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Intussusception in Children : A Clinical Review

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DOI: 10.1080/00015458.2015.11681124

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Intussusception in Children 1
Acta Chir Belg, 2015, 115, 000-000

Intussusception in Children : A Clinical Review

T. Charles, L. Penninga, J. C. Reurings, M. C. J. Berry
Department of Orthopedic Surgery, University Hospital Erasme, Brussels Belgium ; Department of General Surgery,
Sint Elisabeth Hospital, Willemstad, Curaçao.

Abstract. Intussusception is the most common cause of small bowel obstruction in young infants. Therefore a high index
of suspicion and thorough knowledge of this condition is of major importance to be able to diagnose and treat this poten-
tially life threatening condition.
In this review we describe epdidemiology, etiology and clinical symptoms of intussuception. Furthermore, we describe
diagnostic modalties, especially ultrasonography as the primary choice for diagnosis. In addition, non-operative treat-
ment with different types of enema reduction techniques, and operative treatment by laparotomy and laparoscopy, and
outcomes have been reviewed.

Introduction hyperplasia of Peyer patches in the lymphoid-rich termi-

Intussusception was first described in 1793 by the Scot-
tish surgeon James Hunter, and is defined as a proximal
bowel segment, or intussusceptum, that like a telescope
moves into a distal bowel segment, or intussuscipiens.
The associated mesentery is dragged within the invagi-
nated segment leading to venous congestion and edema.
This results in ischemia, and eventually bowel necrosis,
perforation and peritonitis if left untreated (1, 2).
There are different types of intussusception ; the ileo-
colic, ileo-ileo-colic, ileo-ileal, jejuno-jejunal and the
colo-colic type (3). The ileo-colic type, where the distal
ileum invaginates through the ileocaecal valve into the
caecum, is the most frequent type of intussusception and
accounts for 90% of the cases (1).
Epidemiology
Intussusception is after appendicitis, the second most
common cause of an acute abdomen in children (1, 2),
and the most common cause of small bowel obstruction
in young infants (4).
The worldwide incidence of intussusceptions ranges
from 15 to 300 / 100 000 children per year (4). Intestinal
intussusception is usually seen in children between 3
months and 3 years of age, with a peak incidence be-
tween 4 and 9 months of age (1-4). Boys are affected ap-
proximately twice as often as girls (1, 3, 5, 6).
Pathogenesis
About 75 to 90% of cases intestinal intussusceptions are
idiopathic, although in these cases the presence of lym-
phoid hyperplasia is frequently reported (1, 2, 4). This
nal ileum could act as lead point for intussusception.
In many cases of intussusception an influence of viral
factors has been suspected as almost 30% of the children
experience a viral illness before the onset of intussuscep-
tions (5, 7, 8). An association with the adenovirus species
C in more than one third of the cases has been reported in
a prospective case-control study in Vietnam and
­Australia (8). Recently an association with the respirato-
ry syncytial virus has also been described (9).
The presence of a pathological lead point (PLP) ­occurs
in 1,5 to 12% of the cases of intussusceptions (1). The
most common PLP in children is the Meckel’s
­diverticulum, but other PLP as polyps, tumor, lympho-
ma, ­duplication cysts, parasites, hematoma, vascular
malformation, inflamed appendix and inverted appendi-
ceal stump have also been described (1-3). Usually PLP
are found in children younger than 3 months of age and
in children older than 5 years, with the incidence increas-
ing with advancing age (1, 3).
Systemic conditions such as Henoch-Schonlein
­purpura, cystic fibrosis, Peutz-Jegher syndrome, familial
polyposis and nephritic syndrome are described as
­predisposing factors of intussusceptions (1-3). Indeed in-
tussusception caused by intestinal wall hematoma, thus
acting as a PLP, is the most common surgical complica-
tion of Henoch-Schonlein purpura (1).
Intussusception has also been described in association
with abdominal trauma and during the postoperative
­period (1).
Clinical manifestations
Abdominal pain occurs in 80 to 95% of cases (1). It is
characterized by the sudden onset of intermittent, crampy,
 2 T. Charles et al.

severe and progressive abdominal pain, usually with 15

to 20 minutes interval. In between episodes patients may
be completely asymptomatic. With prolonged intussus-
ception, the abdomen becomes more distended and signs
of peritonitis may occur when perforation occurs.
The presence of gross or occult blood in the stool is
reported in 50 to 70% of cases, and the mixture of blood
and mucus give a typical redcurrant jelly appearance (1).
However the absence of blood in the stool does not
­exclude intussusception. The palpation of an abdominal
mass, typically a sausage-shaped mass in the upper right
quadrant of the abdomen has been described in up to 60%
of patients (1). This may be accompanied by emptiness
due the absence of bowel in the right lower quadrant of
the abdomen.
The classic clinical triad of Ombredanne consists of Figure 1. This figure represents a longitudinal view of an intus-
intermittent abdominal pain, redcurrant jelly stool and a susceptions as seen on the ultrasonography.
sausage-shaped abdominal mass. This triad is found in
7.5 to 40% of cases (2, 3).
Currently, there are no international guidelines for the
Other symptoms like emesis (60%), diarrhea (30%),
diagnosis of intussusception. The Japanese Guidelines
crying, lethargy, and altered consciousness, sepsis, shock
published in 2011 (3) divided clinical and radiological
and syncope have also been associated with the presence
findings associated with intussusception into 3 criteria ;
of intussusceptions (1). These more aspecific findings
criteria A (pain, bloody stool and palpable mass), criteria
make the diagnosis of intussusception difficult (1, 10,
B (vomitus, pallor, lethargy, shock and bowel gas pattern
11).
on abdominal x-ray) and criteria C (all characteristic im-
ages of intussusception by contrast enema, ultrasonogra-
Investigations phy, CT or MRI). Definitive diagnosis of intussusception
is confirmed by the presence of 1 criteria C.
Plain abdominal X-rays have a sensitivity between 29
Treatment
and 50%. In up to 25% of the cases plain abdominal X-
rays are completely normal (1). Therefore, they should
Treatment of intussusception has to start as soon as pos-
be reserved for cases when perforation is suspected (6).
sible after suspicion of diagnosis with fluid resuscitation
Ultrasonography is the method of choice to diagnose in-
management. Early fluid resuscitation is important be-
tussusception. It has a sensitivity between 98 and 100%
cause most children with intussusception are dehydrated
and a specificity between 88 and 100% (1-3, 11). There-
due to vomiting, decreased oral intake and third
fore, all children with clinical suspicion of intussuscep-
­spacing (1-3).
tion should undergo abdominal ultrasonography (6).
Two typical features are described (1, 2). The first sign is
Conservative treatment
the target/doughnut sign, seen on transverse views, and
represented by a central hyperechoic core, the intussus- Non-operative management is indicated in hemodynami-
ceptum and a hypoechoic outer rim of homogeneous tis- cally and clinically stable children, with high clinical sus-
sue, the intussuscipiens. The second sign is the pseu- picion of intussusception or radiological evidence of in-
dokidney sign, seen on longitudinal views (Fig. 1). It is tussusception, but without any evidence of bowel
represented by a hyperechoic tubular centre covered by a perforation (1-3, 11-13).
hypoechoic rim producing a kidney-like appearance. Non-operative management of intussusception uses
Color Doppler can be used in addition to ultrasonogra- an enema reduction technique.
phy. A lack of color Doppler flow in the bowel wall of The first report of reduction of intussusception by hy-
the intussusceptum may suggest bowel ischemia and drostatic pressure was published in 1876 by Hirschprung.
­predict potential irreducibility (2). Ravitch and Morgan set the guidelines and popularized
Due to the high sensitivity and specificity of abdomi- the use of barium for enema reduction of intussusception
nal ultrasonography and the radiation exposure associat- in 1948 (1, 3). The barium enema technique is the
ed with a CT of the abdomen (3), an abdominal-CT ­therefore the most well-known reduction technique, and
should only be used when other diagnostic modalities are often referred as ‘the golden standard’. Until the past
unrevealing (1). ­decade it remained also the most used method. Because
 Intussusception in Children 3
of complications of chemical peritonitis, infection and
adhesions when perforation occurs while using barium,
other methods to perform enema reduction were devel-
oped and applied (2).
The use of water-soluble contrast has been described
in few reports. These hypertonic solutions could induce
rapid fluid shifts and electrolyte disturbances when per-
foration occurs, and should therefore be diluted to iso-
osmolar concentrations (13).
The use of normal saline for reduction of intussuscep-
tion is not widely reported in the literature. Although, a
few studies reported better reduction rates with saline en-
ema compared to barium enema reduction techniques.
Furthermore, this technique may cause less morbidity
when perforation occurs compared with other enema re-
duction techniques (3,14). Bowel perforation with saline
techniques does not include the risk of chemical peritoni-
tis, the risk of fluid shift nor the risk of tension pneumo-
peritoneum.
Enema reduction using air has become the preferred
method of reduction of intussusception. Success rates
with air enema reduction appear to be higher compared
to other enema techniques. Reduction is described as be-
ing easier, presumably because air reduction allows the
use of higher intra-colonic pressures. It might be safer,
because during air reduction true intra-colonic pressures
can be monitored and controlled (1). Bowel visualization
is more difficult with air enema reduction, especially
when a large amount of gas in the small bowel is present
near the intussusception (13, 15).
When different techniques for enema reduction re-
ported are compared, air enema reduction has the highest
reduction rates (1, 2, 5, 17, 20, 21). A prospective study
reported reduction rates for air enema of 90%, for hydro-
static enema of 80% and for barium enema of 73% (5). In
this german study, they calculated that the introduction of
air enema therapy as first-line treatment for intussuscep-
tion might prevent 104 surgeries per year in Germany. A
recent meta-analysis showed that for every nine patients
treated with air enema reduction instead of hydrostatic
enema reduction, 1 failure would be avoided (NNT of
9) (12). Another advantage for air enema reduction is
shorter fluoroscopic time and lower radiation exposure to
the patient (19).
Over time, fluoroscopy has been replaced by ultraso-
nography to monitor the reduction of the intussusception.
Ultrasonography has many advantages such as absence
of radiation exposure, better visualization of the intus-
susception and its reduction, and the possibility to detect
and recognize pathological lead points (2, 13). It was
speculated that detection of perforation during the proce-
dure could be less accurate with ultrasonography com-
pared to fluoroscopy (13), but 2 retrospective studies
showed no problems in detection of perforation during
ultrasonography guided procedures (20, 22).
Overall success rates of enema reduction techniques
for intussusception vary between 61 and 95% (1, 2, 5, 6,
10-13, 14, 17, 18, 20-22). Factors which decrease the
success rate for enema reduction techniques include
younger age (younger than one year of age), longer dura-
tion of symptoms (usually more than 24 hours), early re-
currence, and other factors that suggest that the condition
has progressed like ; the presence of bloody stool and
radiographic signs of intestinal obstruction (12, 13, 23).
Differences in success rates might reflect the learning
curve of the radiologist with the used technique (6, 10,
16), and the patient population encountered. Lower suc-
cess rates would be expected in regions of the world
where a delay occurs in patients getting to the hospital in
a timely manner (9, 23, 24).
Medical interventions have been attempted to increase
reduction rates. Buscopan has been administrated, but
no comparative studies exist to prove efficacy of busco-
pan (3). In addition, glucagon has been studied in three
randomised trials to increase reduction rates of intussus-
ception, but no benefits of its use was found (3, 13, 25,
26).
Sedation and general anesthesia have also been used
to improve reduction rates. It was observed that sedation
interferes with the Valsalva maneuver, and it is assumed
that this maneuver could protect against perforation (27).
Sedation requires proper monitoring and, until now there
is little evidence to support or refute the use of sedation.
A recent study by Purenne et al. reported an increase in
reduction rates from 72 to 90% while using general anes-
thesia compared to the use of sedation for enema reduc-
tion (28), while an older study showed no significant dif-
ference comparing sedation to general anesthesia to
perform an enema reduction (29). Because of the promis-
ing results of the recent study by Purenne et al., it could
be useful to perform other studies to confirm or reject
their results.
Perforation is the most feared complication of enema
reduction, and perforation rates vary between < 1% to
4% (1-3, 13). The variation in perforation rate might be
related to the learning curve of the radiologist, a too ag-
gressive enema reduction approach, too high reduction
pressures and patient selection, as in some centers pa-
tients considered to be at risk for perforation directly un-
dergo laparotomy or laparoscopy (13, 20). Risk factors
for perforation include infants younger than 6 months of
age, presumably because of a thinner bowel wall (13,
30). Delay between onset of symptoms and treatment is
also described as a risk factor, and the duration of symp-
toms in these cases is usually reported to be 36 to
48 hours, or longer (2, 9, 13, 23, 24, 30).
Bramson and Blickman suggested in 1992 that bowel
perforation already might be present prior to an attempt-
ed reduction, where the apposition of the two bowel seg-
ments could prevent the escape of intraluminal air into
 4 T. Charles et al.
the peritoneal cavity. When reduction is achieved, air
escapes and this gives the typical clinical and radiologi-
cal picture of free intraperitoneal air (31).
Perforation with air during air enema reduction might
cause a tension pneumoperitoenum, in which intraperito-
neal air under pressure causes life-threatening ventilatory
and hemodynamic compromise. This is prevented by dis-
continuing the enema and releasing the air from the co-
lon (20). Needle decompression of the abdomen is found
to be a safe and effective way to prevent tension (33).
Though, conflicting with previous reports, all four chil-
dren in their review who complicated with perforation
during air enema reduction needed a bowel resection.
In the past, when intussusception was found to be ir-
reducible after a first attempt of enema reduction, imme-
diate surgery was standard practice. However, by the
time laparotomy was performed some cases of intussus-
ception were found to be spontaneously reduced and
some other cases were really easy reduced manually dur-
ing surgery (3). Therefore, the concept of repeated de-
layed enema reduction has been introduced. A repeated
delayed attempt for enema reduction is indicated only
when the patient is stable, without any evidence of nei-
ther perforation nor peritonitis (3, 34). It is also required
that the first enema attempt was able to move the intus-
susception, thus achieving partial reduction (1, 3, 34).
This because it is believed that a partial reduction and
time interval between two attempts allow venous conges-
tion and edema of the bowel to decrease, thus facilitating
reduction of the residual intussusceptions (3). The time
delay between two attempts varies between 30 minutes
and up to a few hours (2, 34). The optimal time interval
has not been defined yet. Another discussion focuses on
the numbers of attempts that could be made before sur-
gery is indicated. Despite absence of consensus on these
points, the ‘rule of threes’ is largely applied : no more
than 3 attempts of 3 minutes (2, 3). But reports of more
frequent and longer successful attempts exist (34).
Operative treatment
The need for surgical intervention for intussusception
varies in different regions of the world and between dif-
ferent hospitals. The incidence of surgery has been re-
ported to vary from 13% in Asia, 20% in Europe, 28% in
North America, 29% in Oceania and Eastern Mediterra-
nean, 77% in Africa to 86% in Central and South Ameri-
ca (4). As we noticed the need for surgical intervention is
less common in developed areas, whereas in developing
areas the percentages of surgical procedures to treat in-
tussusception remains high. This could be explained by a
delay in seeking for medical attention (patient delay),
less experience in radiological techniques, less access to
medical facilities and differences in healthcare infra-
structures over the world (4, 6, 9, 24, 35).
A difference in surgical intervention rates could also
be explained by a difference in decision making, as in
some centers enema reduction is always stated as the
standard procedure before surgical intervention whereas
in other centers patients considered to be at risk for per-
foration during enema reduction underwent directly a
surgical procedure (13, 20).
There is consensus that primary surgical intervention
is indicated for patients with suspected intussusception
who are hemodynamically unstable, when there is evi-
dence of bowel necrosis, bowel perforation or peritonitis,
or when safe facilities to perform an enema reduction are
not available (1-3, 13, 36). Surgical treatment is also in-
dicated in cases of failure of the conservative therapy (1-
3, 13, 36). In addition, surgery is more often recommend-
ed when a pathological lead point is the cause of
intussusception (1-3, 13).
When manual reduction of the intussusception is not
possible or when a pathological lead point or bowel ne-
crosis is present at the time of laparotomy or laparosco-
py, bowel resection is indicated (3, 37). The need for
bowel resection varies between 25 to 40% (6, 9, 11, 16,
18, 24, 28, 34, 35). Higher rates of bowel resection could
be explained by delayed presentation. A study from
­Nigeria, reported that a delayed presentation of more
than 24 hours predisposed to bowel complications such
as increased irreducibility, and devitalized bowel at time
of operation, and they suggested that this high incidence
of bowel complications might favor primary surgical
­intervention in most of these cases (24). Another retro-
spective study showed that the risk of bowel resection
during surgical reduction of the intussusception was 80%
less when performed in a hospital employing full-time
pediatric surgeons after adjustment of the results (37).
They also noticed that ‘severe disease’ and concomitant
gastro-intestinal pathology where also associated with a
significantly increased risk of bowel resection during
­surgery.
Surgical intervention in patients with intussusception
can be performed both as an open or laparoscopic proce-
dure. Apelt and colleagues reviewed all laparoscopic re-
ductions of intussusception. They identified 10 retrospec-
tive studies with a total of 276 cases of laparoscopic
reduction of intussusceptions (38), and found a success
rate of 71%, with a subsequent conversion rate to lapa-
rotomy of 29%. Complications reported included : intra-
operative complications in 0,4% and postoperative com-
plications in 2,9%. They concluded in their review that
laparoscopy was an effective and safe way to reduce in-
tussusceptions in children. A French study described risk
factors for conversion to laparotomy such as : more than
1.5 days between onset of symptoms and diagnosis, pres-
ence of signs of peritonitis at primary clinical examina-
tion and the presence of a pathological lead point (39).
Laparoscopy is nowadays assumed to reduce hospital
 Intussusception in Children 5
length of stay, postoperative complications especially
wound infections, postoperative pain and improves cos-
metic results. But in this specific setting, we lack pro-
spective randomised trials comparing laparoscopy with
laparotomy to confirm this.
Recurrence
Recurrence tend to occur from 6 hours up to 4 years after
an initial episode of intussusception (40). The reported
recurrence rates of intussusception after enema reduction
varies between 8 and 15%, independent of the used tech-
nique (13, 40-42). One study found that recurrence rates
tended to increase with the number of recurrent epi-
sode (41). After a first enema reduction, the recurrence
rate was found to be approximately 15,7%. After a sec-
ond reduction it increased to 37,7%, after a third episode
to 68,4% and after a fourth episode recurrence rate was
nearly 100%. Recurrence rates after surgical reduction
tend to be less high, between 1 to 3% after manual reduc-
tion during surgery (13, 40-42). No recurrence occurs
when bowel resection is performed during surgery (13,
40-42). There are some controversies about whether or
not an associated ileopexy decreases recurrence rates in
the literature.
Each recurrent episode of intussusception should be
treated as if it was the first episode of intussusception (13,
41, 42). This is recommended both when the reduction
before the recurrence was a successful non-operative re-
duction and in case of a previous successful surgical re-
duction. For recurrences of intussusception, a surgical
reduction should be considered in case of failure of non-
operative treatment, a suspected pathological lead point
or in case of several recurrent episodes. Though, there
are no specific recommendations about after how many
recurrent episodes surgical management is warranted. An
older study suggested that any patient who presented
with a third recurrent episode of intussusception within a
short period of time should be taken to surgery because
of a high incidence of pathological lead point (23). An-
other study referred to the increase of recurrence rates
with the number of episode, and suggested also that
­surgery should be considered after a third episode of
­intussusception (41). In the French study, as mentioned
before, the authors described that recurrences can
­successfully be managed with laparoscopy (39).
Outcomes
The success rates of enema reduction techniques vary
­between 61% and 95%, as described previously in our
review (1, 2, 5, 6, 10-14, 17, 18, 20-22). Besides previous
described suggestions to explain this variation in rates,
some authors also noted the presence of factors that
­predisposed to lower reduction rates. Those risk factors
include ; the younger age, the presence of rectal bleeding
at clinical exam, the presence of radiographic signs of
bowel obstruction, a longer duration of symptoms (usu-
ally more than 24 hours), early recurrence and an ileo-­
ileal or ileo-ileo-colic type of intussusceptions (9, 12, 13,
23, 24). These factors could be considered when decision
making towards the optimal treatment for a specific pa-
tient.
Besides the lack of adequate diagnostic criteria for in-
tussusception, a recognized severity score for this condi-
tion has also not yet been established. The Japanese
Guidelines, published in 2011, proposed a severity as-
sessment in order to optimize decision making regarding
preferred treatment for a patient suffering from intussus-
ceptions (3). Until now, this is the only report to propose
a severity score for this condition. They distinguished
between severe, moderate and mild cases of intussuscep-
tion. The severe form is best described as being similar to
the indications for primary surgery. A moderate intus-
susception includes criteria such as factors known to de-
crease the success rates of enema reduction technique
and others criteria such as the location of the apex of the
intussusception beyond the splenic flexure, high leuco-
cytes, high C-reactive protein values, lack of blood flow
assessed with Color Doppler, and the presence of a path-
ological lead point. Mild intussusceptions are therefore
described as cases of intussusception presenting without
any of the above criteria of severe and moderate intus-
susception.
Exact numbers regarding morbidity associated with
­intussusception are lacking. It seems that morbidity is
highly influenced by the time lapse between onset of
symptoms and diagnosis (24). Most of the patients have
a favorable course if reduction is achieved within 24 hours
from the onset of symptoms, while delayed presentation
decreases reduction rates and increases the need for surgi-
cal intervention and associated surgical complica-
tions (23, 24). As mentioned, a case serie from Nigeria
reported that a delayed presentation predisposed to bowel
complications with an increased irreducibility and pres-
ence of devitalized bowel at the time of diagnose (24).
Mortality in association with intussusception is quite
low (< 1%) in most parts of the world (4). Though, in
Africa mortality up to 9,4% has been reported (4). This
high mortality probably reflects the difference in health-
care infrastructure and the delay in seeking for medical
care (4, 24). Most African reports described a delay in
seeking for medical attention of between 24 hours and
4 days.
Conclusions
Intussusception is the most common cause of small
­bowel obstruction in young infants. Therefore a high
­index of suspicion and thorough knowledge of this
 6 T. Charles et al.
c­ ondition remain of major importance to be able to diag-
nose and treat this potentially life threatening condition.
Abdominal ultrasonography is the primary diagnostic
tool in the work op of intussusception, because of its high
specificity and sensitivity, and the absence of radiation
exposure.
Air enema reduction techniques monitored by ultra­
sonography is the preferred first line treatment in many
institutions with good results and few complications.
Surgery should be performed when bowel necrosis or
perforation is suspected. Furthermore, surgical reduction
is indicated when non-operative treatment with enema
reduction fails, and a laparoscopic approach should be
considered.
International diagnostic criteria guidelines and sever-
ity scores are needed to guide towards adequate and
­optimal treatment for this condition. Furthermore, pro-
spective, preferable randomized studies are warranted to
establish firm evidence regarding treatment guidelines
for this condition.
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 Intussusception in Children 7

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