Intussusception: Pathophysiology

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C H A P T E R 3 8

Intussusception
Alexandra C. Maki Mary E. Fallat

Intussusception is the most frequent cause of bowel


obstruction in infants and toddlers. It is an acquired
invagination of the proximal bowel (intussusceptum) into
the distal bowel (intussuscipiens). It was first described in
1674 by Paul Barbette of Amsterdam, defined by Treves
in 1899, and operated on successfully in 1873 by John
Hutchinson.1,2

PATHOPHYSIOLOGY
The intussusceptum telescopes into the distal bowel by
peristaltic activity. There may or may not be a lead point.
As the mesentery of the proximal bowel is drawn into
the distal bowel, it is compressed, resulting in venous
obstruction and bowel wall edema. If reduction of the
intussusception does not occur, arterial insufficiency
will ultimately lead to ischemia and bowel wall necrosis.
Although spontaneous reduction can occur, the natural
history of an intussusception is to progress to bowel
ischemia and necrosis unless the condition is recognized
and treated appropriately.

Primary Intussusception
The vast majority of cases do not have a lead point and are
classified as primary or idiopathic intussusceptions. The
cause is generally attributed to hypertrophied Peyer
patches within the bowel wall.3 Intussusception occurs
frequently in the wake of an upper respiratory tract infection or an episode of gastroenteritis, providing an etiology
for the hypertrophied lymphoid tissue. Adenoviruses in
children older than age two, and to a lesser extent rotaviruses, have been implicated in up to 50% of cases.4,5 Other
contributing evidence that viruses may play a role in
intussusception includes the rise in cases during seasonal
respiratory viral illnesses and the increased risk associated
with previous rotavirus immunization.6 The newest
immunization formulas available in the USA, RotaTeq
and Rotarix, have not been associated with intussusception in both pre- and post-marketing studies.610

Secondary Intussusception
An intussusception may have an identifiable lesion that
serves as a lead point, drawing the proximal bowel into
the distal bowel by peristaltic activity. The incidence of
a lead point varies from 1.5% to 12% and the presence
of a lead point increases in proportion with age.11,12 The

most common lead point is a Meckel diverticulum followed by polyps and duplications. Other benign lead
points include the appendix, hemangiomas, carcinoid
tumors, foreign bodies, ectopic pancreas or gastric
mucosa, hamartomas from PeutzJeghers syndrome (Fig.
38-1), and lipomas. Malignant causes, although rare,
increase in incidence with age and include lymphomas
and small bowel tumors.13 Systemic diseases, including
HenochSchnlein purpura and cystic fibrosis, have been
associated with intussusception. Other rare diseases
related to intussusception are celiac disease and Clostridium difficile colitis.14

INCIDENCE
Idiopathic intussusception can occur at any age. Most
patients are well-nourished, healthy infants, and approximately two-thirds are boys. The highest incidence occurs
in infants between ages 4 and 9 months,15 and it is also
the most common cause of small bowel obstruction in
this age group.16 Intussusception is uncommon below 3
months and after 3 years of age. The condition has been
described in premature infants where it has been postulated as the cause of small bowel atresia in some cases.17

CLINICAL PRESENTATION
The classic presentation is an infant or a young child with
intermittent, crampy abdominal pain associated with
currant jelly stools and a palpable mass on physical
examination, although this triad is seen in less than a
fourth of children.18 The abdominal pain is sudden and
the child may stiffen and pull the legs up to the abdomen.
The pain can also be associated with hyperextension,
writhing, breath holding and vomiting. The attack often
ceases as suddenly as it started. Between attacks, the child
may appear comfortable but eventually will become
lethargic. Small or normal bowel movements will stop as
the obstruction progresses and becomes associated with
bilious emesis and increasing abdominal distention.
Stools may be blood tinged as impending ischemia causes
mucosal sloughing and compression of mucous glands
leading to evacuation of dark, red mucoid clots or currant
jelly stools. This is often a late sign as are laboratory
derangements. A pitfall is to wait for the currant jelly
stool, leukocytosis, and electrolyte abnormalities that are
often hallmarks of ischemic bowel.
531

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532

SECTION IV Abdomen

FIGURE 38-1 (A) Operative view of the outside of the jejunum shows a palpable mass as the lead point of a reduced intussusception. (B) A hamartomatous polyp is characteristic of PeutzJeghers syndrome. (C) Mucocutaneous macular lesions are seen in this
patient with PeutzJeghers syndrome. Note extension of the pigmentation beyond the vermilion border.

FIGURE 38-2 This 10-year-old boy has a palpable sausageshaped mass (arrows) due to an intussusception.

PHYSICAL EXAMINATION
The childs vital signs are usually normal early in the
disease course. During painless intervals, the child may
appear comfortable and the physical examination may be
unremarkable. However, the cramping episodes usually
occur every 15 to 30 minutes and re-examination may
prove difficult. There may be audible peristaltic rushes,
and a sausage-shaped or curved mass might be palpable
anywhere in the abdomen or even visualized if the child
is relatively thin (Fig. 38-2). The right lower abdominal
quadrant can appear flat or empty (Dance sign) as the
intussuscepted mass is drawn cephalad. On rectal examination, bloodstained mucus or blood may be encountered
as a later sign. If the obstructive process worsens and
bowel ischemia occurs, dehydration, fever, tachycardia,
and hypotension can develop in quick succession as a
result of bacteremia and bowel necrosis.
Prolapse of the intussusceptum through the anus is a
grave sign, particularly when the intussusceptum is
ischemic. The greatest danger in a case of prolapsed
intussusceptum is that the examiner will misdiagnose the
condition as a rectal prolapse and attempt reduction.

FIGURE 38-3 This abdominal radiograph in a patient with intussusception shows dilated loops of small bowel in the right lower
quadrant and a right upper quadrant soft tissue mass density
in the vicinity of the transverse colon near the hepatic flexure
(arrow).

Careful physical examination is mandatory and done by


inserting a lubricated tongue blade along the side of the
protruding mass before reduction. If the blade can be
inserted more than 12cm into the anus along the side
of the mass, the diagnosis of intussusception should be
considered.

DIAGNOSIS
Abdominal Radiography
In half of cases, the diagnosis of intussusception can be
suspected on plain flat and upright abdominal radiographs (Fig. 38-3). Suggestive radiographic abnormalities
include an abdominal mass, abnormal distribution of gas
and fecal contents, sparse large bowel gas, and air-fluid
levels in the presence of bowel obstruction. However,
plain films have limited value in confirming the diagnosis
and are not used as the sole diagnostic test. They are best

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38 Intussusception

533

utilized as a screening tool when one of the abnormal


findings listed above is found.19

intussusception.21 Equivocal findings using this modality


should mandate a conventional contrast or air enema.23

Ultrasonography

Computed Tomography and Magnetic


Resonance Imaging

The use of abdominal ultrasound (US) for the evaluation


of intussusception was first described in 1977.20 Since
then, most institutions have adopted it as a screening tool
because of the lack of radiation exposure, ability to identify pathologic lead points, and decreased cost.21,22 The
characteristic finding on ultrasound has been referred to
as a target or doughnut lesion (Fig. 38-4), which consists of alternating rings of low and high echogenicity
representing the bowel wall and mesenteric fat within
the intussusceptum in a transverse plane. The pseudo
kidney sign is seen on longitudinal section (Fig. 38-5).
This pattern is secondary to the edematous walls of the
intussusceptum within the intussuscipiens. Ultrasonography can also guide the therapeutic reduction of an

FIGURE 38-4 This transverse sonographic image shows the


alternating rings of low and high echogenicity due to an intussusception. This finding has been called a target sign.

Neither computed tomography (CT)24 nor magnetic


resonance imaging (MRI) are routinely used in the evaluation of a patient with intussusception, although either
may confirm this diagnosis and/or pathologic causes for
intussusception, such as a malignancy (i.e., lymphoma).
The characteristic CT finding is a target or doughnut
sign (Fig. 38-6). Transient small bowel intussusceptions
that are discovered on CT or MRI are usually not clinically significant.21 Radiographic or operative treatment

FIGURE 38-5 Sonogram showing the pseudokidney sign seen


with intussusception on longitudinal section.

FIGURE 38-6 Concurrent contrast enema and pelvic CT images of an intussusception. (A) Contrast study showing the intussusception
low in pelvis. (B) CT image of the intussusception. (C) CT image of the layered intussuscepted mass. This is the target sign on CT.

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534

SECTION IV Abdomen

should be based on clinical findings in symptomatic


patients.25 Laparoscopy is an excellent means to evaluate
these patients if surgical intervention is needed.

NONOPERATIVE MANAGEMENT
If the diagnosis of intussusception is suspected, a nasogastric tube may be helpful to decompress the stomach.
Bowel rest and intravenous fluid resuscitation should be
initiated. A complete blood cell count and serum electrolytes are obtained. An air or contrast enema is first-line
treatment as long as there are no contraindications to
nonoperative reduction. Contraindications include intestinal perforation (free intra-abdominal air), peritonitis, or
persistent hypotension. The advantages of nonoperative
reduction are decreased morbidity, cost, and length of
hospitalization.

Hydrostatic and Pneumatic Reduction


The methodology for hydrostatic reduction has not
changed significantly since its first description in 1876.26
Hydrostatic reduction with barium under fluoroscopic
guidance has historically been used.27 More recently, childrens hospitals have transitioned to air or water-soluble
isotonic contrast because of the potential hazard of
barium peritonitis in patients with intestinal perforation16,28. Successful reduction (Fig. 38-7) in uncomplicated patients is seen in about 85% of cases and ranges
from 42% to 95%.29
Pneumatic reduction was first described in 1897.30 It
gained popularity in the late 1980s. Since then, many
institutions have adopted pneumatic decompression
because it is quicker, safer, less messy, and decreases the
exposure time to radiation.31 The procedure is fluoroscopically monitored as air is insufflated into the rectum
(Fig. 38-8). The maximum safe air pressure is 80mmHg

for younger infants and 110120mmHg for older infants.


Potential drawbacks of pneumatic reduction include the
possibility of developing tension pneumoperitoneum,
and poor visualization of lead points and/or the intussusception reduction process, resulting in false-positive
reductions.3234 Rates of perforation range from 0.42.5%
with the most recent publications citing an average rate
of 0.8%.16,35
Tension pneumoperitoneum is best treated with
immediate cessation of the procedure and immediate
release of the pneumoperitoneum using a 14, 16, or
18-gauge needle or angiocatheter above or below the
umbilicus. This should be followed by immediate operative exploration.36
For unsuccessful reduction, several studies have shown
improved reduction rates using a second attempt after
waiting between 30 minutes to 24 hours after the initial
attempt.28 In some instances, this is done in the operating
room prior to laparoscopy or in conjunction with laparoscopic reduction.37
If nonoperative reduction is successful either by
hydrostatic or pneumatic technique, the patient should
be admitted for observation, receive a short period of
bowel rest, and given intravenous fluids. Any clinical
signs of abdominal pain after reduction could be a sign
of ischemic bowel or recurrent intussusception and repeat
ultrasound is necessary.

OPERATIVE MANAGEMENT
Open Approach
An operation is needed when nonoperative reduction is
unsuccessful or incomplete, for signs of peritonitis, the
presence of a lead point, or radiographic evidence of
pneumoperitoneum. Preoperative preparation includes
administration of broad-spectrum antibiotics, intravenous

FIGURE 38-7 Fluoroscopic examination using isotonic contrast for hydrostatic reduction of intussusception. (A) Intussusception
(arrow) seen in midtransverse colon. (B) Reduction has occurred to the hepatic flexure. (C) Complete reduction with reflux of contrast
medium into the terminal ileum. Note the edematous ileocecal valve (arrow).

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38 Intussusception

AA

535

FIGURE 38-8 Plain radiography and fluoroscopic examination using air for pneumatic reduction of an intussusception. (A) Plain
radiograph showing a mass effect in the right upper quadrant. (B) Pneumatic reduction to the vicinity of the cecum with the intussusception still present (arrow). (C) Complete reduction with reflux of air into multiple loops of small intestine. (Courtesy of Charles
Maxfield, MD.)

finding of ischemic bowel, or identification of a lead point


requires resection and bowel anastomosis or diversion,
depending on the condition of the bowel and child.
Ileopexy has been described in patients with recurrent
intussusception after operative reduction.38 However, in
a series of 278 patients, this technique was not shown to
reduce re-intussusception rates when compared to operative reduction and resection of the affected area.39
If surgical reduction is possible, the bowel is then
evaluated for viability, perforation, or a lead point. Questionable ischemic bowel can be warmed with salinesoaked laparotomy pads and re-evaluated. After complete
reduction of the intussusception, an incidental appendectomy is often performed because the location of the
abdominal scar is similar to an open appendectomy
incision.

Laparoscopic Approach
FIGURE 38-9 A right lower quadrant muscle-splitting incision
allows delivery of the intussusception through the incision.
Gentle and continuous massage from distal to proximal usually
results in reduction of the intussusception.

fluid resuscitation, insertion of a urinary catheter, and


placement of a nasogastric tube for gastric decompression.
Most commonly, the cecum and terminal ileum are
involved, and can be delivered through the traditional
right lower abdominal incision (Fig. 38-9). It is important
to evaluate the extent of the intussusceptum before delivering it as it can extend into the rectosigmoid region in
severe cases which usually requires extension of the
incision.
Once the leading edge of the intussusceptum is identified, it is gently manipulated back toward its normal position in the terminal ileum. Excessive force or pulling is
avoided to prevent injury or perforation of the bowel.
Inability to manually reduce the intussusception, the

Initially, the use of laparoscopy in the operative management of intussusception was strictly diagnostic, or was
used in cases with equivocal radiographic studies or in
patients with suspected lead points, and was associated
with conversion rates in up to 70% of cases.40 More
recently, there has been increased success with laparoscopic reduction with some studies showing conversion
rates as low as 5.4%41 but more in the range of
1240%.37,4244
Where laparoscopy fits into a surgeons therapeutic
algorithm is a topic frequently discussed. It would be
beneficial to identify any preoperative risk factors. No
study to date has specifically addressed this topic although
some have noted an increased conversion rate associated
with lead points. Recently, a retrospective analysis of
65 cases found that in patients unable to be reduced
laparoscopically, 33% had a lead point that necessitated
conversion to open (Fig. 38-10).45 Contraindications to
laparoscopy include peritonitis, hemodynamic instability,
and severe bowel distension that precludes adequate
visualization.41

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536

SECTION IV Abdomen

FIGURE 38-10 (A) This laparoscopic photograph shows an incompletely reduced intussusception with the intussusceptum (white
arrow) telescoping into the intussuscipiens (black arrow). (B) A pathologic lead point due to a Burkitt lymphoma was found requiring
conversion to open.

FIGURE 38-11 Laparoscopic reduction of intussusception with hypertrophied lymph nodes is depicted in these four operative photographs. (A) Intussusceptum (white arrow) is seen telescoping into the intussuscipiens (black arrow). (B) The intussusception has
almost been completely reduced. (C) This intussusception has been completely reduced and the bowel appears viable. (D) Hypertrophied mesenteric lymphadenopathy (arrows) is seen. This lymphadenopathy may reflect a recent viral illness.

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38 Intussusception

The majority of minimally invasive approaches


describe the use of three abdominal ports: one in the
infraumbilical region with two other ports along the
left side of the abdomen. Laparoscopic reduction is
accomplished by applying gentle pressure distal to the
intussusceptum using atraumatic graspers. Although
counterintuitive to the conventional open method, traction is usually required proximal to the intussuscipiens to
complete the reduction (Figs. 38-11). Appendectomy is
not routinely performed with laparoscopic reduction and
up to the surgeons discretion. Careful inspection of the
bowel is performed to evaluate for any signs of ischemia,
necrosis, or perforation. A criticism of laparoscopic
reduction is the loss of tactile sense that can lead to
missed pathology. If resection is required, this can often
be accomplished by exteriorizing the bowel by enlarging
the periumbilical incision. If this is not possible, the
operation should be converted to a laparotomy.

RECURRENT INTUSSUSCEPTION
Recurrent intussusception has been described in association with nonoperative intervention in 1015% of cases,
with about one-third occurring within 24 hours and the
majority within 6 months of the initial episode.46 Recurrences are less likely to occur after operative reduction or
resection.47 After laparoscopic reduction, a recurrence
rate as high as 10% has been reported.37
Patients with recurrent intussusception tend to be
seen earlier in their course because their parents are more
aware of how to recognize the signs and symptoms.
Success rates with enema reduction after one recurrence
are comparable to those with the first episode and are
better if the child did not previously require operative
reduction. This finding has led to a nonoperative approach
for initial management of recurrence in most patients as
long as they are not toxic or show signs of peritonitis or
hemodynamically instability.29,46 A concern in recurrent
intussusception is occult malignancy. Unfortunately, the
clinical findings or pattern of recurrence do not predict
the presence of a malignant lead point and radiographic
reduction with ultrasound is recommended to look for an
occult pathology.48,49

POSTOPERATIVE INTUSSUSCEPTION
Postoperative intussusception is a rare clinical entity that
has been described after ileocolic intussusception reduction and resection, retroperitoneal dissections, long
intra-abdominal procedures, a Ladd procedure, or extraabdominal operations.50,51 It accounts for 3% to 10% of
cases of postoperative bowel obstruction and most often
occurs in the initial 10 days following a procedure.52,53
Ileus and adhesive obstruction are more frequently
encountered as a cause for intestinal obstruction in the
postoperative patient. Thus, an index of suspicion is
needed and ultrasound is a useful diagnostic tool.51 Most
postoperative intussusceptions are ileoileal and respond
to operative reduction without resection.

537

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