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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

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.

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.
Air contrast enema shows intussusception in the cecum.

Barium enema shows intussusception in the descending colon.

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

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

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
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]

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:

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