit profusely. They eventually become lethargic and hyporeactive, with
muscle flaccidity. They can develop skin mottling, cardiovascular instability, and
neurological involvement (irritability or coma).
A proximal small-bowel obstruction results in loss of fluids that resemble gastric juice and
thus produces hypokalemic and hypochloremic metabolic alkalosis. With distal small-bowel
obstruction, fluid losses are usually isotonic, and thus, serum electrolytes are normal until
sufficient dehydration results in metabolic acidosis, as demonstrated by tachypnea, low serum
bicarbonate levels, and elevated serum chloride values.
Adequate tissue perfusion is evaluated by observing the patient's capillary refill time, pulse
rate, blood pressure, and urine output. If the patient is severely dehydrated, tenting of the skin
may be noted.
About 32% of infants with jejunal atresia and 20% of those with ileal atresia have jaundice,
which is characteristically due to indirect hyperbilirubinemia. [17]
Abdominal distention is most evident in cases of ileal atresias, in which it is diffuse, as
opposed to proximal jejunal atresias, in which the upper abdomen is distended and the lower
abdomen is scaphoid.
Intestinal loops and their peristalsis may be seen through the thin abdominal wall of
newborns (see the image below).

Clinical image of newborn male

with diffuse abdominal distention. Bowel loops and their peristalsis could be seen on
abdominal surface. Collateral venous distention is also evident. Patient had distal type IIIa
ileal atresia.
View Media Gallery
These babies’ abdomens are usually soft, without signs of peritonitis. In-utero perforations
usually seal before delivery. However, an excessively dilated proximal segment may undergo
torsion, necrosis, or perforation. In these cases, the patient appears septic and dehydrated, and
the abdominal wall may be discolored.
The patient's ability to pass some meconium does not exclude intestinal atresia. Cellular
debris and swallowed amniotic fluid and lanugo form meconium, explaining this finding; this
formation occurs earlier in gestation than the insult that produces the atresia.
Upon laboratory examination, an elevated hematocrit level secondary to hemoconcentration
due to reduced plasma and extracellular fluid volume loss may be detected. The white blood
cell (WBC) count may be either normal or elevated. Patients may present with indirect
hyperbilirubinemia and the electrolyte disturbances mentioned above
Antenatal Ultrasonography
In many case, the diagnosis of duodenal atresia is suggested by antenatal ultrasonography.
Duodenal atresia is the most commonly detected form of intestinal obstruction in the fetus. A
maternal history of polyhydramnios is common (~75% in one series). [61]
Antenatal sonographic evaluation of the fetus at 22-23 weeks' gestation can reliably detect
two dilated fluid-filled structures consistent with a double bubble. The unavailability of a
sonographic diagnosis until relatively late in gestation can pose an ethical dilemma for
prospective parents, who may consider elective termination of pregnancy on the basis of the
association of duodenal atresia with trisomy 21.
The specificity of ultrasonography has markedly improved over time. Persistently dilated
bowel loops on serial sonograms have a 66.7% positive correlation with intestinal atresia
diagnosed after birth. [62, 5] About 25-40% of the amniotic fluid is swallowed in month 4 or 5,
and the fluid is reabsorbed in the first 25-30 cm of the jejunum. [63] Proximal atresias thus are
easier to diagnose than ileal or colonic atresias because the distal lesions may not be
associated with polyhydramnios. Improved sonographic criteria for jejunoileal atresia are
needed. [64]
During the second trimester of pregnancy, atresia can also be diagnosed on antenatal
ultrasonography as a single sonolucent cyst without signs of intestinal obstruction. [65]
Unexplained umbilical cord ulceration and hemorrhage on antenatal ultrasonography has
been reported in fetuses suspected of having intestinal atresia. [66]
Radiography

Plain films of kidneys, ureters, and bladder

A plain abdominal film should be obtained in every newborn with an intestinal obstruction
(see the images below).
 Plain abdominal radiograph of newborn reveals dilated
gastric bubble and massively dilated duodenum and proximal jejunum with gasless abdomen
distal to level of obstruction; these findings are consistent with jejunal atresia.
View Media Gallery

Plain abdominal radiograph of newborn with

distal type IIIa ileal atresia demonstrates diffuse small-bowel (and gastric) distention, with
gasless pelvis. Image courtesy of Rodrigo Díaz, MD.
View Media Gallery
Although the double-bubble sign is considered pathognomonic of duodenal atresia, it can be
seen in very proximal jejunal atresias. The radiographic findings characteristic of jejunoileal
atresia are distended bowel loops with air-fluid levels proximal to the level of the obstruction.
The lower the atresia in the gastrointestinal (GI) tract, the greater the number of intestinal
loops that appear distended on the radiograph. Peritoneal calcifications, seen in 12% of
patients, suggest meconium peritonitis, a sign of in-utero intestinal perforation.

Upper gastrointestinal series

A contrast-enhanced upper GI series is seldom required. This study is typically performed to

rule out partial obstruction or malrotation, which is present in 10% of patients with
jejunoileal atresia. [17] When performed, the study shows gastric dilatation and an enlarged
small bowel up to the level of the atresia, where a blind pouch can be seen. In cases of
intestinal stenosis, the prestenotic segment appears dilated, a transition point is evident, and
contrast material can be seen in the distal bowel. (See the images below.)

Upper GI contrast study demonstrates dilated

stomach and duodenum, with enlarged upper jejunum and lack of passage of contrast agent to
distal small bowel; these findings are consistent with high jejunal atresia.
View Media Gallery
 Upper GI contrast study demonstrates
dilated stomach and proximal duodenum without further passage of contrast in newborn with
duodenal atresia.
View Media Gallery

Contrast enema study

The radiographic image of the colon of a newborn lacks the characteristic haustra seen in
older children. A contrast enema can be useful for the following purposes:
 To distinguish large-bowel distention from small-bowel distention
 To identify a site of colonic or distal ileal obstruction
 To determine the presence of a microcolon (see the images below)
 To identify the position of the cecum (this is useful in cases of abnormal rotation and
fixation of the intestine)
 Barium enema study reveals microcolon in
infant with long-standing ileal atresia. Image courtesy of Rebecca Stein-Wexler, MD.
View Media Gallery

Lower GI contrast study in newborn with ileal

atresia demonstrates microcolon with dilated non-contrast-enhanced stomach and proximal
small bowel. Image courtesy of Rodrigo Díaz, MD.
View Media Gallery
Most neonates with jejunoileal atresia have an unused microcolon, except when the vascular
accident leading to atresia occurs late in gestation, as in the case of idiopathic in-utero
intussusception. [28] It should be kept in mind that patients with meconium ileus also present
with a microcolon.
Endoscopy
Although video capsule endoscopy has been used for some time in the diagnosis of specific
GI conditions in adults, it is only comparatively recently that it has come to be used in the
diagnosis of small-intestine atresia in neonates. [67]
Rectal Biopsy
When a neonate does not pass meconium in the first 24 hours of life, Hirschsprung disease
should be a concern. In the absence of radiologic findings suggestive of atresia, suction rectal
biopsy should be performed. This is done at the bedside and does not require anesthesia. If
the patient has Hirschsprung disease, rectal biopsy reveals the absence of ganglion cells and
nerve bundle hypertrophy. [26]
Approach Considerations
The key to successful treatment of neonates with intestinal atresia is comprehensive
perioperative care. This is usually best accomplished by a team that includes experienced
surgeons, neonatologists, and nutritional support teams. Early diagnosis, proper preoperative
stabilization, the right choice of surgical procedure, and good postoperative neonatal care are
the most important considerations.
The use of growth factors to facilitate intestinal adaptation and advances in small-bowel
transplantation may further improve the long-term outcome of children with small intestinal
atresia and stenosis in the future. Although mortality and morbidity are still high, even in
large experienced centers, intestinal transplantation is emerging as a feasible management
option for children with irreversible intestinal failure and for those with serious complications
related to total parenteral nutrition (TPN).
Surgical Therapy

Preparation for surgery

Neonates tolerate surgical procedures best when they are metabolically and hemodynamically
stable. Attention should be directed to preventing or correcting hypothermia, hypovolemia,
hypoglycemia, and hypoxemia.
The patient is admitted to the neonatal intensive care unit (NICU) and restricted to receiving
nothing orally (nil per os [NPO]). The baby should be kept in a warm environment (eg, an
incubator) with humidified air, and the oxygen saturation should be monitored. Vital signs
should be frequently assessed. The airway is kept clear with frequent nasopharyngeal
aspiration. Intubation for respiratory support in patients with severe abdominal distention or
sepsis may be necessary.
Baseline laboratory investigations are performed, and blood is crossmatched. Use of
umbilical lines should be avoided, because they are associated with an increased risk of
infection and because they become suboptimal for the transverse incision employed in the
laparotomy. Specific fluid and electrolyte management of the newborn patient is beyond the
scope of this chapter. Acute hypovolemia is managed with 10- to 20-mL/kg boluses of
lactated Ringer solution.
An orogastric tube should be placed for gastric decompression and to avoid aspiration.
Neonates are obligate nasal breathers; breathing through the mouth is a learned reflex that
occurs around age 3 months. Gastric output must be replaced as well. Because urine output is
the best clinical indicator of hemodynamic stability, a bladder catheter is used to ensure an
output of 1-2 mL/kg/hr. Before surgery, 1 mg of vitamin K is administered intramuscularly
(IM), and broad-spectrum antibiotics are given intravenously (IV).
Neonates lose heat more rapidly and have higher metabolic requirements than older patients.
Care must be taken in keeping the metabolic demands satisfied and the baby warm at all
times, including during transport to the surgical suite and during induction of anesthesia.

Operative details

During anesthesia, the patient is carefully monitored. The blood loss is quantified and the
intravascular volume kept adequate.
Classically, the abdomen is entered through a supraumbilical transverse incision (see the
image below). Duodenal web resection can be safely performed laparoscopically and has
even been reported to have been done via single-incision endosurgery. [68]

Surgical approach to jejunoileal atresia is

via upper-right-quadrant transverse incision that can extend across midline if necessary.
View Media Gallery
Transumbilical and laparoscopic approaches have been reported. [69, 70, 71] Of course,
advanced laparoscopic skills are necessary. The entire intestine is delivered through the
incision to assess the anatomy and type of atresia and to rule out other anomalies. A
perforation, if present, should be controlled at this stage before further exploration is done.
The definitive way to exclude distal atresias, which occur in 6-21% of patients, is to irrigate
normal saline solution into the distal pouch and to milk it caudally.
If other anomalies are ruled out, the intestine is returned to the abdominal cavity while the
atretic segment is kept exposed. When the intestinal length is normal, the dilated proximal
pouch can be resected by removing 10-15 cm of dilated bowel proximal to the atresia, to
avoid postoperative physiologic obstruction due to lack of peristalsis.
Instillation of normal sodium chloride solution with a 24-gauge needle through a purse-string
suture into a clamped distal pouch may be useful for distending that segment and reducing
the size discrepancy between proximal and distal intestine (see the image below).

Intestinal atresia type IIIa. Clamp

is applied to distal bowel, and sodium chloride solution is instilled through purse-string suture
to dilate intestine and diminish size discrepancy between two loops so as to facilitate
anastomosis. Dotted line marks area of resection.
View Media Gallery
The proximal intestine is transected at a right angle to maximize its vascularity; the distal
bowel is transected obliquely and the incision continued along the antimesenteric border as a
fishmouth to equalize the size of the openings on the two sides for the anastomosis (see the
image below). [72]
 Proximal dilated pouch is
transected at 90° angle to maximize its vascularity, while distal intestine is transected
obliquely to diminish size discrepancy between segments.
View Media Gallery
Louw et al experimented with occluding the blood supply to some parts of the intestine under
development [2] and concluded that the supply to bowel areas adjacent to the atretic segment
was likely to be compromised, not enough to cause necrosis but enough to cause a functional
problem with resultant defective peristalsis. Thus, they recommended resection of the blind
bulbous end of the proximal intestine before the anastomosis. Mortality from intestinal atresia
at Great Ormond Street Hospital decreased from 69% to 33%. [73]
A one- or two-layer end-to-back (end-to-oblique) anastomosis is performed. The mesenteric
gap is then approximated with fine absorbable sutures, with care taken not to kink the
anastomosis or to damage the mesenteric vessels. The patency of the anastomosis can be
tested by milking intestinal air through it. The intestinal segment is then moistened with
warm saline solution and returned to the abdominal cavity. The abdominal wall is closed in
layers with absorbable sutures.
Zani et al, in a study comparing duodenoduodenostomy with duodenojejunostomy in 92
neonates with duodenal atresia, found no significant differences in outcome between the two
repairs. [74] They suggested that this finding may be pertinent to laparoscopic repair of this
condition, in that the latter procedure may be easier to perform via minimally invasive
approach.
Intestinal stenosis and type I atresias (membranes) should be treated in the same way
described above. Bypass procedures are generally suboptimal because they fail to remove the
abnormal intestine, and side-to-side anastomoses pose the risk of creating blind loops. For
membranous atresias, membrane excision with transverse enteroplasty is sometimes
appropriate, particularly when membranes are present as part of multiple atresias.
Gastrostomy tubes are not routinely used; postoperative orogastric suction suffices for gastric
decompression and is the authors' preference. For a very high jejunal atresia, however, some
authors recommend using a gastrostomy tube for stomach decompression and passing a
transanastomotic tube to allow early postoperative enteral drip feeding (see the image
below). [4] Another option for early postoperative enteral feeding is to pass an orojejunal
transanastomotic tube.

Gastrostomy used to decompress stomach

and to pass transanastomotic tube for early postoperative enteral feeding.
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Romao el al reported good results in eight patients who underwent silicon stenting after
multiple intestinal anastomoses as a means of avoiding short-bowel syndrome in the setting
of multiple viable segments of gut, such as type IV intestinal atresia.[75] According to Hall, a
transanastomotic tube significantly shortens time to full enteral feeds in infants with
congenital duodenal obstruction, significantly reducing the need for central venous access
and parenteral nutrition. [76]
When bowel length is reduced (for type III or IV atresias) and the intestine may be severely
shortened, an antimesenteric tapering jejunoplasty may be performed over a 26-French tube
by diminishing the diameter of the proximal bowel and preserving bowel length. This can be
performed manually or by using a GI anastomosis (GIA) stapler and oversewing the staple
line with Lembert sutures (see the images below). Some patients require repeat surgery to
manage functional obstruction due to an aperistaltic, dilated bowel at the anastomosis site.

Tapering jejunoplasty performed over 26F

tube to diminish diameter of proximal bowel and preserve bowel length.
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Manually sewn tapering jejunoplasty.

View Media Gallery
If the need for long-term TPN is anticipated, a central venous catheter is placed. The author
prefers to place the central catheter in the same anesthetic procedure, after the laparotomy.
In atresia associated with gastroschisis, closure of the abdominal wall takes priority over
repair of the atresia. [77] Indications for primary anastomosis versus stoma formation depend
on the degree of damage, the dilatation of the preatretic intestine at initial presentation, and
the patient's overall medical condition. Primary anastomosis is preferred but is inadvisable
when the intestine's vascular integrity is questionable or when severe peritonitis or
complicated meconium ileus is present. In such cases, resection of the atretic segment and
enterostomy are advisable.
When multiple atresias are present, a judgment must be made about whether all atresias can
be managed at the same time or whether a staged repair is necessary. In the latter case, a
temporary ostomy or ostomies are used at the appropriate levels. One-stage restoration of
intestinal continuity with preservation of maximal intestinal length should be the basic
principle of any operative management in cases of multiple intestinal atresia. [78]
Yardley et al reported on a case of combined multiple jejunoileal and colonic atresia managed
with nine primary anastomoses over a gastroperineal transanastomotic tube. [79] This seems to
be a good alternative in order to avoid the use of stomas and their attendant complications.
Although extremely rare, the association of duodenal atresia with more distal intestinal
atresias has been previously reported. However, the incidence of this association is so low
that routine exploration of the more distal intestine in cases of duodenal atresia is not
generally recommended. [80]
Some surgeons advocate revising the rest of the small bowel during duodenal intestinal
repair; however, in the largest series of duodenal atresia patients compiled to date, the rate of
a concomitant jejunoileal atresia is lower than 1%. This low incidence is not high enough to
mandate extensive inspection of the entire bowel in these patients, and a second atresia
should not be a concern during laparoscopic repair of duodenal atresia. [81]
Grosfeld et al reported on the treatment of 128 patients with jejunoileal atresia in their 25-
year review of patients at James Whitcomb Riley Hospital for Children in Indianapolis,
Indiana. [9] Resections were performed in 97 (76%) of 128 patients (anastomosis in 45 [46%],
tapering enteroplasty in 23 [24%], temporary ostomy in 29 [30%]), ostomy alone in 25
(26%), web excision in five (5%), and the Bianchi procedure in one (1%).
Stollman et al reported that primary anastomosis was performed in 69% of 114 infants with
jejunoileal atresia, whereas temporary enterostomies were used in 26%. [11]
Postoperative Care
After the procedure, the patient is transferred to the NICU. Thermoregulation with an
incubator is most important. Oxygen saturation should be monitored and maintenance fluids
administered. The gastric output is closely monitored and replaced volume for volume. Extra
boluses of 10-20 mL/kg of lactated Ringer solution may be necessary to maintain urine
output at 1-2 mL/kg/hr. Transfusion is administered if indicated.
Glucose, hemoglobin, electrolytes, and bilirubin levels are frequently monitored during the
first postoperative days, and adjustments are made accordingly. Phototherapy to avoid
kernicterus is sometimes necessary.
Although the literature does not support the use of prophylactic antibiotics beyond 24-48
hours after the procedure, the authors continue antibiotic coverage for 5 days after surgery.
A feeding gastrostomy should not be necessary for postoperative management of an
uncomplicated duodenal repair. Gastroduodenal function usually returns within 5-7 days, at
which time enteral feeding can be initiated with small boluses and the volume progressively
advanced as tolerated. One of the most problematic issues following repair of duodenal
atresia is delayed transit, usually associated with a persistently dilated and dyskinetic
proximal duodenum.
Even with the preferred diamond anastomosis, a persistent megaduodenum with symptomatic
partial obstruction and stasis can occur. This complication may be managed by means of
either tapering duodenoplasty or lateral seromuscular resection. [82] A significant number of
infants with corrected duodenal atresia also experience gastroesophageal reflux, which may
be exacerbated by an impairment in gastric emptying.
Enteral feedings are carefully started after signs of propulsive peristalsis occur, as indicated
by clear, low-volume nasogastric output; a soft, nondistended abdomen; and evidence that the
baby is passing flatus or stool.
Enteral intake can be started with most oral rehydration solutions at low rates. Breast milk, if
tolerated, is preferable to any commercial formula. If breast milk is not available from either
the mother or the milk bank, diluted or half-strength/half-volume formula can be used. The
concentration and volume are progressively increased as the patient tolerates the formula.
Hydrolyzed isotonic or low-osmolality formulas based on cow's milk protein are
recommended for this setting.
Achieving full enteral nutritional support may take several days (mean, 5-7 days) or longer,
even months later. In patients in whom return of intestinal function is predicted to be
prolonged, centrally or peripherally delivered TPN is of prime importance.
Guidelines for TPN are well established, and the use of TPN should be judicious. Estimated
nutrition goals in the first 6 months of life are approximately 100-110 kcal/kg/day and 2-3 g
of protein. A complicated postoperative period increases the patient's caloric needs by 20-
30% and should be taken into consideration.
TPN should be advanced over 3-5 days to caloric goals, and fat intake should not be
neglected. A general rule is that calories from fat (enteral, IV as intralipid formulations, or the
like) should be 40% of total calories. The goal for IV fat intake for neonates is 3 g/kg/day.
Although TPN is the main adjunctive treatment for these cases, it delays intestinal adaptation
and may cause cholestasis and subsequent liver damage. [19] Therefore, TPN should be a
bridge to full enteral nutrition, and a concerted effort should be made to use it as such. As
intestinal function returns, the patient is progressively weaned from parenteral to enteral
nutrition until the full nutritional requirements are enterally obtained.
Graduated enteric feedings and growth hormone, glutamine, and modified diets have been
successfully used to diminish TPN requirements and enhance nutrient absorption. [7] Although
this feeding is being established, metabolic surveillance of patients is important.
Daily laboratory workup should include a basic metabolic panel and assessment of calcium,
phosphorus, and magnesium levels. Triglyceride levels should be monitored while IV fat
formulations are advanced until the goal is reached. Persistent hypertriglyceridemia may
reflect metabolic dysfunction and warrants further evaluation. Also, IV fat should be withheld
from septic or hemodynamically unstable patients until this problem is solved.
A physiologic principle to remember is that the longer the time without enteral feedings, the
more severe the brush border atrophy and the longer the time needed to restore intestinal
absorptive capabilities. Enterocytes are exclusively nourished by glutamine. Lack of
glutamine supplementation reduces levels of immunoglobulin A and increases bacterial
translocation. This photosensitive substance should be added to the patient's parenteral
nutrition. Overall, the principle is to start enteral feedings as soon as the patient's clinical
condition permits.
The current definition of intestinal failure is no longer anatomic but, rather, functional:
Intestinal failure is now defined as the presence of malabsorption after clinically significant
small-bowel resection. [83]
Although the minimal length of intestine necessary to maintain nutritional status is not fixed,
newborns are estimated to require at least 10-20 cm of postduodenal small bowel to avoid
short-bowel syndrome if the ileocecal valve and colon are preserved. When the ileocecal
valve is resected, this requirement increases to 40 cm. [84]
The loss of the ileum is the hardest to compensate for. The jejunum cannot perform some
ileal functions, such as the absorption of bile acid and vitamin B12. Therefore, a patient with
ileal loss needs nutritional supplementation. The final length of the intestine is difficult to
determine at the time of the operation—first, because the length of the proximal dilated
pouch may be overestimated, and second, because the neonatal bowel is undergoing growth.
The final length may be substantially larger than the length seen at surgery.
Surgical bowel-lengthening procedures and small-bowel transplantation are beyond the scope
of this article, but they may prove beneficial in patients with short-bowel syndrome
associated with multiple atresias. This is an exciting area, with new developments and
opportunities to improve the survival and quality of life in this patient population.