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Imaging Strategy
The 3-step approach is based on the abdominal radiographic
findings in neonates with suspected intestinal obstruction.
First, initial review should rule out ominous signs, such as
pneumoperitoneum (Fig. 1), intestinal pneumatosis, or portal venous gas (Fig. 2), because these children require emergency surgical assessment. Second, one should look for classic signs, such as the following: a nasogastric tube lodged in
proximal pouch in esophageal atresia, a double bubble in
duodenal atresia, bubbly gas within the chest in congenital
diaphragmatic hernia, a triple bubble or large featureless
loop in jejunal atresia, and a soap bubble pattern within the
right lower quadrant in meconium ileus. The third step involves deciding whether the bowel gas pattern is most consistent with a high or low intestinal obstruction pattern.1 The
high intestinal obstruction pattern shows few scattered airfilled loops in the upper abdomen, whereas the low intestinal
obstruction pattern demonstrates many (3) dilated gas-
Imaging Technique
High intestinal obstruction may be attributable to esophageal
atresia with or without fistula, duodenal atresia, malrotation
with or without midgut volvulus, or jejunal atresia. Air may
be injected through the nasogastric tube as negative contrast, which further defines the level of intestinal obstruction.
An upper GI study for high intestinal obstruction is performed immediately, whereby the newborn is given contrast
barium through bottle or nasogastric tube. The neonate is
imaged in the right lateral decubitus or semiprone position,
and images are taken to demonstrate a normal duodenal Csweep (a duodenal course of back, down, anterior and up on the
oblique lateral projection). Once the barium filled duodenal
C-sweep is demonstrated, frontal supine images are obtained to
show the duodenojejunal junction coursing to the left of the L1
pedicle at the same level of the first portion of the duodenum to
confirm normal bowel rotation. At the end of the study, the
residual barium is withdrawn from the stomach through the
tube to prevent aspiration of refluxed contrast.
Low intestinal obstruction is most commonly attributable
to ileal atresia, meconium ileus, Hirschsprung disease, small
left colon syndrome, colonic atresia, or imperforate anus.
These cases can be definitively diagnosed with enema and a
slightly hyperosmolar water-soluble contrast. The study is
performed by placing a small caliber (8F) flexible Foley-type
catheter in the distal rectum just beyond the anal verge and
taping it well at the perineum. With the infant in left lateral
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J.R. Reid
22
Figure 3 Newborn with bilious vomiting. Left lateral decubitus radiograph shows a paucity of bowel gas with a few air fluid levels
(arrows), consistent with high intestinal obstruction.
Figure 1 Newborn with respiratory distress and abdominal distension. Supine abdominal radiograph shows massive free air (arrows).
Imaging Findings
decubitus position, contrast is slowly infused by gravity drip
as images are taken during the initial filling phase of the
rectosigmoid colon. Contrast is then followed through the
colon to the level of obstruction or refluxed into terminal and
distal ileum for meconium ileus. Care must be taken to avoid
rectal stimulation, and it is unwise to inflate a rectal balloon
because of potential rectal perforation.
Figure 5 Premature newborn with esophageal atresia who was ventilated with failure of the nasogastric tube to pass. Supine chest
radiograph shows the endotracheal tube (white arrow) and the nasogastric tube (black arrow), terminating in a blind-ending upper
esophageal pouch. Note air in the stomach (arrowheads) implies a
distal fistula to the tracheobronchial tree.
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Figure 6 Newborn with esophageal atresia who presented with failure to pass the nasogastric tube. Supine abdominal radiograph
shows a proximal esophageal pouch (arrow) and gasless abdomen
indicating the rare absence of a distal fistula in esophageal atresia.
Esophageal Atresia
Esophageal atresia (EA) results from abnormal tracheoesophageal cleavage during development.3 There may be an associated fistula. The incidence of EA is 1 in 3000 births, with an
equal incidence in males and female infants.3 The VACTERL
(ie, vertebral, anal atresia, cardiac, tracheoesophageal fistula,
renal and limb anomalies) association may be found in up to
50% of cases.3 Although sometimes suspected from prenatal
ultrasound findings of microgastria or polyhydramnios, the
classic clinical history of EA is failure of passage of an orogas-
Duodenal Atresia
Duodenal atresia (DA) results from lack of canalization of the
periampullary segment of the foregut early in gestation.5 The
incidence is 1 in 6-10,000 live births with no gender predilection.5 The classic clinical presentation of DA is bilious
emesis and radiographic double bubble (dilated stomach
and proximal duodenum). However, in reality, many are diagnosed prenatally by the ultrasound double bubble sign
and polyhydramnios (Figs. 8 and 9).6 Duodenal atresia is
associated with biliary and other anomalies in 50% of cases.7
24
As many as one-third of patients with DA have trisomy 21,
yet only 15% of fetuses with trisomy 21 have DA.3 Other
associations include preduodenal portal vein, which is rarely
the primary cause of obstruction in these infants, and annular
pancreas in 20%.3 Duodenal atresia/stenosis may also be seen
with VACTERL association. Imaging includes a supine abdominal radiograph in the stable newborn with nonbilious
vomiting. Air may be injected as negative contrast with exclude duodenal stenosis over atresia.8 Rarely, malrotation
with midgut volvulus may present with high intestinal obstruction pattern on abdominal radiographs and mimic DA.9
With this clinical concern, an upper GI study is performed
with the use of single contrast barium. If high-grade obstruction is demonstrated with upper GI study, then urgent laparotomy is indicated to exclude malrotation with midgut volvulus. Ultrasound or magnetic resonance imaging (MRI) is of
limited value in defining the anatomy before surgical correction for DA. With duodenal stenosis, an upper GI study is
performed with barium to better define the anatomy and
distinguish between web (which rarely shows a windsock
deformity in the newborn), stenosis, annular pancreas and
atresia with Y-connection of the common bile/pancreatic
duct beyond the ampulla of Vater (Fig. 10).
Malrotation and Midgut Volvulus
Intestinal malrotation represents embryologic nonrotation
or lack of complete 270 of foregut rotation around the
superior mesenteric artery (SMA) pedicle. In this condi-
J.R. Reid
Figure 10 Infant with duodenal web who presented with feeding intolerance. Upper gastrointestinal (UGI) study shows a dilated second portion of the duodenum containing a curvilinear filling defect (arrows).
25
Figure 12 Newborn with midgut volvulus who presented with bilious vomiting. Doppler ultrasound showed reversal of the SMA/
SMV (not shown) and the classic whirlpool of the SMA at the site
of midgut volvulus (arrows). (Color version of figure is available
online.)
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J.R. Reid
affected fetuses shows an abdomen full of moderately dilated anechoic loops showing hyperperistalsis (Fig. 17).
Meconium Ileus
Meconium ileus, which may be suspected prenatally, is a
result of thick succus entericus (secondary to lack of lubrication because of abnormal sodium-chloride pump) effectively
blocking the distal ileum during fetal and early neonatal life.
A patient with meconium ileus on contrast enema has cystic
fibrosis until proven otherwise. In contrast, only 15% of patients with cystic fibrosis will have meconium ileus.3 Prenatal
ultrasound typically shows dilated echogenic bowel, whereas
postnatal ultrasound demonstrates dilated loops of bowel
containing hyperechoic material with hypoperistalsis (Fig.
18).17 Recent study has shown that prenatal MRI may be a
promising imaging modality in differentiating between ileal
atresia and meconium ileus based on signal intensity of the
succus entericus on T2-weighted images. It has been reported that the signal intensity of succus entericus is very low
in atresia and intermediate in ileus on T2-weighted MR images.17 However, the differences in signal intensity throughout the small bowel on T1-weighted images must be interpreted with caution in attempting to determine levels of
bowel obstruction from prenatal MRI.18 The differential diagnosis for echogenic bowel on prenatal ultrasound includes
fetal systemic infection, chromosomal anomalies, and fetal
swallowing of blood early in gestation from occult placental
intra-amniotic hemorrhage. A majority of affected patients
have abdominal radiographs that are consistent with low in-
27
Figure 18 Prenatal ultrasound image shows echogenic bowel (arrows) in a fetus with meconium ileus.
28
Figure 19 Newborn with bilious vomiting and abdominal distension. Image from water-soluble enema shows microcolon. There is
filling of the appendix and multiple filling defects are encountered
in the terminal ileum (arrow) without abrupt cutoff to suggest ileal
atresia. With persistence, there was filling of dilated meconiumfilled ileum beyond the level of obstruction (not shown); the enema
is both diagnostic and therapeutic in patients with meconium ileus.
twice that of serum osmolarity) are often necessary to completely evacuate the ileum.6
Hirschsprung Disease
Failure of craniocaudad migration of ganglion cells to the
submucosal (Meiners) and intermuscular (Auerbachs)
plexus of the distal large bowel results in failed relaxation of
the involved distal colonic segment of bowel with upstream
obstruction in Hirschsprung disease (HD). Histology will
show positive acetylcholinesterase staining and the presence
of thickened dysplastic nerves in the biopsy specimen.6
There is a 4:1 male-to-female ratio in HD.6 The disease involves a short segment of the rectosigmoid in 90% but can
involve longer segments proximal to this level in a continuous fashion.6 Ultrashort and longer-segment disease is less
common than short-segment HD. Total colonic HD is the
rarest form, with an equal incidence demonstrated in male
and female patients.6 HD has no racial predilection. However, total colonic disease is more common in white patients
and associated with trisomy 21 (in 2%-16%), hydronephrosis, and dysplastic kidneys.6 Total colonic HD may be part of
a systemic neurocristopathy with central hypoventilation
(Ondines curse) and multifocal neuroblastoma.21
Children with HD present with failure to pass meconium
within the first 24 hours of birth and progressive abdominal
distension. The most serious complication related to delay in
diagnosis is Hirschsprung enterocolitis, where there is transmural inflammatory ischemia and inflammation of the colon
prone to perforation. Imaging of HD begins with abdominal
radiographs that show a low intestinal obstruction pattern.
J.R. Reid
Figure 21 Three-week-old infant with HD who presented with progressive abdominal distension and failure to pass meconium at
birth. Lateral image from water-soluble enema shows an abnormal
rectosigmoid index where the rectum (white arrow) is narrower
than the sigmoid (black arrow) suspicious for Hirschsprung disease.
Rectal biopsy confirmed the diagnosis of HD.
29
Figure 22 Infant with small left colon syndrome and a diabetic mother;
the infant presented with feeding intolerance. Image from water-soluble enema shows normal rectal caliber with relative narrowing of the
sigmoid and distal descending colon (arrows) with abrupt transition to
normal caliber at the proximal to mid descending colon (arrowhead).
Colonic Atresia
Colonic atresia, like jejunal and ileal atresia, is thought to be
caused by a prenatal vascular event resulting in an ischemic
obliteration of the colonic lumen.6 There is no gender or
racial predilection.24 This is the rarest form of intestinal atresia and has no known associations.24 Newborns with colonic
atresia usually present with progressive abdominal distension and vomiting. Abdominal radiographs often show a
nonspecific, low intestinal obstruction pattern; however, in
some cases there is characteristic massive distension of the
cecum above a competent ileocecal valve. Water-soluble enema demonstrates a microcolon (unused colon) that terminates blindly at the point of atresia (Fig. 24).
Imperforate Anus
The incidence of imperforate anus is 1-4 in 5000 live
births and is equal in frequency in boys and girls.2 This
disorder is along the spectrum of cloacal malformation,
whereby there is a blind-ending rectum and a fistula to the
Figure 23 Large meconium plug evacuated during water-soluble enema for neonatal small left colon syndrome. (Color version of figure
is available online.)
J.R. Reid
30
Conclusions
Many cases of newborn intestinal obstruction now arrive
with a preliminary diagnosis determined from prenatal ultra-
References
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