Intussusception: Pathophysiology
Intussusception: Pathophysiology
Intussusception: Pathophysiology
Intussusception
Alexandra C. Maki Mary E. Fallat
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.
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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).
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
38 Intussusception
533
Ultrasonography
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.
534
SECTION IV Abdomen
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.
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).
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.)
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.
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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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.
38 Intussusception
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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