7/31/2018 Intussusception: Practice Essentials, Background, Etiology and Pathophysiology

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Intussusception
Updated: Jul 03, 2017
Author: A Alfred Chahine, MD; Chief Editor: Carmen Cuffari, MD more...

OVERVIEW

Practice Essentials
Intussusception (see the image below) is a process in which a segment of intestine invaginates into
the adjoining intestinal lumen, causing bowel obstruction. With early diagnosis, appropriate fluid
resuscitation, and therapy, the mortality rate from intussusception in children is less than 1%. If left
untreated, however, this condition is uniformly fatal in 2-5 days.

Air contrast enema shows intussusception in the cecum.

History

The patient with intussusception is usually an infant, often one who has had an upper respiratory
infection, who presents with the following symptoms:

Vomiting: Initially, vomiting is nonbilious and reflexive, but when the intestinal obstruction occurs,
vomiting becomes bilious

Abdominal pain: Pain in intussusception is colicky, severe, and intermittent

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Passage of blood and mucus: Parents report the passage of stools, by affected children, that
look like currant jelly; this is a mixture of mucus, sloughed mucosa, and shed blood; diarrhea can
also be an early sign of intussusception

Lethargy: This can be the sole presenting symptom of intussusception, which makes the
condition’s diagnosis challenging

Palpable abdominal mass

Physical examination

The hallmark physical findings in intussusception are a right hypochondrium sausage-shaped mass
and emptiness in the right lower quadrant (Dance sign). This mass is hard to detect and is best
palpated between spasms of colic, when the infant is quiet. Abdominal distention frequently is found if
the obstruction is complete.

See Clinical Presentation for more detail.

Diagnosis
Imaging studies used in the diagnosis of intussusception include the following:

Radiography: Plain abdominal radiography reveals signs that suggest intussusception in only
60% of cases

Ultrasonography: Hallmarks of ultrasonography include the target and pseudokidney signs

Contrast enema: This is the traditional and most reliable way to make the diagnosis of
intussusception in children

See Workup for more detail.

Management

Nonoperative reduction

Therapeutic enemas include the following:

Hydrostatic: With barium or water-soluble contrast

Pneumatic: With air insufflation; this is the treatment of choice in many institutions, and the risk
of major complications with this technique is small

Surgical reduction

Traditional entry into the abdomen is through a right paraumbilical incision. The intussusception is
delivered into the wound, and manual reduction is attempted. It is important that the intussusception
be milked out of the intussuscipiens.

If manual reduction is not possible or perforation is present, a segmental resection with an end-to-end
anastomosis is performed.

Laparoscopy has been added to the surgical armamentarium for intussusception [1, 2] and can be
performed in all cases of intussusception.

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See Treatment and Medication for more detail.

Background
Intussusception is a process in which a segment of intestine invaginates into the adjoining intestinal
lumen, causing bowel obstruction. A common cause of abdominal pain in children, intussusception is
suggested readily in pediatric practice based on a classic triad of signs and symptoms: vomiting,
abdominal pain, and passage of blood per rectum. (See History and Physical Examination.)

Intussusception presents in 2 variants: idiopathic intussusception, which usually starts at the ileocolic
junction and affects infants and toddlers, and enteroenteral intussusception (jejunojejunal, jejunoileal,
ileoileal), which occurs in older children. The latter is associated with special medical situations (eg,
Henoch-Schönlein purpura [HSP], cystic fibrosis, hematologic dyscrasias) or may be secondary to a
lead point and occasionally occur in the postoperative period. Intussusception is demonstrated in the
images below. (See Etiology and Pathophysiology.)

Abdominal radiograph shows small bowel dilatation and paucity of gas in the right lower and upper quadrants.

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Air contrast enema shows intussusception in the cecum.

Barium enema shows intussusception in the descending colon.

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CT scan reveals the classic ying-yang sign of an intussusceptum inside an intussuscipiens.

Abdominal ultrasonography reveals the classic target sign of an intussusceptum inside an intussuscipiens.

Laparoscopic view of a jejuno-jejunal intussusception

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Go to Pediatric Intussusception Surgery for complete information on this topic.

Patient education
Educate parents and caregivers of a patient treated with nonoperative reduction with regard to the
risks and signs and symptoms of recurrence so that the initiation of care is not delayed.

For patient education information, see the Digestive Disorders Center, as well as Abdominal Pain in
Children, Colic, Rectal Bleeding, and Barium Enema.

Etiology and Pathophysiology

The pathogenesis of idiopathic intussusception is not well established. It is believed to be secondary
to an imbalance in the longitudinal forces along the intestinal wall. In enteroenteral intussusception,
this imbalance can be caused by a mass acting as a lead point or by a disorganized pattern of
peristalsis (eg, an ileus in the postoperative period).

As a result of imbalance in the forces of the intestinal wall, an area of the intestine invaginates into the
lumen of adjacent bowel. The invaginating portion of the intestine (ie, the intussusceptum) completely
“telescopes” into the receiving portion of the intestine (ie, the intussuscipiens). This process continues
and more proximal areas follow, allowing the intussusceptum to proceed along the lumen of the
intussuscipiens.

If the mesentery of the intussusceptum is lax and the progression is rapid, the intussusceptum can
proceed to the distal colon or sigmoid and even prolapse out the anus. The mesentery of the
intussusceptum is invaginated with the intestine, leading to the classic pathophysiologic process of
any bowel obstruction.

Early in this process, lymphatic return is impeded; then, with increased pressure within the wall of the
intussusceptum, venous drainage is impaired. If the obstructive process continues, the pressure
reaches a point at which arterial inflow is inhibited, and infarction ensues. The intestinal mucosa is
extremely sensitive to ischemia because it is farthest away from the arterial supply. Ischemic mucosa
sloughs off, leading to the heme-positive stools and subsequently to the classic "currant jelly stool" (a
mixture of sloughed mucosa, blood, and mucus). If untreated, transmural gangrene and perforation of
the leading edge of the intussusceptum occur.

Lead points

In approximately 2-12% of children with intussusception, a surgical lead point is found. Occurrence of
surgical lead points increases with age and indicates that the probability of nonoperative reduction is
highly unlikely. Examples of lead points are as follows:

Meckel diverticulum [3]

Enlarged mesenteric lymph node

Benign or malignant tumors of the mesentery or of the intestine, including lymphoma, polyps,
ganglioneuroma, [4] and hamartomas associated with Peutz-Jeghers syndrome

Mesenteric or duplication cysts

Submucosal hematomas, which can occur in patients with HSP and coagulation dyscrasias

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Ectopic pancreatic and gastric rests

Inverted appendiceal stumps

Sutures and staples along an anastomosis

Intestinal hematomas secondary to abdominal trauma

Foreign body

Hemangioma

Kaposi sarcoma [5]

Post-transplantation lymphoproliferative disorder (PTLD) [6]

Henoch-Schönlein purpura
Children with HSP often present with abdominal pain secondary to vasculitis in the mesenteric,
pancreatic, and intestinal circulation. If pain precedes cutaneous manifestations, differentiating HSP
from appendicitis, gastroenteritis, intussusception, or other causes of abdominal pain is difficult.

Occasionally, children with HSP develop submucosal hematomas, which can act as lead points and
cause small bowel intussusception. Elucidating the cause of the pain is essential in any child in whom
HSP is suspected.

Since the intussusception associated with HSP is usually enteroenteral (small bowel to small bowel),
these patients require surgery rather than an enema.

During the initial investigation, obtain supine and upright plain radiographs of the abdomen to identify
the small bowel obstruction associated with intussusception. If radiographic findings are normal,
assume the patient with HSP has mesenteric vasculitis and treat with steroids.

Hemophilia and other coagulation disorders

Patients with hemophilia and other bleeding disorders may develop intestinal submucosal
hematomas, leading to intussusception. Differential diagnosis includes retroperitoneal hemorrhage in
addition to other usual causes of abdominal pain. Radiographs of the abdomen should reveal a
pattern of small bowel obstruction if intussusception is present. In the absence of intussusception,
treatment is supportive with correction of coagulopathy.

Postoperative intussusceptions
Intussusception is a rare postoperative complication, occurring in 0.08-0.5% of laparotomies. It can
take place independently of the site of the operation. The likely mechanism is due to a difference in
activity between segments of the intestine recovering from an ileus, which produces the
intussusception. [7] Intussusception is suggested in any postoperative patient who has a sudden onset
of a small bowel obstruction after a period of ileus, usually within the first 2 weeks after surgery.
Intestinal obstruction secondary to adhesions usually occurs more than 2 weeks after the operation.
The treatment is prompt operative reduction.

Indwelling catheters

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Very rarely, indwelling jejunal catheters can lead to intussusception by acting as a lead point, which is
especially true if the tip of the catheter has been manipulated or cut so that its surface is not smooth.
The clinical picture is that of a small bowel obstruction. Diagnosis can be facilitated by injecting
contrast proximal to the catheter and then through the tip of the catheter. Surgery is required to
remove the tip of the catheter and to reduce the intussusception.

Cystic fibrosis
Intussusception occurs in approximately 1% of patients with cystic fibrosis. Intussusception is
assumed to be precipitated by the thick, inspissated stool material that adheres to the mucosa and
acts as a lead point. Often, the course is indolent and chronic. Differential diagnosis includes distal
intestinal obstruction syndrome and appendicitis. The majority of these patients require operative
reduction.

Other causes

Electrolyte derangements associated with various medical conditions can produce aberrant intestinal
motility, leading to enteroenteral intussusception.

Experimental studies in animals showed that abnormal intestinal release of nitric oxide, an inhibitory
neurotransmitter, caused relaxation of the ileocecal valve, predisposing to ileocecal intussusception.
[8] Other studies have demonstrated that certain antibiotics cause ileal lymphoid hyperplasia and
intestinal dysmotility, with resultant intussusception.

A viral etiology has also been implicated. A seasonal variation in the incidence of intussusception that
corresponds to the peaks in frequency of gastroenteritis (spring and summer) and respiratory illnesses
(midwinter) has been described. Lappalainen et al have studied prospectively the role of viral
infections in the pathogenesis of intussusception. They concluded that the simultaneous presence of
human herpesvirus-6 and adenovirus infections appeared to correlate with risk for intussusception. [9]

An association was found between the administration of a rotavirus vaccine (RotaShield) and the
development of intussusception. [10] RotaShield has since been removed from the market. These
patients were younger than usual for idiopathic intussusception and were more likely to require
operative reduction. It was hypothesized that the vaccine caused reactive lymphoid hyperplasia, which
acted as a lead point.

In February 2006, a new rotavirus vaccine [RotaTeq] was approved by the US Food and Drug
Administration [FDA]. RotaTeq did not show an increased risk for intussusception compared with
placebo in clinical trials. [11] A study that involved more than 63,000 patients who received Rotarix or
placebo at ages 2 and 4 months reported a decreased risk for intussusception in those patients
receiving Rotarix. [12] However, a 2015 study reported that there was a small increase in the risk of
intussusception hospitalizations in infants in California since the introduction of rotavirus vaccines,
RotaTeq (2006) and Rotarix (2008). [13, 14]

A study by Tate et al that analyzed the intussusception hospitalization rates before (2000–2005) and
after (2007–2013) rotavirus vaccine introduction reported that when compared to the prevaccine
baseline (11.7 per 100,000), the intussusception hospitalization rate significantly increased by 46% to
101% (16.7-22.9 per 100,000) in children 8-11 weeks of age in the years after the rotavirus vaccine
was introduced. No significant change in hospitalization rates were seen in children < 12 months of
age and in children 15-24 weeks and 25-34 weeks of age. [15]

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Analysis of data from the Kid’s Inpatient Database in the United States has shown a lower than
expected rate of hospital discharges for intussusception in infants since the reintroduction of the
rotavirus vaccine in 2006. [16]

Familial occurrence of intussusception has been reported in a few cases. Intussusception in dizygotic
twins has also been described; however, these reports are extremely rare.

Idiopathic
In most infants and toddlers with intussusception, the etiology is unclear. This group is believed to
have idiopathic intussusception. One theory to explain the possible etiology of idiopathic
intussusception is that it occurs because of an enlarged Peyer patch; this hypothesis is derived from 3
observations: (1) often, the illness is preceded by an upper respiratory infection, (2) the ileocolic
region has the highest concentration of lymph nodes in the mesentery, and (3) enlarged lymph nodes
are often observed in patients who require surgery. Whether the enlarged Peyer patch is a reaction to
the intussusception or a cause of it is unclear.

Epidemiology
A wide geographic variation in incidence of intussusception among countries and cities within
countries makes determining a true prevalence of the disease difficult. Studies on the absolute
prevalence of intussusception in the United States are not available. Its estimated incidence is
approximately 1 case per 2000 live births. In Great Britain, incidence varies from 1.6-4 cases per 1000
live births.

Overall, the male-to-female ratio is approximately 3:1. With advancing age, gender difference
becomes marked; in patients older than 4 years, the male-to-female ratio is 8:1.

Two thirds of children with intussusception are younger than 1 year; most commonly, intussusception
occurs in infants aged 5-10 months. Intussusception is the most common cause of intestinal
obstruction in patients aged 5 months to 3 years.

Intussusception can account for as many as 25% of abdominal surgical emergencies in children
younger than 5 years, exceeding the incidence of appendicitis. Although extremely rare,
intussusception has been reported in the neonatal period.

Prognosis
The prognosis in patients with intussusception is excellent if the condition is diagnosed and treated
early; otherwise, severe complications and death may occur.

The recurrence rate of intussusception after nonoperative reduction is usually less than 10% but has
been reported to be as high as 15%. [17] Most intussusceptions recur within 72 hours of the initial
event; however, recurrences have been reported as long as 36 months later. More than 1 recurrence
suggests the presence of a lead point. A recurrence is usually heralded by the onset of the same
symptoms as appeared during the initial event. Provide similar treatment for a recurrence unless the
suggestion of a lead point is very strong (in which case, surgical exploration should be contemplated).

The recurrence rates after air enema and barium enema are 4% and 10%, respectively. Recurrences
respond to nonoperative reduction in almost 95% of cases.

Complications associated with intussusception, which rarely occur when the diagnosis is prompt,
include the following:
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Perforation during nonoperative reduction

Wound infection

Internal hernias and adhesions causing intestinal obstruction

Sepsis from undetected peritonitis (major complication from a missed diagnosis)

Intestinal hemorrhage

Necrosis and bowel perforation

Recurrence

With early diagnosis, appropriate fluid resuscitation, and therapy, the mortality rate from
intussusception in children is less than 1%. If left untreated, this condition is uniformly fatal in 2-5
days.

Clinical Presentation

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