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Pediatr Radiol (2014) 44:535541

DOI 10.1007/s00247-014-2873-8

ORIGINAL ARTICLE

Perforated appendicitis: an underappreciated mimic


of intussusception on ultrasound
Beverley Newman & Matthew Schmitz & Rakhee Gawande &
Shreyas Vasanawala & Richard Barth

Received: 17 October 2013 / Accepted: 6 January 2014 / Published online: 26 January 2014
# Springer-Verlag Berlin Heidelberg 2014

Abstract
Background We encountered multiple cases in which the US
appearance of ruptured appendicitis mimicked intussusception, resulting in diagnostic and therapeutic delay and multiple
additional imaging studies.
Objective To explore the clinical and imaging discriminatory
features between the conditions.
Materials and methods Initial US images in six children (age
16 months to 8 years; 4 boys, 2 girls) were reviewed independently and by consensus by three pediatric radiologists. These
findings were compared and correlated with the original reports and subsequent US, fluoroscopic, and CT images and
reports.
Results All initial US studies demonstrated a multiple-ringlike appearance (target sign, most apparent on transverse
views) with diagnostic consensus supportive of intussusception. In three cases, US findings were somewhat discrepant
with clinical concerns. Subsequently, four of the six children
had contrast enemas; two were thought to have partial or
complete intussusception reduction. Three had a repeat US
examination, with recognition of the correct diagnosis. None
of the US examinations demonstrated definite intralesional
lymph nodes or mesenteric fat, but central echogenicity
caused by debris/appendicolith was misinterpreted as fat. All
showed perilesional hyperechogenicity that, in retrospect, represented inflamed fat walling off of the perforated appendix.
There were four CTs, all of which demonstrated a double-ring
appearance that correlated with the US target appearance, with
B. Newman (*) : M. Schmitz : R. Gawande : S. Vasanawala :
R. Barth
Department of Radiology, Stanford University,
Lucile Packard Childrens Hospital, 725 Welch Road, MC 5913,
Stanford, CA 94305, USA
e-mail: bevn@stanford.edu

inner and outer rings representing the dilated appendix and


walled-off appendiceal rupture, respectively. All six children
had surgical confirmation of perforated appendicitis.
Conclusion Contained perforated appendicitis can produce
US findings closely mimicking intussusception. Clinical correlation and careful multiplanar evaluation should allow for
sonographic suspicion of perforated appendicitis, which can
be confirmed on CT if necessary.

Keywords Children . Appendicitis . Intussusception .


Ultrasound . Computed tomography

Introduction
Acute appendicitis and ileocolic intussusception are common
causes of acute abdominal pain in young children. Timely and
accurate diagnosis of these conditions is essential for prompt,
appropriate treatment and minimization of patient morbidity.
Imaging evaluation of abdominal pain in children often
begins with a sonogram of the abdomen and pelvis [1]. The
typical sonographic appearances of acute appendicitis and
intussusception have been well illustrated [24]. Several potential mimics of intussusception on imaging have been described [1, 5, 6]. Little, however, has been written regarding
the confusion of the sonographic appearances of ruptured
appendicitis and intussusception. We consider ruptured appendicitis to be an important mimic of intussusception, with
potential for delayed diagnosis or misdiagnosis. We also suggest that careful examination of specific findings on a sonogram can help in differentiating the conditions in a timely
fashion.

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Materials and methods


The peer-review missed-case process identified six children
over a 7-year period (20062013) who presented to the emergency room (ER) with abdominal symptoms including pain
and had an initial sonographic diagnosis of intussusception
but subsequent diagnosis of ruptured appendicitis. In all cases
initial US images were obtained in the emergency room; these
included gray-scale, cine and color Doppler images acquired
by US technologists trained in adult and pediatric sonography.
In two cases, the children were also scanned by a pediatric
fellow (n=1) or attending physician (n=1). Most US studies
were obtained on a GE Logiq E9 scanner (GE Healthcare,
Milwaukee, WI), with one initial and several follow-up US
studies on a Siemens Sequoia unit (Siemens Healthcare,
Mountainview, CA). Two of the three follow-up studies were
obtained in the pediatric sonography unit with dedicated pediatric technologists. All three repeat US studies were scanned
by a faculty pediatric radiologist.
Three pediatric radiologists (2 faculty, each with over
30 years of experience in pediatric radiology, 1 fellow) independently and with subsequent consensus evaluated the initial
US studies in a non-blinded fashion. These evaluations were
compared with the formal reports of the initial and follow-up
US studies. Reviewers, aware of the final diagnosis of ruptured appendicitis, were asked whether they would have accepted the initial US diagnosis of intussusception and what
US findings were most suggestive of the correct diagnosis.
The images and reports of subsequent contrast enemas and
CT scans were reviewed and correlated with the US images.
Demographic data, clinical presentation and management, and
surgical findings were obtained from a review of the patients
charts; IRB permission was obtained for image and chart
review.

Results
The number of cases scanned in the same hospital ER for
appendicitis or intussusception over the exact time period is
not known. However a report from the same hospital noted
that approximately 1,200 children were evaluated in the ER
for suspected appendicitis during a 5-year period between
2003 and 2008 [7]. The six children described in the current
report (Table 1) (20062013) ranged in age from 16 months to
8 years (mean age 4.4 years); there were 4 boys and 2 girls
(Table 1). All six children presented with abdominal pain; four
of the six had a history of fever or were febrile in the ER; three
of six had an elevated white blood cell count (Table 1). Three
children (ages 68 years) were older than the typical age for
pediatric ileocolic intussusception, and in two cases clinical
symptoms of periumbilical- to right-lower-quadrant pain were
more typical of appendicitis than intussusception.

Pediatr Radiol (2014) 44:535541


Table 1 Ruptured appendicitis mimicking intussusception
Demographic data
n=6

Age: 16 months8 years, mean 4.4 years


4 boys, 2 girls

Clinical findings
n=6

Abdominal pain6
Fever4
Elevated WBC3
Idiopathic ileocolic intussusception4
Intussuscepting calcified ileocolic mass1
Ileoileal intussusception1
Suggested ruptured appendicitis3
(additional clinical information and consultation
available, all three scanned directly by
radiologist, one case after negative enema
Normal1
Pericolic inflammation, loculated free air1
(suggestive of ruptured appendicitis)
Intussusception reduced2 (partial in 1)
All diagnostic of ruptured appendicitis

Initial US diagnosis
n=6
Repeat US
n=3

Contrast enema
interpretation
n=4
Contrast-enhanced
CT
n=4
Surgery
n=6

Acute laparoscopic appendectomy3


Percutaneous abscess drainage1
Antibiotics and interval appendectomy2

WBC white blood cell count

The initial US images were obtained in the ER and were


preliminarily interpreted by a radiology resident (1) or pediatric radiology fellow (2), with final interpretation (the same
day in four and the next day in two cases) by various faculty
pediatric radiologists with a wide range of experience. In all
six cases sonographic diagnoses of right mid- to lowerquadrant mass consistent with intussusception were suggested. This was thought to be ileocolic and idiopathic in four
cases (Figs. 1 and 2) (Table 1). In one child, the lesion was
noted to contain a large central shadowing calcification that
was interpreted as a probable pathological calcified mass and
the lead point of an intussusception (Fig. 3). In a second child,
the interpreting pediatric radiologist recommended a CT scan
because of the patients age (8 years) and clinical symptoms of
pain and fever, although the US findings were thought to be
consistent with ileocolitis and an ileoileal intussusception. A
third child, 23 months old, with an emergency-room US
diagnosis of intussusception had repeated US imaging in the
ER with real-time scanning by the pediatric radiologist prior to
a planned air-reduction enema. Recognition of marked
perilesional inflammatory changes and fluid led to a change
in the suggested diagnosis and cancellation of the enema.
Independent and consensus review of the six initial US
examinations produced diagnostic agreement among the three
reviewing pediatric radiologists that the initial diagnosis of
intussusception was reasonably supported by the imaging
appearance. Each case exhibited a right lower abdominal mass
with an alternating hypoechoic and echogenic ring-like appearance, suggesting bowel within bowel (Figs. 1, 2 and 3).

Pediatr Radiol (2014) 44:535541

537

Fig. 1 Ruptured appendicitis in a


3-year-old girl who presented
with cramping abdominal pain,
was afebrile and had normal white
blood cell count. a
Anteroposterior abdominal
radiograph shows multiple dilated
bowel loops and a paucity of gas
in the right lower quadrant. The
properitoneal fat appears normal,
and the cecum is not visualized.
Clinical and imaging findings are
most suggestive of
intussusception. b Transverse US
image demonstrates a mass in the
right lower quadrant with
multiple alternating layers
suggestive of intussusception.
Eccentric internal echogenicity
(arrows) was thought to be
suggestive of mesenteric fat. In
retrospect, moderate perilesional
echogenicity (inflammation) can
be seen. c, d Axial contrast
enhanced CT (c) and coronal
reconstruction (d). The CT was
obtained after a normal water
soluble contrast enema followed
by repeat US which showed the
mass to be unchanged. The CT
(c, d) demonstrated a complex
mass in the right lower quadrant
with central enhancing appendix
(arrows) containing an
appendicolith and surrounding
fluid and inflammation, consistent
with perforated appendicitis

This US appearance was considered too complex to simply


represent thickened cecum.
Subsequently four of the six children underwent contrast
enemas for intussusception reduction. Experienced attending
pediatric radiologists performed these studies. One enema in a
3-year-old was interpreted as normal; a repeat US demonstrated a persistent mass; the possibility of perforated appendicitis
was considered and a CT scan was recommended (Fig. 1)
(Table 1). A water-soluble enema in a 16-month-old child
revealed a small bubble of air adjacent to the cecum in the
right lower quadrant as well as pericecal inflammatory changes, suggesting ruptured appendicitis. However, in two 6-yearold children, the pediatric radiologists performing the enema
examinations were convinced that intussusception had been
present; one was thought to have been rapidly reduced
(Fig. 2), and in the other case it was thought to have been
incompletely reduced with a residual right lower quadrant

mass (Fig. 3). The child who was thought to have had an
intussusception reduced on enema had progressive clinical
symptoms of right lower quadrant pain and fever. He
underwent a repeat US with direct scanning by an attending
pediatric radiologist and discussion with the pediatric surgeon.
Although the US appearance was similar to that of the prior
study, marked echogenicity was recognized around the mass,
suggesting inflammation, leading to the conclusion that ruptured appendicitis was probably a more likely diagnosis. This
resulted in a confirmatory CT scan and prevented a planned
repeat enema for intussusception reduction (Fig. 2).
A total of four children had contrast-enhanced CTs demonstrating ruptured appendicitis. All the CT examinations
demonstrated a double-ring appearance correlating with the
US findings of the target sign, with inner and outer rings
representing the dilated appendix and walled-off appendiceal
rupture, respectively (Figs. 1, 2 and 3), with variable

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Pediatr Radiol (2014) 44:535541

Fig. 2 Abdominal pain and


perforated appendicitis
mimicking ileocolic
intussusception in a 6-year-old
boy. a, b Transverse (with color
Doppler) (a) and longitudinal (b)
US images of the right lower
quadrant demonstrate a 3.53-cm
mass with sonolucent and
hyperechoic layers thought to
represent an intussusception. A
central partially shadowing
echogenic focus (arrows) was
misinterpreted as central fat (fat
should not be expected to shadow
to this extent). c Fluoroscopic
image from a water-soluble
enema at which an
intussusception was thought to
have been rapidly reduced. There
are mild right-side pericolic
inflammatory changes, but no
mass is evident. The appendix is
not filled. d, e CT scan with IV
contrast the next day, after repeat
US suggested possible ruptured
appendicitis. Axial (d) and
coronal reconstruction (e) images
show a central dilated fluid-filled
appendix (arrows) with wall
enhancement and periappendiceal fluid collection or
abscess, suggestive of acute
perforated appendicitis. There is
residual bowel contrast from the
prior enema. An appendicolith
can be seen at the base of the
appendix (arrow in e) and there is
an additional fluid collection in
the pelvis

surrounding phlegmon or fluid. Reaching the correct diagnosis of ruptured appendicitis took between 3 h and 24 h in these
six children, with a mean of 10.5 h.
All six children eventually had surgical confirmation of
ruptured appendicitis with no evidence of appendiceal or other
intussusception; in three cases laparoscopic appendectomy
and drainage were performed acutely. One of these children
developed subsequent adhesive small-bowel obstruction

requiring additional surgery. One child underwent percutaneous abscess drainage by interventional radiology, developed a
colocutaneous fistula and had a subsequent interval appendectomy and drain removal. Two other children were treated with
antibiotics acutely with interval appendectomy (Table 1).
Upon further review of the initial US examinations, all six
children demonstrated an appearance suggesting multiple
concentric rings of bowel (target sign, most apparent on

Pediatr Radiol (2014) 44:535541

539

Fig. 3 Ruptured appendicitis in a 6-year-old girl. a Initial US, transverse


view, shows a mass (4.74 cm) consisting of multiple concentric rings
with a central echogenic shadowing focus (arrow) that was initially
interpreted as an intussusception, with a calcified mass as the likely lead
point. b An air enema later the same day demonstrates reflux of air into
the small bowel with mass effect on the cecum (arrows) thought to

represent a residual unreduced intussusception with air having escaped


into small bowel past the mass. c Axial post-contrast CT the same day
demonstrates ruptured appendicitis with a central fluid-filled appendix
and appendicolith (arrow) and surrounding fluid and inflammation corresponding to the multiple layers seen on ultrasonography

transverse views), but none demonstrated typical echogenic


crescentic central mesenteric fat or intralesional lymph nodes.
Focal central echogenicity on US was misinterpreted in some
cases as possible fat but proved to be debris or appendicoliths.
All six of our cases had surrounding thick hyperechoic
echogenicity that, in retrospect, likely represented inflamed
fat walling off the perforated appendix. In five of six children
the size of the mass on US ranged from 3 cm to 4.7 cm, with a
mean size of 3.7 cm by 3 cm, similar to typical ileocolic
intussusception (35 cm). Mass size was 2 cm by 2 cm in
one child thought to have ileoileal intussusception.

The clinical presentations of children with acute appendicitis (including ruptured appendicitis) or intussusception can
overlap, with both conditions capable of resulting in leukocytosis, fever and acute abdominal pain. A palpable mass in the
right flank or lower abdomen is also common to both entities.
Idiopathic intussusception is most often seen in children,
mostly boys, between 6 months and 3 years of age and is
considered to be related to hypertrophy of bowel lymphoid
tissue (Peyer patches), often triggered by a preceding gastrointestinal illness. Intussusception in neonates, as well as older
children, is less likely to be idiopathic and is frequently
associated with a pathological lead point such as a Meckel
diverticulum, duplication cyst, appendicitis, inspissated meconium (in cystic fibrosis), or a neoplasm such as a polyp or
lymphoma [8]. Small-bowel intussusception can be a transient
incidental finding on imaging, but symptomatic cases might
require operative management, especially in postoperative
patients, in association with small-bowel obstruction and with
a longer segment of involvement [9, 10].
US examination is often the first imaging study performed
in children with an acute abdominal presentation. Because of
concerns for radiation exposure, especially in young children,
CT is reserved for cases in which US findings are equivocal or
non-diagnostic [1, 7]. The classic sonographic appearance of
ileocolic intussusception is well-established [13, 5]. US is
considered a very good imaging study for the detection of
intussusception, with reported sensitivity of 100% and

Discussion
Acute abdominal pain in children is a common diagnostic
dilemma, with acute appendicitis and intussusception
representing two of the most important diagnostic considerations [1, 6]. The classic symptoms of acute appendicitis
include anorexia, periumbilical pain followed by right lower
quadrant pain, and vomiting, which can be present in many
other causes of acute abdominal pain in children.
Classic symptoms of intussusception include intermittent
abdominal pain and irritability with later diarrhea, bloody
stools and lethargy, although approximately half of patients
do not present with typical symptoms [1, 5].

540

specificity of 88%. The target, bulls-eye, or doughnut sign of


multiple hypoechoic concentric rings caused by edematous
bowel walls, lymph nodes, and blood vessels with a crescentic
hyperechoic focus of mesenteric fat between the two layers of
bowel are well-known sonographic findings (Fig. 4) [2, 3].
The transverse appearance of a crescent in a doughnut is
considered almost pathognomonic [3, 5] for intussusception.
On longitudinal scans, the intussusception lesion is ovoid in
shape, with different tissues appearing layered longitudinally
and often referred to as a sandwich or pseudokidney sign
(Fig. 4) [2]. Intralesional lymph nodes are commonly seen in
ileocolic intussusception [10]. Other conditions can mimic the
appearance of intussusception on US, including enterocolitis,
volvulus, inflammatory bowel disease and other causes of
edematous or hemorrhagic bowel, as well as feces-filled colon
and even psoas hematoma [2, 5, 11]. Small-bowel intussusception has the same appearance but is usually more central in
location, smaller than the typical 3- to 5-cm size of the mass in
ileocolic intussusception and containing less mesenteric fat
and fewer lymph nodes [1, 10].
Appendicitis can occur at any age but is relatively uncommon in younger infants. Appendiceal intussusception is very
unusual, occurring either as an isolated entity or very rarely as
part of an ileocolic intussusception [12, 13]. Appendiceal
intussusception in children is associated with appendicitis or
an appendiceal mass. None of our cases had surgical evidence
of appendiceal or other intussusception.
The classic sonographic appearance of acute appendicitis
has been well-described. A blind-ending tubular structure
arising from the base of the cecum measuring greater than
6 mm in diameter and having hyperemic walls as well as
surrounding hyperechoic fat, free fluid or enlarged lymph
nodes while being non-compressible is diagnostic of acute
appendicitis [1, 4, 7]. Appendiceal rupture is particularly
common in children [1]. Ruptured appendicitis can prove to
be a difficult diagnosis, both clinically and sonographically.
Sonographic signs of appendiceal perforation include loss of
the echogenic mucosa, increased periappendiceal
Fig. 4 Imaging in a 17-monthold boy who presented with
cramping abdominal pain and
ileocolic intussusception.
Transverse (a) and sagittal (b) US
images show the typical
appearance of ieocolic
intussusception, with a
multilayered multiple-ring
appearance with central
echogenic fat and intralesional
hypoechoic nodes (arrows). Note
the absence of surrounding
echogenic inflamed fat. The child
underwent a successful airreduction enema

Pediatr Radiol (2014) 44:535541

echogenicity from surrounding inflammation, and a complex


mass or focal fluid collection [4]. The appendix itself might
not be seen at all or be difficult to distinguish from surrounding inflammation, fluid and gas.
We found that sonographic appearances of ruptured appendicitis can very closely mimic the sonographic findings of
intussusception; we recognize that our sample size of six cases
is small and that the unblinded methodology used to reevaluate the US studies is biased, but it still confirms that this
misinterpretation can readily occur. Careful imaging, clinical
correlation and an astute radiologist are required to distinguish
the entities. The correct diagnosis is most likely to be considered when there is real-time visualization of the lesion in
multiple planes by a pediatric radiologist, with recognition
of surrounding echogenicity caused by inflamed fat, and the
absence of central mesenteric fat or intralesional nodes. Lesion size was not helpful in our cases because the mass sizes
were close to those expected in typical ileocolic intussusception, with the exception of one case, which was thought to be
ileoileal intussusception. Clinical presentation and features
such as fever and leukocytosis that might raise suspicion for
appendicitis were not present in several of our cases.
Also, the detailed clinical information was usually not
available to the radiologist at the time of initial US examination. Because the US imaging of intussusception is typically
highly reliable, the diagnosis was readily accepted and then
acted on by clinicians. One result was a delay in diagnosis and
treatment; mean diagnostic delay was 10.5 h but was as long
as 24 h in one case. Another consequence was the addition of
multiple imaging studies, including four contrast enemas,
three repeat US studies and four CT scans.
Very few prior reports of this misdiagnosis are in the
literature, although we have encountered and been confounded by this problem on multiple occasions, as have other
experienced pediatric radiologists (personal communications).
A published review of emergency pediatric US imaging illustrates a case of ruptured appendicitis in a 7-year-old that
closely mimicked an intussusception on US; he underwent

Pediatr Radiol (2014) 44:535541

what was deemed an unsuccessful air-enema reduction and


was only correctly diagnosed at surgery, similar to our cases
[1]. Another case report detailed misdiagnosis of ruptured
appendicitis on both US and CT as probable appendiceal
intussusception in an 11-year-old girl [12]. In another case
report an appendiceal hematoma in an 8-year-old girl with von
Willebrand disease was originally thought to be an intussusception on US but was later identified as a hematoma on CT
[11]. These individual reports suggest that the most helpful
differential features were older patient age and smaller size of
the lesion compared to that of typical idiopathic
intussusception.
The CT scans obtained in our cases were diagnostic of
ruptured appendicitis with no confusion with intussusception
(Figs. 1, 2 and 3). In situations where US or enema findings
are unusual or at odds with the clinical picture, radiation
concerns should not prevent the appropriate move to CT for
clarification. MR is being used more commonly for the diagnosis of acute appendicitis and might also be an appropriate
alternative in suitable patients.
Our cases illustrate that ruptured appendicitis can appear as
a rounded, mass-like structure with multiple rings that can be
easily mistaken for intussusception. Awareness of the possibility of this confusion should encourage more detailed,
thoughtful scrutiny of the initial images, with a greater consideration of the possibility of complicated appendicitis.

Conclusion
Ruptured appendicitis is an important mimic of intussusception on US examination. Being mindful of ruptured appendicitis as a diagnostic possibility in children with atypical age or
clinical features and US findings suggestive of intussusception should allow for careful attention to subtle differences in
the sonographic appearances between the entities. Particular
attention should be paid to evaluating the presence of surrounding inflamed fat in appendicitis and intralesional
hypoechoic lymph nodes in ileocolic intussusception. An
inner crescent of echogenic mesenteric fat between bowel
loops is a virtually pathognomonic feature of intussusception
that was not definitely present in our cases of ruptured appendicitis, although it was misinterpreted as such when central

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echogenic debris or appendicolith was present. Real-time US


imaging by a radiologist in multiple planes and direct communication with clinicians were useful in suggesting the correct diagnosis or correcting an erroneous one. CT was diagnostic in all four cases in which it was employed and should be
considered promptly when there are equivocal US features or
there is a discrepancy between US and clinical findings.

Conflict of interest None

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