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Intussusception in children
Seiji Kitagawa, MD; Mohamad Miqdady, MD Up to date on line 16.1 This topic last updated: Fevereiro 4, 2008 Modificado: Jefferson P Piva
INTRODUCTION Intussusception,
the invagination of a part of the
intestine into itself, is the most
common abdominal emergency in early
childhood, particularly in children
younger than 2 years of age, and the
second most common cause of
intestinal obstruction after pyloric
stenosis [1]. Intussusception is unusual
in adults, and the diagnosis is
commonly overlooked. In the majority
of cases in adults, a pathologic cause is
identified [2]. In contrast, the majority
of cases in children are idiopathic.
EPIDEMIOLOGY Intussusception is
the most common cause of intestinal
obstruction in infants between 6 and 36
months of age. Approximately 60
percent of children are younger than 1
year old, and 80 percent are younger
than 2. Intussusception is rare before 3
months and after 6 years of age. In a
population-wide survey in Switzerland,
the yearly mean incidence of
intussusception was 38, 31, and 26
cases per 100,000 live births in the
first, second, and third year of life,
respectively, then fell to less than half
that rate in older age groups [3]. Most
episodes occur in otherwise healthy and
well-nourished children.
Intussusception appears to have a
slight male predominance, with a
male:female ratio of approximately 3:2.
PATHOGENESIS Intussusception
occurs most often near the ileocecal
junction (ileo-colic), although ileo-ileo-
colic, jejuno-jejunal, jejuno-ileal, or
colo-colic intussusception also have
been described. The proximal segment
of bowel telescopes into the distal
segment, dragging the associated
mesentery with it. This leads to the
development of venous and lymphatic
congestion with resulting intestinal
edema, which can ultimately lead to
ischemia, perforation and peritonitis.
A lead point can be identified in up to
25% of children with ileo-colic
intussusception. The remaining 75%
are considered to be idiopathic,
although an increasing body of
evidence suggests that viral triggers
may play a role in some cases:
The incidence of intussusception has a
seasonal variation, with peaks
coinciding with seasonal viral
gastroenteritis in some populations
[3,4] .
Intussusception has been associated
with some forms of rotavirus vaccine.
An early form of the vaccine (RRV-TV:
Rotashield) was removed from the
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Invaginao intestinal 2
market because of a 22-fold increase
in intussusception among vaccinated
infants. The vaccine currently used in
the United States (PRV: Rotateq)
appears to have rates of
intussusception that are similar to
expected background rates. However,
there may be additional cases of
intussusception that have not been
reported. Providers should be alert for
cases of intussusception that may be
associated with rotavirus vaccine, and
report all suspected cases to the
Vaccine Adverse event Reporting
System (VAERS).
Approximately 30% of patients
experience viral illness (upper
respiratory tract infection, otitis
media, flu-like symptoms) before the
onset of intussusception.
A strong association with adenovirus
infection has been shown in a variety
of populations. In 30 to 40 % of
cases, there is evidence of recent
infection with enteric and non-enteric
species of adenovirus [5-9]. In a
prospective case-control study
examining a variety of potential
infectious triggers for intussusception
in Vietnam and Australia, infection
with adenovirus, as the strongest
predictor of intussusception in both
populations [9].
Viral infections, including enteric
adenovirus, can accentuate lymphatic
tissue in the intestinal tract, resulting in
hypertrophy of Peyer patches in the
lymphoid-rich terminal ileum, which
potentially acts as a lead point for ileo-
colic intussusception [5]. A role for
corticosteroids in the prevention of
recurrence has been proposed in light
of this possible association with
lymphoid hyperplasia [10,11].
A lead point is recognized more
commonly in children older than five
years [1,4,12]. A variety of pathologic
conditions associated with
intussusception, including small bowel
lymphoma [13-15], Meckel diverticulum
[16], duplication cysts [17,18] , polyps
[19], vascular malformations [20] ,
inverted appendiceal stumps [21,22] ,
parasites (eg, Ascaris lumbricoides)
[23,24], Henoch-Schnlein purpura
[25], and cystic fibrosis [26], have
been described.
The mechanisms leading to
intussusception differ depending upon
the specific cause. As examples:
Small bowel intussusception may
occur after an episode of Henoch-
Schnlein purpura, with a bowel wall
hematoma acting as the lead point. In
these cases, intussusception typically
occurs after resolution of the
abdominal pain.
In patients with cystic fibrosis, thick
inspissated stool may act as the lead
point [26] .
Patients with celiac disease may
develop small bowel intussusception
secondary to dysmotility and
excessive secretion or bowel wall
weakness [27,28].
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Invaginao intestinal 3
Patients with Crohn disease may
develop intussusception because of
inflammation and stricture formation
[29] .
Postoperative: Bowel intussusception
(usually jejuno-jejunal) also has been
described in the postoperative setting
where it is an uncommon but insidious
cause of intestinal obstruction. It
usually occurs within two weeks of a
laparotomy and is caused by adhesions.
The patient typically does well for
several days and may even resume oral
intake before developing symptoms of
mechanical obstruction. The diagnosis
can be difficult to establish because it
may be confused with postoperative
paralytic ileus. The ultrasonography and
computed tomography (CT) scanning
may be helpful. Diagnostic contrast
studies are not as helpful in
postoperative intussusception because
most cases occur in the small intestine,
CLINICAL MANIFESTATIONS:
Patients with intussusception typically
develop the sudden onset of
intermittent, severe, crampy,
progressive abdominal pain,
accompanied by inconsolable crying and
drawing up of the legs toward the
abdomen. The episodes usually occur at
15 to 20 minute intervals. They become
more frequent and more severe over
time. Vomiting may follow episodes of
abdominal pain. Initially emesis is non-
bilious, but it may become bilious as
the obstruction progresses. Between
the painful episodes, the child may
behave relatively normally and be free
of pain. As a result, initial symptoms
can be confused with gastroenteritis
[30]. As symptoms progress, increasing
lethargy develops.
In up to 70 % of cases, the stool
contains gross or occult blood [31]. The
stool may be a mixture of blood and
mucous, giving it the appearance of
currant jelly. A sausage-shaped
abdominal mass may be felt in the right
side of abdomen. However, the
classically described triad of pain, a
palpable sausage shaped abdominal
mass, and currant-jelly stool is seen in
less than 15 percent of patients at the
time of presentation [30,32] . As many
as 20% of young infants have no
obvious pain. Approximately one-third
of patients does not pass blood or
mucus or develop an abdominal mass.
Occasionally, the initial presenting sign
may be lethargy or altered
consciousness alone, without pain,
rectal bleeding, or other symptoms that
suggest an intra-abdominal process
[33-35]. In most cases, this clinical
presentation occurred in infants, and
was often confused with sepsis. Thus,
intussusception should be considered in
the evaluation of lethargy or altered
consciousness, especially in infants.
DIAGNOSIS AND NONSURGICAL
TREATMENT A high index of
suspicion coupled with early diagnosis
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Invaginao intestinal 4
of intussusception may obviate the
need for surgical intervention.
For patients in whom the diagnosis is
unclear at presentation, initial workup
may include abdominal ultrasound or
abdominal plain films, provided that
these studies do not significantly delay
the definitive diagnosis and treatment
of intussusception.
Patients with a typical presentation, in
whom there is a high index of suspicion
for intussusception, may proceed
directly to an enema contrast study.
These studies can establish the
diagnosis of ileocolic intussusception,
and frequently also are therapeutic.
Before obtaining a contrast study, the
patient should be stabilized and
resuscitated with intravenous fluids; the
stomach should be decompressed with
a nasogastric tube. The patient should
be considered fit for surgery, in case
surgery is necessary, before he or she
undergoes radiographic imaging. The
surgical team should be notified before
attempted reduction because there is a
risk of perforation and because surgical
intervention may be necessary if
contrast enema fails to reduce the
intussusception.
Abdominal plain films Plain
radiographs of the abdomen may be
helpful because they may show frank
intestinal obstruction or massively
distended loops of bowel with absence
of colonic gas. Although rarely seen,
the presence of pneumoperitoneum
suggests that bowel perforation has
occurred. A target sign, consisting of
two concentric radiolucent circles
superimposed on the right kidney
representing peritoneal fat surrounding
and within the intussusception,
appeared in 26% of 94 patients in one
report [36]. A soft-tissue density
projecting into the gas of the large
bowel (intussusception image) is called
the "crescent sign".
The presence of air in the ascending
colon can help to exclude
intussusception in cases with a low
clinical suspicion of the disease. In a
retrospective study from a single
center, plain radiographs with three
views (supine, lateral, and prone) were
used to screen patients with suspected
intussusception [37]. The presence of
air in the ascending colon on at least
two of these views had high sensitivity
for excluding intussusception in this
patient population with a low clinical
suspicion of disease (sensitivity 96.3
percent, 95% CI 89.2-100). However,
the sensitivity of plain radiographs may
be considerably lower in different
clinical settings or when fewer views
are analyzed. Therefore, we do not
recommend relying on plain
radiography to exclude intussusception
if there is a significant clinical suspicion
of the disease.
Ultrasonography Ultrasonography
also can be useful; its sensitivity and
specificity approach 100 percent in the
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Invaginao intestinal 5
hands of an experienced
ultrasonographer [38-40] . The classic
ultrasound image of intussusception is a
"bull's eye" or "coiled spring" lesion
representing layers of the intestine
within the intestine. In addition, a lack
of perfusion in the intussusceptum
detected with color duplex imaging may
indicate the development of ischemia.
An advantage of ultrasonography is
that it can diagnose the rare ileoileal
intussusception and identify the lead
point of intussusception in
approximately two-thirds of cases in
which underlying pathology exists [41].
Ultrasonography may also be useful in
predicting which children are most
likely to need surgical intervention for
small bowel intussusception. In a small
retrospective study, cases in which the
intussusceptum was less than 3.5 cm
were more likely to resolve
spontaneously during or shortly after
the ultrasonographic examination as
compared to cases with a longer
intussusceptum [42] .
Reduction under ultrasonographic
control without fluoroscopy has been
described [43,44] . Signs of successful
reduction include the disappearance of
the intussusception and the retrograde
flow of the water-soluble contrast into
the small intestine. Ultrasound guided
water enema also has been used [45].
Signs of successful reduction with water
include the disappearance of the
intussusception and the appearance of
water and bubbles in the terminal
ileum.
Contrast studies The standard
procedure for diagnosis and treatment
of ileocolonic intussusception is a
contrast (air or radiopaque) enema
[46]. Broad-spectrum antibiotics may
be administered before attempting
nonoperative reduction since there is a
risk of perforation with these
procedures.
As compared with ileocolonic
intussusception, small bowel (ileoileal,
jejunoileal, or jejunojejunal)
intussusception is less likely to be
reducible by contrast enema [47,48].
Intussusceptum length as measured by
ultrasonography may be helpful in
determining which patients may be
managed expectantly, and which are
likely to require surgical intervention.
Barium and water-soluble
contrast Traditionally, barium has
been the preferred contrast agent in
most North American and European
centers [49-51]. Before instilling
barium, a water-soluble contrast enema
should be considered, particularly if
there is a high risk of perforation.
Water-soluble agents reduce the risk of
electrolyte disturbances and peritonitis
in patients in whom perforation has
occurred.
Barium enema reduction of
intussusception can be performed if
there is no evidence of perforation. The
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Invaginao intestinal 6
standard method of reduction is to
place a reservoir of barium 1 meter
above the patient so that constant
hydrostatic pressure is generated. With
experience (and depending upon the
clinical status of the patient), a
physician may undertake a more
aggressive reduction.
In a typical ileo-colic intussusception,
the barium column appears as an
intraluminal-filling defect. The
intussusception can be found in any
part of the large bowel, even the
rectum. Occasionally, some barium may
coat the outer surface of the
intussuscipiens, resulting in a coiled
spring pattern.
Successful reduction is indicated by the
free flow of contrast into the small
bowel. Reduction is complete only when
a good portion of the distal ileum is
filled with barium, thus excluding ileo-
ileal intussusception. Other indications
of successful reduction include relief of
symptoms and disappearance of the
abdominal mass. A characteristic sound
also may be appreciated with
auscultation. In occasional patients, the
contrast material does not reflux freely
into the small bowel even with a
complete reduction, but no filling defect
in the cecum is present apart from the
ileocecal valve and symptoms and signs
of these patients resolve.
A post-reduction filling defect in the
cecum commonly is seen, probably the
result of residual edema in the ileocecal
valve. However, benign and malignant
lesions cannot be distinguished by
radiologic examination alone. As a
result, a repeat study or even
laparotomy may indicate if any concern
of a benign or malignant lead point
exists [53].
After the successful reduction of an
ileo-colic intussusception, a
temperature higher than 38C
(100.4F) often is noted because of the
release of endotoxin, cytokines, or
bacterial translocation. The patient
should be admitted to the hospital for
12 to 24 hours for observation.
Nasogastric suction usually is
maintained until bowel function has
returned and the patient has had
passage of a bowel movement.
Feedings then are advanced as
tolerated.
Air contrast Air reduction
techniques have gained popularity as
an alternative to the contrast
hydrostatic methods [54-57]. The
technique begins with insertion of a
Foley catheter into the rectum.
Fluoroscopy is used to assess the
presence of bowel gas in the abdomen.
Air is then instilled, initially by hand
pump, until the diagnosis is established
and the intussusceptum is pushed back
gently [49,50] .
An electric pump is connected if the
intussusceptum stops moving despite
the use of the hand pump. Both of
these pumps must have a pressure
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Invaginao intestinal 7
release system so that the pressure
remains between 80 and 120 mmHg.
Carbon dioxide can be used instead of
air. It has the advantage of being
absorbed rapidly from the gut, is
associated with less discomfort, and is
less dangerous than air, which
potentially could cause an air embolism
(although air embolisms have not been
reported). Reflux of air into the
terminal ileum and the disappearance
of the mass at the ileocecal valve
indicate reduction.
An advantage of this method is the
ease with which the intussusception can
be reduced with less radiation exposure
and cost, and the relatively harmless
nature of air in the peritoneal cavity,
compared with that of other contrast
materials.
Risk and complications The main
risk of hydrostatic or pneumatic
reduction is perforation of the bowel,
which occurs in 1% or fewer patients
[58-60]. The perforation usually occurs
on the distal side of the
intussusception, often in the transverse
colon, and commonly where the
intussusception was first demonstrated
by radiographic studies [61,62] . The
risk of perforation is greatest in infants
younger than 6 months of age who
have had symptoms for three days or
longer and in patients with evidence of
small bowel obstruction [63].
Nonoperative reduction should not be
attempted in patients with prolonged
symptoms or any signs of peritoneal
irritation or free peritoneal air.
Success rate Nonoperative
reduction using barium or air contrast
techniques is successful in
approximately in 60 to 90% of patients
with ileo-colic intussusception [3,64-
66]. Success is more likely to be
achieved in patients with idiopathic
intussusception (ie, no identifiable lead
point), although it also can be
accomplished in patients with a
recognized lead point [53]. Successful
radiographic reduction is more likely if,
during reduction, the small bowel is
visualized before the appendix [67].
lleo-ileo-colic intussusception may be
more difficult to reduce because the
barium often percolates along the loops
of small bowel in the colon, reducing
the effective pressure of the enema.
In addition, success is less likely to be
achieved in infants younger than 1 year
of age (particularly younger than 3
months), in children older than 5 years
of age, and when plain films show signs
of intestinal obstruction [30,58,64] .
Although some authors have noted a
reduced likelihood of reduction when
symptoms have been present for longer
than 48 hours [30,58,66,68] , others
have found no such correlation [65] . In
these high-risk cases, the barium study
should still be performed to confirm the
diagnosis and attempt therapy, but a
pediatric surgeon should be readily
available in the imaging department.
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Invaginao intestinal 8
Recurrence after successful
nonoperative reduction is close to 10%
[30,69,70]. Recurrence is not
necessarily an indication for surgery.
Each recurrence should be handled as if
it were the first episode, provided that
each is successfully reduced [71].
SURGERY Surgery is indicated when
nonoperative reduction is incomplete or
when a persistent filling defect,
indicating a mass lesion, is noted [71] .
As noted above, a residual filling defect
may be seen because of edema of the
ileocecal valve. Thus, when a filling
defect appears to be associated with
edema in the area, an ultrasonographic
or repeat contrast study should be
performed provided that symptoms
have resolved.
Broad-spectrum intravenous antibiotics
should be given before surgery. Manual
reduction at operation is attempted in
most cases, but resection with primary
anastomosis needs to be performed if
manual reduction is not possible or if a
lead point is seen.
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