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DOI 10.1007/s00247-012-2573-1
REVIEW
Received: 22 June 2012 / Revised: 21 October 2012 / Accepted: 29 October 2012 / Published online: 12 December 2012
# Springer-Verlag Berlin Heidelberg 2012
Abstract Intestinal obstruction is common in newborns, include surgical and non-surgical causes ranging from the
and the radiologist plays a critical role in the care of these benign and nearly universal gastroesophageal reflux (GER)
children. Diagnosing and managing the potentially to life-threatening midgut volvulus [1]. In between the be-
obstructed newborn can be challenging, especially given nign and the life-threatening are myriad etiologies of vomit-
the myriad underlying pathologies that range from benign ing that produce different patterns on abdominal
to acutely life-threatening. A familiarity with the most com- radiographs, contrasted upper gastrointestinal examination
mon diagnoses is essential, but equally important to the (UGI), and contrast enema. A familiarity with these patterns
radiologist is a systematic approach to management of the allows triage of cases that can be managed medically, those
child in this setting. We propose an approach based on the requiring elective surgery and those requiring emergent
recognition of eight radiographic patterns, five upper gas- surgery.
trointestinal examination (UGI) patterns and four contrast We present a pattern-based approach to the workup and
enema patterns. Recognition of these patterns directs further management of the vomiting newborn. Through identifica-
imaging when necessary and allows triage of children who tion of eight patterns on abdominal radiograph, five patterns
can be managed medically, those requiring elective or ur- on UGI and four patterns on contrast enema, the radiologist
gent surgery and those requiring emergent surgery. can competently and confidently manage this common and
potentially life-threatening clinical presentation.
Keywords Newborn . Neonatal . Obstruction .
Gastrointestinal
Clinical presentation
The bilious or non-bilious character of the vomiting is not Double bubble (Fig. 1)
a reliable discriminator for the presence of midgut volvulus.
Most neonates with bilious emesis do not, in fact, have The true double bubble—two upper abdominal gas bubbles,
midgut volvulus [2]. The presence of bile simply reflects one on each side of the midline, and no distal gas—can be
that the obstruction is below the ampulla of Vater. For considered diagnostic of duodenal atresia (Fig. 2) [9]. A
instance, the vomiting in 70% of cases of duodenal atresia UGI is unnecessary, both because this pattern is highly
is bilious [3], and in distal (ileal and colonic) obstructions specific for duodenal atresia and because contrast (barium
bilious vomiting [4] typically develops over hours to days. or iodinated) will not characterize the abnormality any better
Meconium passage can provide additional clues to diag- than does air.
nosis. Normal newborns pass meconium in the first 24 h of Because the obstruction caused by duodenal atresia is
life. Non-passage of meconium suggests a complete distal already long-standing at birth, the proximal duodenum is
obstruction. Infants with Hirschsprung disease or functional markedly dilated. This is in contradistinction to midgut
obstructions might pass small amounts of meconium [5]. volvulus, an acute obstruction that does not distend the
Similarly, the surgeon’s physical exam is not always a duodenum significantly.
reliable discriminator. Even in cases of midgut volvulus, the The treatment for duodenal atresia is elective surgical
abdomen is typically soft and non-tender until ischemia devel- repair. The critical question, however, is whether this pattern
ops [2]. These relatively benign symptoms and physical exam warrants any concern for midgut volvulus, which would
findings prior to the onset of catastrophic bowel infarction necessitate emergent, rather than elective, surgery. There is
justify a high index of suspicion and explain the central considerable debate in the literature concerning whether
importance of the radiologist in managing the vomiting infant. midgut volvulus needs to be excluded in the setting of a
With near-universal utilization of prenatal US screening, newborn with a double bubble, because of concern that
many congenital bowel obstructions are detected in utero midgut volvulus could either produce a double bubble pat-
[6]. Duodenal atresia, as manifested by a fluid-filled double tern [10] or complicate duodenal atresia [11]. Some believe
bubble plus polyhydramnios, is more reliably detected than such concern is unwarranted [12, 13]. What is undeniable is
duodenal stenosis, malrotation and distal obstructions [7]. that if air is not passing distal to the duodenum, neither will
Pitfalls in the diagnosis of congenital obstructions are well contrast material; a UGI would not rule out malrotation.
documented and necessitate a post-natal imaging workup
before surgery is contemplated [6]. The single bubble (Fig. 1)
Fig. 1 The eight radiographic patterns. a Normal. Gas is distributed level of the iliac crests. To assign this pattern with confidence, the third
uniformly throughout non-dilated stomach and bowel. The L1 inter- gas bubble must be contiguous with the more superior bubbles. e
pedicular distance can be used as an internal standard for the upper Single bubble with distal gas. Gas is present in the stomach,
limits of normal for bowel distension. b The double bubble. This which is sometimes distended. There is also a small amount of
pattern consists of two large rounded lucencies (bubbles) in the upper gas distal to the stomach in a limited number of decompressed
abdomen, with no distal gas. The left-side bubble is larger and more bowel loops. f Double bubble with distal gas. This pattern implies
superiorly located. The right-side bubble is slightly smaller but clearly the presence of gas distal to an otherwise classic double bubble
distended, with its epicenter to the right of the spine. c The single pattern. Distal gas is variable in amount, but distal loops are
bubble. Gas is limited to the stomach. The stomach may be distended typically decompressed. g Diffusely dilated loops. In this pattern,
or may be decompressed by an enteric tube or vomiting. The key to dilated, non-separated bowel loops fill the distended abdomen and
recognizing this pattern is the absence of any gas distal to the stomach. extend into the pelvis. h Dilated, scattered bowel loops. This
d Triple bubble. There are three lucencies in the upper abdomen. The pattern is characterized by bowel loops that are mildly dilated
third bubble is dilated and is often oriented horizontally. There is no but noncontiguous, featureless in appearance and scattered ran-
gas distal to the three large bubbles, which rarely extend below the domly throughout the abdomen and pelvis
duodenum and a jejunal bowel loop just proximal to the be caused by failure of recanalization [3] and is therefore
atretic segment (Fig. 4). One might argue that the con- typically an isolated atresia.
fident recognition of this pattern obviates UGI examina-
tion, but if all three loops are not distended, this pattern Single bubble with distal gas (Fig. 1)
is easily confused with other radiographic patterns that
necessitate UGI to exclude malrotation and midgut Gastric distention out of proportion to non-distended
volvulus. distal bowel loops is a very common and typically
Many surgeons also request a contrast enema to exclude benign finding at any age, including the neonatal peri-
an additional colonic atresia (which would change surgery) od. However, in the setting of bilious vomiting, this
or meconium ileus (which might obviate surgery). A search pattern must be considered as ominous, as it is well
for additional atresias is reasonable because jejunal atresia is described in association with midgut volvulus [8]. Even
thought to be most often secondary to a vascular insult [16], though midgut volvulus obstructs distal to the gastric
which can cause more than a single atresia. This is in outlet, the obstruction is too acute to dilate the duode-
contradistinction to duodenal atresia, which is thought to num appreciably, and so the pattern frequently suggests
Pediatr Radiol (2013) 43:318–329 321
Fig. 4 Triple bubble in a newborn girl who had abnormal prenatal US.
Fig. 2 Double bubble in a newborn boy intolerant of first feed.
Initial radiograph demonstrates three rounded upper abdominal lucen-
Abdominal radiograph demonstrates dilated stomach and duodenum
cies and no distal gas. Elective surgery confirmed jejunal atresia
with no distal gas. Elective surgery was performed without additional
imaging and confirmed duodenal atresia
a gastric outlet obstruction. This pattern should trigger Ultimately, the vomiting infant with a double bubble
UGI examination in this clinical setting. needs surgery, whether there is distal gas or not. It is the
radiologist’s role to assist the surgeon in determining wheth-
Double bubble with distal gas (Fig. 1) er the surgery is elective/urgent or emergent. This is deter-
mined with the help of UGI.
Whether one is dealing with a single or double bubble, the
presence of even a tiny amount of distal gas is a critical Diffusely dilated loops (Fig. 1)
finding that alters the differential diagnosis and necessities
UGI examination. It can be impossible to distinguish colon from small bowel
In the setting of the double-bubble pattern, the identifi- in infants, particularly when the bowel is distended. The
cation of distal gas (Fig. 5) virtually excludes duodenal appearance of multiple (four or more) dilated (wider than
atresia (exception being the rare case of the bifid common the L1 interpedicular distance) loops of bowel throughout
bile duct inserting above and below the atretic segment) [17] the abdomen and extending into the pelvis (Fig. 6) is sug-
and widens the differential diagnosis to include midgut gestive of a low obstruction, typically at the level of the
volvulus, along with duodenal stenosis/web. ileum or colon. Contrast enema is indicated to evaluate
Fig. 6 Pattern of diffusely dilated loops. This girl, born at 24 weeks’ Fig. 7 Pattern of dilated scattered bowel loops on abdominal radio-
gestational age, had become progressively distended over the first 72 h graph obtained in a 1-day-old girl with bilious vomiting. Based on
after birth. The abdominal radiograph demonstrates diffusely dilated physical exam findings, the girl was taken directly to surgery, where
bowel caused by a functional obstruction of prematurity midgut volvulus was found
Pediatr Radiol (2013) 43:318–329 323
in the workup of the vomiting newborn. Clearly, the most possible non-surgical causes [20], the more common causes
immediate goal of the UGI examination is to exclude malro- being gastroesophageal reflux and sepsis.
tation with midgut volvulus. The radiologist must be aware of
the various appearances of malrotation and midgut volvulus Malposition of the duodenojejunal junction (Fig. 8)
but otherwise should not approach the UGI examination feel-
ing the necessity to make a specific diagnosis. Rather, the Given malposition of the duodenojejunal junction (DJJ) in a
radiologist’s goal in performance of the UGI in this setting vomiting infant (Fig. 9), one must assume that the vomiting
should be to categorize the vomiting infant into one of three is from intermittent midgut volvulus. While that is not
groups, based on clinical management: (1) those requiring always the case, and should not necessarily be assumed in
emergent surgery, (2) those requiring elective surgery and the older child or in the absence of vomiting, this finding is
(3) those who can be treated non-surgically. an indication for emergent surgery in the infant with bilious
We propose that the recognition of the following five vomiting. We acknowledge that the differentiation between
patterns on the UGI series narrows the differential diagnosis a malpositioned and a normally positioned DJJ can be
and allows assignment into one of the three management subtle, particularly in the setting of ileus or distal bowel
paths (Fig. 8). obstruction, which can displace the normal DJJ because of
ligamentous laxity and result in a false-positive UGI [21].
We refer the reader to excellent reviews of this topic [22].
Significance of each of the five UGI patterns
Corkscrew duodenum (Fig. 8)
Normal (Fig. 8)
This pattern (Fig. 10) is diagnostic of midgut volvulus
A normal UGI excludes structural causes of vomiting. Vom- and requires emergent surgery with detorsion and Ladd
iting is presumed secondary to any of the large number of procedure [23].
Fig. 8 The five patterns on UGI. a Normal. A normal UGI demon- narrowing or spiraling. c Corkscrew duodenum. The duodenum spirals
strates prompt gastric emptying, a normal-caliber duodenum, and a inferiorly without crossing to the left of the bony spine. d Complete
normally positioned DJJ. Normally, the duodenum crosses to the left of duodenal obstruction. Contrast material does not pass beyond the mid-
the left vertebral body pedicle and rises to the level of the duodenal duodenum. Air, however, is seen in more distal bowel loops, ruling out
bulb. On the lateral view, the duodenum remains retroperitoneal duodenal atresia. e Partial duodenal obstruction with normally
throughout its course. The radiologist should use the lateral view to positioned DJJ. This pattern is assigned when a duodenal narrow-
ensure the entire duodenum remains posterior. b Malposition of the ing (and typically, dilation proximally) is seen, but sufficient
DJJ. The duodenum fails to cross the midline and ascend to its normal contrast material is able to traverse the narrowing to document a
position in the left upper quadrant. There is no duodenal dilatation, normally positioned DJJ
324 Pediatr Radiol (2013) 43:318–329
Fig. 13 The four patterns on contrast enema. a Microcolon. The entire terminates at some point short of the cecum. c Colonic caliber change.
colon is small in luminal diameter (less than the height of an upper lumbar There is a demonstrable transition from small- or normal-caliber colon
vertebral body) and nondistensible. b Short microcolon. The colon is distally to more distended colon proximally. d Normal. The colon is of
small or “micro” (as defined above) in caliber and its retrograde filling normal and uniform caliber throughout
326 Pediatr Radiol (2013) 43:318–329
Fig. 15 Ileal atresia in an infant with microcolon who, like the infant Fig. 17 Small left colon syndrome. Image shows colonic caliber
in Fig. 14, presented with vomiting, abdominal distension and failure change at the splenic flexure (arrows) characteristic of small left colon
to pass meconium. Contrast material is refluxed into decompressed syndrome. Passage of plugs of meconium and abdominal decompres-
terminal ileum (arrows), which is devoid of meconium filling defects. sion followed within hours of this contrast enema
No contrast material could be refluxed into more proximal ileum. Mid-
ileal atresia was confirmed at surgery bowel contents from reaching the colon, leaving it narrow
and non-distensible, i.e. a microcolon [25].
The two most common causes of high-grade distal ileal
Significance of the four contrast enema patterns obstruction leading to this pattern are meconium ileus and
ileal atresia. Meconium ileus is a bowel obstruction caused
Microcolon (Fig. 13) by the inspissation of abnormal meconium in the distal
ileum. Most children with meconium ileus have cystic fi-
Normally, the developing fetal colon is stimulated by dis- brosis [26]. Ileal atresia is caused by intrauterine vascular
tension from meconium, sloughed cells and secreted fluid. insult with subsequent reabsorption [16].
A high-grade distal ileal obstruction prevents sufficient
Fig. 19 The algorithm for the diagnostic imaging workup of the newborn with potential bowel obstruction
The two can usually be distinguished by the contrast confident that sufficient back pressure was achieved with
enema. If refluxed contrast reaches ileal loops containing the contrast enema such that the termination of the contrast-
multiple filling defects, a diagnosis of meconium ileus can filled colon truly represents an atretic segment. Correlation
be made (Fig. 14). If no reflux can be achieved beyond with the abdominal radiograph often shows a massively
small-caliber ileum devoid of filling defects, then ileal atre- dilated terminal loop just proximal to the atresia. Treatment
sia is the presumptive diagnosis (Fig. 15). This distinction is is surgical [29].
important because uncomplicated meconium ileus can be
treated with contrast enemas [27] whereas ileal atresia Colonic caliber change (Fig. 13)
requires surgery [4].
It should be noted that total colonic Hirschsprung A demonstrable transition from small- or normal-caliber
disease can also produce a microcolon appearance colon distally to more dilated colon proximally narrows
[28]. This, however, is an uncommon presentation of the differential diagnosis to two entities: Hirschsprung dis-
an uncommon entity. ease and small left colon syndrome (SLCS). Both are func-
tional obstructions. Hirschsprung disease results from
Short microcolon (Fig. 13) absence of ganglion cells that innervate the colon, and this
disease must be treated surgically [30]. SLCS is caused by a
Recognition of a short or incomplete microcolon (Fig. 16) functional immaturity of those ganglion cells, and it usually
allows a diagnosis of colonic atresia. The challenge is to be resolves after the contrast enema [31].
328 Pediatr Radiol (2013) 43:318–329
Several imaging features distinguish these two entities. familiar with the spectrum of imaging findings and common
The location of the colonic caliber change is often helpful. diagnoses and by using a systematic approach to imaging
SLCS is the presumptive diagnosis when the caliber transi- strategies, the radiologist can work with his or her surgical
tion is at the splenic flexure (Fig. 17), assuming that meco- and neonatology colleagues to provide the best care to the
nium evacuation and decompression follow the contrast neonate with bowel obstruction.
enema within 24–48 h. Hirschsprung disease is the likely
diagnosis if the transition is elsewhere in the colon (Fig. 18), Conflicts of interest None.
especially at the classic rectosigmoid level but even at the
splenic flexure in a baby whose abdominal symptoms do not
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