ORIGINAL ARTICLE: GASTROENTEROLOGY

Ultrasonographic Diagnosis of Midgut Volvulus

With Malrotation in Children


Francesco Esposito, yValerio Vitale, zDomenico Noviello, §Marco Di Serafino,
y
Gianfranco Vallone, yMarco Salvatore, and
Patrizia Oresta

ABSTRACT
(SMA) axis in patients with malrotation. During fetal development,
Objectives: Midgut volvulus with malrotation is typical in newborns. We
the primary intestinal loop is placed on the sagittal median plane
present our experience to emphasize the importance of suspecting midgut
fixed to the posterior abdominal wall by a common mesentery, in
volvulus as a cause of abdominal pain also beyond infancy, particularly in
which is localized the SMA that divides the main loop in a
relation to malrotation, and the relevance of ultrasonographic (US) signs in
prearterial loop segment (from which originate the jejunum and
its diagnosis.
ileus) and a postarterial segment (from which originate terminal
Methods: A total of 34 patients (10 boys, 24 girls; ages between 1 day and
ileus, cecum, ascending colon, and transverse colon). The SMA
12 years) diagnosed as having malrotation or malrotation with volvulus,
forms the rotation axis of the intestinal loop. Following tilting of the
surgically confirmed, between 2006 and 2013 were retrospectively selected
dorsal mesentery, rapid elongation of the intestine after week 5,
among all of the patients referred to our institution for acute abdomen and
combined with rapid growth and expansion of the liver, results in
bilious vomiting. All of them underwent US and color Doppler examination
temporary herniation of the intestinal loops of the midgut into the
before surgery. The US examinations were performed with 6 to 10 MHz
umbilical cord. Between 5 and 10 weeks, the intestinal loop takes an
microconvex and 7.5 to 10 Mhz linear transducer. The transducer was placed
axial position after a counterclockwise 908 rotation. In this phase,
under xiphoid, and, with axial projection, the presence of anatomic position
the prearterial segment is on the right of the median line and SMA,
reversed between superior mesenteric vein (SMV) and superior mesenteric
and the postarterial segment is left sided. Between 10 and 11 weeks’
artery (SMA), as a sign of malrotation, and the presence of ‘‘whirlpool sign’’
gestation, the primary intestinal loop takes a 1808 rotation around
(WS) (wrapping of the SMV and the mesentery around the SMA), as a sign
the SMA axis and returns inside the abdominal cavity.
of midgut volvulus, were evaluated.
At first, prearterial segment and jejunum take position in the
Results: In 27 of these 34 patients, midgut volvulus was present; 7 patients
left abdomen, after postarterial segment on the right side. In the
had intestinal malrotation. In 2 of 7 (28%) patients with malrotation, SMA
beginning, the cecum is ahead of the SMA and intestinal loops; after
and SMV were inverted. Among the patients with volvulus, 2 showed
its rotation movement, it gets positioned below the liver and finally
reversed vessel position and 22 patients presented the WS in association with
in left iliac fossa.
SMA/SMV inversion (22/27, 81%).
A failure in 1 phase of the normal counterclockwise rotation
Conclusions: Midgut volvulus with malrotation can appear beyond the
leads to a ‘‘malrotation.’’ In the case of a rotation less than 2708,
neonatal age group as demonstrated in our case. The WS sign is sufficiently
duodenal–jejunum junction is localized near the cecum, with a
sensitive for its diagnosis and should be routinely researched at all ages of
shortening of the mesenteric root (1). In this situation, there is an
pediatric population. Anatomic inversion between SMV and SMA seems to
increased risk of midgut volvulus and secondary ischemia with, in
be not enough sensitive in isolated malrotation diagnosis.
untreated patients, focal necrosis of the intestinal loops (2). Thus, an
Key Words: malrotation, midgut volvulus, ultrasonography early diagnosis is important to proceed with surgical treatment.
Malrotation or volvulus is usually present in newborn and infants,
(JPGN 2014;59: 786–788) but it can also be diagnosed in children and adults (3,4). Clinical
symptoms are aspecific, such as bilious vomiting, abdominal ten-
sion, and, in some patients, blood in feces (2–5). Imaging has an

I ntestinal malrotation, or as it is more correctly known as

‘‘incomplete rotation,’’ results from an abnormal intestinal
rotation and fixation during fetal development; midgut volvulus
is a complete small bowel torsion along superior mesenteric artery
Received and accepted July 7, 2014.
From the
Department of Radiology, ‘‘Santobono-Pausilipon’’ Hospital,
the yDepartment of Radiology, University Hospital ‘‘Federico II,’’ the
zSection of Ultrasonography and Interventional Ultrasound, ‘‘D.
Cotugno’’ Hospital, Naples, and the §Department of Radiology, Santa
Maria alla Gruccia Hospital, Arezzo, Italy.
Address correspondence and reprint requests to Dr Francesco Esposito,
Santobono Hospital, Via M. Fiore, 6, 80100 Naples, Italy (e-mail:
fra.in@libero.it).
The authors report no conflicts of interest.
Copyright # 2014 by European Society for Pediatric Gastroenterology,
Hepatology, and Nutrition and North American Society for Pediatric
Gastroenterology, Hepatology, and Nutrition
DOI: 10.1097/MPG.0000000000000505
important role in the diagnosis of malrotation. In the early days,
barium enema had a primary role in the evaluation of abnormal
cecum position to make a diagnosis of malrotation (6). Later,
radiographic study of the upper gastrointestinal tract was used to
evaluate duodenum morphology and duodenal–jejunum junction
position (7). Since the 1980s, ultrasonographic (US) study, B-mode
and then color Doppler (CD), is used to identify indirect signs of
malrotation and volvulus, such as mesenteric vessel reversed
position and ‘‘whirlpool sign’’ (WS) (8,9). The ‘‘superior mesen-
teric vein (SMV) malrotation’’ sign refers to the abnormal posi-
tioning of the SMV on the left ventral aspect of the SMA instead of
its normal right-sided position. As the malrotation becomes tighter
and leads to volvulus, the WS can be detected, the typical pattern of
SMA wrapped by coils of SMV and bowel (2,5,9,10).
METHODS
A total of 34 patients (10 boys, 24 girls; ages between 1 day
and 12 years) diagnosed as having malrotation or malrotation with

786 JPGN  Volume 59, Number 6, December 2014

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 JPGN  Volume 59, Number 6, December 2014 Ultrasonographic Diagnosis of Midgut Volvulus With Malrotation

volvulus, surgically confirmed, between 2006 and 2013 were retro-

spectively selected among all of the patients referred to our institu-
tion for acute abdomen and bilious vomiting.
US and CD examinations were performed with 6 to 10 MHz
microconvex and 7.5 to 10 MHz linear transducer (Esaote MyLab
Twice, Genova, Italy). The transducer was placed under xiphoid,
and, with axial projection, the SMA was identified from its aortic
origin and the SMV from its portal origin. So the presence of their
abnormal reversed position, as a sign of malrotation (Fig. 1A and
B), and the presence of WS (Fig. 2), as a sign of midgut volvulus,
were evaluated; B-mode study was integrated with CD in the
vascular examination (Fig. 3).

RESULTS
In 27 of these 34 patients, both malrotation and midgut
volvulus were present; 7 patients had isolated intestinal malrota-
tion. In 2 of 7 (28%) patients with malrotation, the SMA and the
SMV were inverted. Among patients with volvulus, 2 showed
reversed vessel position, 22 patients presented the WS in associ-
ation with SMA/SMV inversion (22/27, 81%), and 3 patients had a
false-negative US with no SMA/SMV inversion or the WS
(Table 1).
FIGURE 2. Midgut volvulus: transverse view shows twisting of the
mesentery around the superior mesenteric artery (arrow) in a ‘‘whirl-
pool sign’’ pattern.
DISCUSSION
Midgut volvulus is the most frequent cause of acute abdomen
in newborns, and it is a common consequence of intestinal mal-
rotation (11). Nevertheless, it could affect children also (12,13), as
confirmed in our series. Few cases of US findings beyond infancy
were reported, to our knowledge (4,5,11,14–17).
Early diagnosis is important in this disease, to avoid the risk
of intestinal infarct and necrosis. Imaging diagnosis of abnormal
intestinal rotation and fixation has been modified in the last several
years. Initially, barium enema had a primary role in the evaluation
of abnormal cecum (1,6). Later, radiographic study of the upper
gastrointestinal tract was used to evaluate duodenum morphology
and duodenal–jejunum junction position, and it is still considered
the criterion standard (1,7,10,18). Since the 1980s, in addition to
these invasive examinations that use x-rays, ultrasound examination
with CD is used to identify indirect signs of malrotation and
volvulus (8,9,19), such as distal duodenum obstruction, SMA
pulsatility, and duodenal wall thickening with peritoneal fluid
(8,15,20,21). Yousefzadeh (22) suggested the US evaluation of
the third portion of the duodenum to prove its retromesenteric
localization. Nevertheless, it is often difficult to evaluate correctly
the third portion of the duodenum for the interposition of intestinal
bloating (especially in patients with a high intestinal obstruction),

FIGURE 1. Sonographic findings: transverse view. A, Normal vascular

relation between superior mesenteric vein (arrow) and superior
mesenteric artery (arrowhead). B, Midgut malrotation. Reversal in
the position of the mesenteric vessels: the superior mesenteric vein
(arrow) is on the left of the superior mesenteric artery (arrowhead).
Note the dilated descending duodenum (
).
FIGURE 3. Whirlpool sign. The color Doppler shows a spiraling course
of the superior mesenteric vein. Surgical findings confirmed midgut
malrotation and volvulus.

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 Esposito et al
TABLE 1. Surgical and sonographic findings in the examined patients
SMA/SMV WS þ SMA/SMV None Total
Malrotation 2 — 5 7
Malrotation þ volvulus 2 22 3 27
SMA ¼ superior mesenteric artery; SMV ¼ superior mesenteric vein;
WS ¼ whirlpool sign.
so we focused on mesenteric vessel reversed positioning and the
WS, the typical pattern of SMA wrapped by coils of SMV and
bowel (2,5,9,10,23), because we consider its research more easily
executable in a hospital emergency department. Although reversed
AMS/SMV position is frequently recognized in patients with mal-
rotation and volvulus at US examination, this sign is not considered
specific. Indeed, approximately one-third of the affected patients
have a normal vessel anatomic position (5,10,12,24). Nevertheless,
the sensitivity of this sign is even lower in our series. Instead, the
WS seems to have a high sensitivity and specificity for the diagnosis
of volvulus (5,25); however, we found that in the literature, a clear
difference between malrotation and volvulus as a consequence of
intestinal malrotation was not made, so we divided the 2 patient
groups’ analysis.
In effect, reversed SMA/SMV position showed a low sen-
sitivity (28%) in isolated malrotation without volvulus diagnosis, in
our series; instead, it appeared more reliable, if associated with the
WS, in the diagnosis of volvulus in association with malrotation
(81%). It is not clear why the reversed SMA/SMV position is
present only in some patients with malrotation, although there are
some theories about the intestinal tract embryonal development
(1,5). It could be that patients with intestinal malrotation without
reversed position of the cranial tract of mesenteric vessels could
have an inversion of their distal tract that is not identified with US
examination (11). Noninvasiveness, absence of x-rays, and low
costs could make US examination a useful screening test in patients
with suspected intestinal malrotation; however, malrotation and
volvulus are fatal diseases, so a valid screening test should not have
false-negatives. Although in the case of reversed mesenteric vessel
positioning an isolated malrotation should be suspected, this sign
does not have a good sensitivity in isolated malrotation without
volvulus diagnosis, so upper gastrointestinal radiographic examin-
ation is considered the criterion standard in the suspicion and
diagnosis of this pathological entity (11,20). Instead, the WS shows
a discrete sensitivity and a high specificity in the diagnosis of
volvulus associated with malrotation (4,5,11,25). In our series, 81%
of patients with malrotation and volvulus, surgically confirmed, had
a SMA wrapped by coils of SMV and bowel. US examination with
CD allows an optimal visualization of the abdominal vessels and
WS, demonstrating the spiral aspect of SMV around SMA.
In conclusion, midgut volvulus should always be considered
in the differential diagnosis of abdominal pain and vomiting in the
pediatric population. Whirlpool US sign could be considered
pathognomonic in midgut volvulus diagnosis (5,25), and we want
to emphasize the importance of routinely evaluating its presence at
all ages in the pediatric population, not only in the infancy stage, as
demonstrates our series. Upper gastrointestinal radiographic exam-
ination is still the criterion standard in isolated malrotation diag-
nosis when it is suspected, despite a negative US examination.
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JPGN  Volume 59, Number 6, December 2014
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