Intussusception in Children: A Clinical Review

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 potentially 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 treatment with different types of enema
reduction techniques, and operative treatment by laparotomy and laparoscopy, and outcomes
have been reviewed.

Introduction

Intussusception was first described in 1793 by the Scottish 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
invaginated 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 ileocolic, 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 between 4 and 9 months of age (1-4). Boys are
affected approximately 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 lymphoid hyperplasia is frequently reported (1, 2, 4). This hyperplasia
of Peyer patches in the lymphoid-rich terminal 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 intussusceptions (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 respiratory 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, lymphoma, duplication cysts, parasites, hematoma, vascular
malformation, inflamed appendix and inverted appendiceal 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 increasing 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 intussusception caused by intestinal wall hematoma, thus
acting as a PLP, is the most common surgical complication of Henoch-Schonlein purpura (1).
Intussusception has also been described in association with abdominal trauma and during the
postoperative period (1).

Clinical Manifestasion

Abdominal pain occurs in 80 to 95% of cases (1). It is characterized by the sudden

onset of intermittent, crampy, severe and progressive abdominal pain, usually with 15 to 20
minutes interval. In between episodes patients may be completely asymptomatic. With
prolonged intussusception, 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 intermittent abdominal pain,
redcurrant jelly stool and a sausage-shaped abdominal mass. This triad is found in 7.5 to 40%
of cases (2, 3).
Other symptoms like emesis (60%), diarrhea (30%), crying, lethargy, and altered
consciousness, sepsis, shock and syncope have also been associated with the presence of
intussusceptions (1). These more aspecific findings make the diagnosis of intussusception
difficult (1, 10, 11).

Investigations

Plain abdominal X-rays have a sensitivity between 29 and 50%. In up to 25% of the
cases plain abdominal Xrays are completely normal (1). Therefore, they should be reserved
for cases when perforation is suspected (6). Ultrasonography is the method of choice to
diagnose intussusception. It has a sensitivity between 98 and 100% and a specificity between
88 and 100% (1-3, 11). Therefore, all children with clinical suspicion of intussusception
should undergo abdominal ultrasonography (6). Two typical features are described (1, 2). The
first sign is the target/doughnut sign, seen on transverse views, and represented by a central
hyperechoic core, the intussusceptum and a hypoechoic outer rim of homogeneous tissue, the
intussuscipiens. The second sign is the pseudokidney sign, seen on longitudinal views (Fig.
1). It is represented by a hyperechoic tubular centre covered by a hypoechoic rim producing a
kidney-like appearance.
Color Doppler can be used in addition to ultrasonography. A lack of color Doppler
flow in the bowel wall of the intussusceptum may suggest bowel ischemia and predict
potential irreducibility (2).
Due to the high sensitivity and specificity of abdominal ultrasonography and the
radiation exposure associated with a CT of the abdomen (3), an abdominal-CT should only be
used when other diagnostic modalities are unrevealing (1).
Currently, there are no international guidelines for the diagnosis of intussusception.
The Japanese Guidelines published in 2011 (3) divided clinical and radiological findings
associated with intussusception into 3 criteria ; criteria A (pain, bloody stool and palpable
mass), criteria B (vomitus, pallor, lethargy, shock and bowel gas pattern on abdominal x-ray)
and criteria C (all characteristic images of intussusception by contrast enema,
ultrasonography, CT or MRI). Definitive diagnosis of intussusception is confirmed by the
presence of 1 criteria C.

Figure 1. This figure represents a longitudinal view of an intussusceptions as seen on the ultrasonography

Treatment

Treatment of intussusception has to start as soon as possible after suspicion of

diagnosis with fluid resuscitation management. Early fluid resuscitation is important because
most children with intussusception are dehydrated due to vomiting, decreased oral intake and
third spacing (1-3).

Conservative treatment

Non-operative management is indicated in hemodynamically and clinically stable

children, with high clinical suspicion of intussusception or radiological evidence of
intussusception, but without any evidence of bowel perforation (1-3, 11-13).
 Non-operative management of intussusception uses an enema reduction technique.
The first report of reduction of intussusception by hydrostatic pressure was published
in 1876 by Hirschprung. Ravitch and Morgan set the guidelines and popularized the use of
barium for enema reduction of intussusception in 1948 (1, 3). The barium enema technique is
the therefore the most well-known reduction technique, and often referred as ‘the golden
standard’. Until the past decade it remained also the most used method. Because of
complications of chemical peritonitis, infection and adhesions when perforation occurs while
using barium, other methods to perform enema reduction were developed 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 perforation occurs,
and should therefore be diluted to isoosmolar concentrations (13).
The use of normal saline for reduction of intussusception is not widely reported in the
literature. Although, a few studies reported better reduction rates with saline enema compared
to barium enema reduction techniques. Furthermore, this technique may cause less morbidity
when perforation occurs compared with other enema reduction techniques (3, 14). Bowel
perforation with saline techniques does not include the risk of chemical peritonitis, the risk of
fluid shift nor the risk of tension pneumoperitoneum.
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 being 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 reported 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 hydrostatic 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 intussusception 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 ultrasonography to monitor the reduction
of the intussusception. Ultrasonography has many advantages such as absence of radiation
exposure, better visualization of the intussusception and its reduction, and the possibility to
detect and recognize pathological lead points (2, 13). It was speculated that detection of
perforation during the procedure could be less accurate with ultrasonography compared 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
duration of symptoms (usually more than 24 hours), early recurrence, 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 success 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 buscopan (3). In
addition, glucagon has been studied in three randomised trials to increase reduction rates of
intussusception, 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
anesthesia compared to the use of sedation for enema reduction (28), while an older study
showed no significant difference comparing sedation to general anesthesia to perform an
enema reduction (29). Because of the promising 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 aggressive enema reduction approach, too high
reduction pressures and patient selection, as in some centers patients considered to be at risk
for perforation directly undergo 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 symptoms 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 attempted reduction, where the apposition of the two bowel segments
could prevent the escape of intraluminal air into the peritoneal cavity. When reduction is
achieved, air escapes and this gives the typical clinical and radiological picture of free
intraperitoneal air (31).
Perforation with air during air enema reduction might cause a tension
pneumoperitoenum, in which intraperitoneal air under pressure causes life-threatening
ventilatory and hemodynamic compromise. This is prevented by discontinuing the enema and
releasing the air from the colon (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 children in their review who complicated with perforation during air enema reduction
needed a bowel resection.
In the past, when intussusception was found to be irreducible after a first attempt of
enema reduction, immediate surgery was standard practice. However, by the time laparotomy
was performed some cases of intussusception 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 intussusception, thus achieving partial reduction (1, 3, 34). This because
it is believed that a partial reduction and time interval between two attempts allow venous
congestion 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 surgery 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 different 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
Mediterranean, 77% in Africa to 86% in Central and South America (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 intussusception 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 indicated in cases of
failure of the conservative therapy (1-3, 13, 36). In addition, surgery is more often
recommended 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 laparoscopy, 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 gastrointestinal 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 procedure. Apelt and colleagues reviewed all laparoscopic reductions 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 laparotomy of 29%. Complications reported included:
intraoperative complications in 0,4% and postoperative complications in 2,9%. They
concluded in their review that laparoscopy was an effective and safe way to reduce
intussusceptions 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, presence
of signs of peritonitis at primary clinical examination and the presence of a pathological lead
point (39). Laparoscopy is nowadays assumed to reduce hospital length of stay, postoperative
complications especially wound infections, postoperative pain and improves cosmetic results.
But in this specific setting, we lack prospective 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 technique (13, 40-42). One study found
that recurrence rates tended to increase with the number of recurrent episode (41). After a
first enema reduction, the recurrence rate was found to be approximately 15,7%. After a
second 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 reduction 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 reduction and in case of a previous successful
surgical reduction. 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). Another 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 (usually 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 patient.
Besides the lack of adequate diagnostic criteria for intussusception, a recognized
severity score for this condition 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 intussusceptions (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 intussusception. The severe form is best described as
being similar to the indications for primary surgery. A moderate intussusception includes
criteria such as factors known to decrease 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 leucocytes, 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 intussusception.
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 surgical intervention and associated surgical complications (23, 24). As
mentioned, a case serie from Nigeria reported that a delayed presentation predisposed to
bowel complications with an increased irreducibility and presence 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 healthcare 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 condition 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, prospective,
preferable randomized studies are warranted to establish firm evidence regarding treatment
guidelines for this condition.