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Pediatr Radiol (2013) 43:318–329

DOI 10.1007/s00247-012-2573-1

REVIEW

A pattern-based approach to bowel obstruction


in the newborn
Charles M. Maxfield & Brett H. Bartz & Jennifer L. Shaffer

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

Introduction Vomiting newborns sometimes present with additional signs


and symptoms, such as feeding intolerance, abdominal disten-
The radiologist plays a crucial role in the diagnosis and sion, failure to pass meconium and peritoneal signs [2]. The
management of the vomiting newborn. This common and symptoms do not reliably discriminate the causes of vomiting,
potentially life-threatening presentation is not limited to although they can provide clues to the underlying etiology.
children’s hospitals. All radiologists must be familiar with Vomiting typically presents earlier with proximal
the workup and management of potential bowel obstruction obstructions than it does with more distal obstructions [3].
in the newborn. Etiologies of vomiting in the newborn Duodenal atresia, for example, typically presents with the
first feeding. The various causes of duodenal stenosis,
CME activity This article has been selected as the CME activity for whether intrinsic or extrinsic, present at more variable times,
the current month. Please visit the Society for Pediatric Radiology Web depending on the degree of stenosis. Distal obstructions
site at www.pedrad.org on the Education page and follow the
instructions to complete this CME activity.
(ileal and colonic) progress over the first 24–48 h of life,
often with more abdominal distension [2]. Midgut volvulus
C. M. Maxfield (*) : B. H. Bartz : J. L. Shaffer
presents more variably, 50% during the first week and 60%
Department of Radiology, Duke University Medical Center,
Box 3808, Durham, NC 27710, USA by the end of the first month [2], often with a sudden onset
e-mail: charles.maxfield@duke.edu of bilious vomiting after initially tolerating feeds.
Pediatr Radiol (2013) 43:318–329 319

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)

The true single bubble, with no gas beyond a distended


Eight patterns on radiographs: definitions stomach, is characteristic of gastric (antral or pyloric) atresia
[14]. It is important to note, however, that gastric atresia is
The postnatal imaging workup of the vomiting infant often exceedingly uncommon. More often, this pattern is character-
begins with an abdominal radiograph. We propose that ized by a stomach that is partially decompressed by vomiting
recognition of the eight patterns on the frontal supine ab- or a nasogastric tube, with no gas distally. In such cases, the
dominal radiograph shown in Fig. 1 helps narrow the dif- pattern must be considered incompletely characterized. Fre-
ferential diagnosis and, more important, triage the neonate quently, a subsequent radiograph will reveal better distension.
with bowel obstruction to the next phase of management— If not, the pattern might be fully characterized by a portable
either additional imaging or admission to the medical or radiograph obtained following insufflation of the stomach
surgical team for treatment. with approximately 10–20 ml of air through the nasogastric
tube. One of three patterns will then become manifest, two of
which establish diagnoses and obviate UGI evaluation: (1) a
Significance of the eight patterns and associated single bubble with distal gas (discussed below), which should
management prompt an emergent UGI; (2) a true double bubble, diagnostic
of duodenal atresia, an indication for elective surgery (Fig. 3);
Normal (Fig. 1) (3) a true single bubble, distended and without any distal gas, a
finding indicative of a complete gastric outlet obstruction,
A normal abdominal radiograph might seem reassuring in such as the rare gastric atresia.
this setting until one realizes that many cases of midgut
volvulus present with a normal abdominal radiograph [8]. Triple bubble (Fig. 1)
If the vomiting is bilious or if there is another reason to
suspect malrotation with midgut volvulus, the workup If confidently identified, this pattern is indicative of
should proceed to UGI. jejunal atresia [15], with dilation of the stomach,
320 Pediatr Radiol (2013) 43:318–329

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. 3 Duodenal atresia initially


presenting as a single bubble. a
Initial abdominal radiograph
obtained after newborn girl
vomited first feed demonstrates
no air distal to a decompressed
stomach. b The subsequent
radiograph demonstrates
gaseous distension of stomach
and distended duodenum, with
no distal gas. Notice the
nasogastric tube had migrated
out of the stomach, allowing the
distension, and demonstration of
the classic double bubble pattern
322 Pediatr Radiol (2013) 43:318–329

suggests meconium ileus [18]. Alternatively, multiple dilat-


ed loops with a single disproportionately distended loop of
bowel suggest a distal atresia, often colonic [4]. These clues
do not obviate a contrast enema, however.
If the infant with this radiographic pattern is very ill with
bilious vomiting and peritoneal signs, midgut volvulus
should be considered. In this rare case, the diffuse dilation
is not secondary to obstruction but rather to an ileus sec-
ondary to ischemia or infarction [19]. It is therefore impor-
tant in this setting that a normal contrast enema be followed
immediately by UGI to exclude midgut volvulus.

Dilated, scattered bowel loops (Fig. 1)

Whether encountered in the infant, the older child or the adult,


this pattern can be recognized by the experienced radiologist
as that of a “sick belly” (Fig. 7). The loops are dilated but
Fig. 5 Double bubble with distal gas. This infant boy had vomited
since birth, prompting this abdominal radiograph, which demonstrates noncontiguous, featureless in appearance, and scattered ran-
two rounded upper abdominal lucencies, with additional gas in non- domly throughout the abdomen and pelvis. This pattern can be
dilated distal small bowel loops. UGI (not shown) demonstrated a seen with distal obstruction (such as complicated meconium
partial duodenal obstruction and normally positioned DJJ. A duodenal ileus), proximal obstruction (midgut volvulus) and in utero
web was found at surgery 1 month later
bowel perforation. The radiologist should be prepared to
perform UGI and contrast enema before surgery.
further. A contrast enema is usually diagnostic and can be
therapeutic in the case of meconium ileus.
Radiographs demonstrating this pattern sometimes pro- Five UGI patterns
vide additional clues to a specific diagnosis. For instance, a
soap bubble appearance, or absence of air-fluid levels, At least four and as many as six of the eight radiographic
patterns presented should prompt UGI as the next examination

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. 9 Duodenojejunal malposition in a 3-day-old. UGI, performed


after a single episode of bilious vomiting, demonstrates an abnormal
duodenum that fails to cross the midline or ascend to the level of the
duodenal bulb. The infant was sent emergently to surgery, where
malrotation was confirmed. There was no midgut volvulus at the time
of surgery

Complete duodenal obstruction (Fig. 8)

Like the corkscrew duodenum, this pattern indicates the


need for emergent surgery. The appearance is strongly sug-
gestive of midgut volvulus, perhaps resulting from a tighter
twist than would produce the corkscrew pattern (Fig. 11).
Duodenal atresia can be excluded on the basis of air distal to
the duodenum and by the fact that the proximal duodenum is
not markedly dilated.

Fig. 11 Midgut volvulus in a 4-day-old girl. a Abdominal radiograph


demonstrates a mildly distended stomach, with gas seen distally in non-
dilated bowel (single bubble with distal gas). b Single image from UGI
examination demonstrates a complete obstruction to contrast agent at the
level of the proximal duodenum (arrow). The stomach is distended with
air. The girl was sent emergently to surgery with the presumptive diag-
nosis of midgut volvulus, which was confirmed at surgery

This pattern requires patience by the radiologist. Time must


be allowed for contrast agent to pass the mid-duodenum and
reach the DJJ. It is only with the failure of contrast agent to pass
that this pattern can be assigned. Recognition of this pattern
necessitates emergent surgery with the presumptive diagnosis
of midgut volvulus.

Partial duodenal obstruction with normally positioned DJJ


(Fig. 8)
Fig. 10 Corkscrew duodenum in an infant girl who developed bilious
vomiting at 2 days old. UGI demonstrates spiraling of the duodenum
A persistent duodenal narrowing demonstrated on UGI is
over the bony spine. Based on this finding, the girl was sent directly to typically a surgical lesion in a vomiting infant. The narrow-
surgery, where midgut volvulus was confirmed ing can be extrinsic (Ladd band, annular pancreas) or
Pediatr Radiol (2013) 43:318–329 325

Fig. 12 Partial duodenal obstruction with normal DJJ in a 3-week-old


girl with congenital heart disease had persistent non-bilious vomiting.
Single image from UGI, with contrast injected through an enteric tube
placed in the proximal duodenum, demonstrates a focal narrowing of
the descending duodenum (arrow). Contrast agent passes distally to
document normal position of the DJJ (arrowhead), allowing elective
surgery. At surgery, an annular pancreas narrowed the descending
duodenum Fig. 14 Meconium ileus in an infant with microcolon who presented
with vomiting, abdominal distension and failure to pass meconium.
Note caliber of colon is less than the height of an upper lumbar
vertebral body. Contrast material is refluxed from a microcolon into
terminal ileal loops (arrows) outlining multiple filling defects indica-
intrinsic (duodenal web, duodenal stenosis) to the duo- tive of meconium ileus. Obstruction was successfully relieved with
denum [24]. It is not always possible—or necessary— contrast enema. Note also reflux into the appendix (arrowhead)
for the UGI to distinguish the cause of the partial
obstruction. It is important only that the UGI distin- Four patterns on contrast enema
guish malrotation (with Ladd band or midgut volvulus)
from the other causes of partial duodenal obstruction. The radiographic pattern of multiple, diffusely dilated bowel
This is best done by patiently waiting for contrast agent loops in the setting of a newborn who is vomiting and has
to pass through the stenotic area and document the not passed meconium suggests a low bowel obstruction and
position of the DJJ. should prompt a contrast enema.
If a normally positioned DJJ can be documented (Fig. 12), The radiologist encounters one of four patterns in perfor-
malrotation is excluded and surgery can be performed elec- mance of a contrast enema in this setting. Each pattern offers
tively rather than emergently, at a time when the child can be a very limited differential diagnosis, which accounts for
optimally prepared for surgery. If a normal DJJ position about 98% of cases, and allows appropriate management
cannot be documented, then emergent surgery is indicated. decisions, which might be surgical or nonsurgical (Fig. 13).

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. 16 Short microcolon, diagnostic of colonic atresia. Single frontal


image from contrast enema performed in a newborn with progressive Fig. 18 Hirschsprung disease. Image shows colonic caliber change in
abdominal distension over the first 2 days after birth. Contrast agent the proximal sigmoid colon (arrows) in a 5-day-old boy, demonstrated
opacifies a small-caliber colon but could be refluxed no further prox- on a lateral view from a contrast enema. Rectal biopsy confirmed
imal than splenic flexure (arrow) Hirschsprung disease
Pediatr Radiol (2013) 43:318–329 327

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
resolve after contrast enema. References
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