eMedicine Specialties > Pediatrics: General Medicine > Gastroenterology
Intussusception
Updated: Feb 4, 2010
Introduction
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 (see Clinical). 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) and can occur secondary to a lead point and occasionally in the postoperative period. This discussion concentrates on idiopathic intussusception, which is the more common of the 2 variants.
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
Pathophysiology
The pathogenesis of intussusception is believed to be secondary to an imbalance in the longitudinal forces along the intestinal wall. 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). Electrolyte derangements associated with various medical problems can produce aberrant intestinal motility, leading to its invagination. Recent experimental studies in animals showed that abnormal intestinal release of nitric oxide,1 an inhibitory neurotransmitter, caused relaxation of the ileocecal valve predisposing to ileocecal intussusception. Other studies have demonstrated that the use of certain antibiotics leads to ileal lymphoid hyperplasia and intestinal dysmotility with resultant intussusception.
As a result of the imbalance, an area of the intestinal wall invaginates into the lumen, with the rest of the intestine following. The invaginating portion of the intestine (ie, intussusceptum) completely invaginates into the receiving portion of the intestine (ie, 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 the rise in the pressure within the wall of the intussusceptum, venous drainage is impaired. Finally, the pressure reaches a point at which arterial inflow is inhibited, and infarction ensues. The mucosa is most sensitive to ischemia because it is farthest away from the arterial supply. Ischemic mucosa sloughs off, which initially leads to the heme-positive stools and then the classic "currant jelly stool" (a mixture of sloughed mucosa, blood, and mucus). If untreated, the process progresses to transmural gangrene and perforation of the leading edge of the intussusceptum.
Frequency
United States
A wide geographic variation in incidence of intussusception among countries and cities within a country makes determining a true prevalence of the disease difficult. Studies for the absolute prevalence of intussusception in the United States are not available. Its estimated incidence is approximately 1 case per 2000 live births.
International
In Great Britain, incidence varies from 1.6-4 cases per 1000 live births.
Mortality/Morbidity
With early diagnosis, appropriate fluid resuscitation, and therapy, the mortality rate from intussusception in children is less than 1%. The morbidity rate is very low after treatment of intussusception.
Race
No significant difference in the incidence of intussusception is reported between races.
Sex
Most series report a slight preponderance of males, with a male-to-female ratio of approximately 3:2.
Age
Two thirds of children with intussusception are younger than 1 year; most commonly, intussusception occurs in infants aged 5-10 months. Although extremely rare, intussusception has been reported in the neonatal period. Intussusception can account for as many as 25% of abdominal surgical emergencies in children younger than 5 years, exceeding the incidence of appendicitis. Intussusception is the most common cause of intestinal obstruction in patients aged 5 months to 3 years.
From a clinical perspective, using a cutoff age of 3 years is helpful for dividing patients with intussusception into 2 groups. Patients aged 5 months to 3 years who have intussusception rarely have a lead point (ie, idiopathic intussusception) and are usually responsive to nonoperative reduction. Older children and adults more often have a surgical lead point to the intussusception and require operative reduction.
Clinical
History
The constellation of signs and symptoms of intussusception represents one of the most classic presentations of any pediatric illness; however, the classic triad of vomiting, abdominal pain, and passage of blood per rectum occurs in only one third of patients. The patient is usually an infant who presents with vomiting, abdominal pain, passage of blood and mucus, lethargy, and a palpable abdominal mass. These symptoms are often preceded by an upper respiratory infection. In rare circumstances, the parents report one or more previous attacks of abdominal pain within 10 days to 6 months prior to the current episode. These patients are more likely to have a surgical lead point causing recurrent attacks of intussusception with spontaneous reduction.
Symptoms include the following:
- Pain is colicky, severe, and intermittent. The parents or caregivers describe the child as drawing the legs up to the abdomen and kicking the legs in the air. In between attacks, the child appears calm and relieved.
- Initially, vomiting is nonbilious and reflexive, but when the intestinal obstruction occurs, vomiting becomes bilious. Any child with bilious vomiting is assumed to have a condition that must be treated surgically until proven otherwise.
- Parents also report the passage of stools that look like currant jelly. This is a mixture of mucus, sloughed mucosa, and shed blood as described in Pathophysiology.
- Lethargy is a relatively common presenting symptom with intussusception.
- The reason lethargy occurs is unknown because lethargy has not been described with other forms of intestinal obstruction.
- Lethargy can be the sole presenting symptom, which makes the diagnosis challenging. Patients are found to have an intestinal process late, after initiation of a septic workup.
- Diarrhea can also be an early sign of intussusception.
Physical
Upon physical examination, the patient is usually chubby and in good health. Intussusception is uncommon in children who are malnourished. The child is found to have periods of lethargy alternating with crying spells, and this cycle repeats every 15-30 minutes. The infant can be pale, diaphoretic, and hypotensive if shock has occurred.
- The hallmark physical findings in intussusception are a right hypochondrium sausage-shaped mass and emptiness in the right lower quadrant (Dance sign). This is hard to detect and is best palpated when the infant is quiet between spasms of colic.
- Abdominal distention frequently is found if obstruction is complete.
- If intestinal gangrene and infarction have occurred, peritonitis can be suggested on the basis of rigidity and involuntary guarding.
- Early in the disease process, occult blood in the stools is the first sign of impaired mucosal blood supply. Later on, frank hematochezia and the classic currant jelly stools appear.
- Fever and leukocytosis are late signs and can indicate transmural gangrene and infarction.
- A rare presentation of intussusception is prolapse of the intussusceptum through the anus.
- This prolapse of the intussusceptum can be confused with rectal prolapse. Careful examination can differentiate between the 2 presentations.
- The anal crypts are everted with rectal prolapse and not with intussusception.
- An examining finger can be passed between the prolapse and the anus in patients with intussusception but not in patients with rectal prolapse.
- Patients with intussusception often have no classic signs and symptoms, which can lead to an unfortunate delay in diagnosis and disastrous consequences.
- Maintaining a high index of suspicion for intussusception is essential when evaluating a child younger than 5 years who presents with abdominal pain or when evaluating a child with Henoch-Schönlein purpura (HSP) or hematologic dyscrasias.
Causes
In most infants and toddlers with intussusception, the etiology is unclear. This group is believed to have idiopathic intussusception. One theory about the 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.
- 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 diverticulum2
- Enlarged mesenteric lymph node
- Benign or malignant tumors of the mesentery or of the intestine, including lymphoma, polyps, ganglioneuroma,3 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
- Other theories have implicated a viral etiology; however, no theory has proven to be reliable.
- 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 but has not been corroborated universally.
- An association was found between the administration of a rotavirus vaccine (RotaShield) and the development of intussusception.4 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. The vaccine is hypothesized to cause a reactive lymphoid hyperplasia, acting 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.
- 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.5 The intussusception data was determined over a 31-day observation period (inpatient or outpatient) after each dose of the Rotarix vaccine; this also included a 100-day surveillance period for all serious adverse events.
- 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.
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| References |
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Further Reading
Keywords
intussusception, bowel obstruction, bowel torsion, abdominal pain, Henoch-Schönlein purpura, HSP, cystic fibrosis, hematologic dyscrasias, idiopathic intussusception, enteroenteral intussusception, jejunojejunal intussusception, jejunoileal intussusception, ileoileal intussusception, peristalsis
