AUTHOR AND EDITOR INFORMATION

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INTRODUCTION

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Background

Intussusception is a process in which a segment of intestine invaginates into the adjoining intestinal lumen, causing a 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 patients. The latter is associated with special medical situations (eg, Henoch-Schönlein purpura [HSP], cystic fibrosis, hematologic dyscrasias) and can occur in the postoperative period. This discussion concentrates on idiopathic intussusception, which is the more common of the 2 variants.

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). 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 progression is rapid, the intussusceptum can proceed to the distal colon or sigmoid and even prolapse out of 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.

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 they 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

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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 often are 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.
• 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, since 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 also can 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 can be confused with rectal prolapse. Careful examination can differentiate between the 2 presentations.
  • The anal crypts are everted with prolapse and not with intussusception.
  • An examining finger can pass 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 can have 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 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 diverticulum
  • Enlarged mesenteric lymph node
  • Benign or malignant tumors of the mesentery or of the intestine, including lymphoma, polyps, 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
• 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.¹ RotaShield has since been removed from the market. These patients were younger than usual with 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) and did not show an increased risk compared with placebo in clinical trials. A study that involved more than 63,000 patients who received Rotarix or placebo at age 2 months and age 4 months reported a decreased risk of intussusception for those patients receiving Rotarix.² 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 been reported; however, these reports are extremely rare.

DIFFERENTIALS

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Other Problems to be Considered

Milk allergy
Incarcerated hernia
Internal hernia
Other rare causes of intestinal obstruction

WORKUP

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Lab Studies

• Laboratory investigation is usually not helpful in the evaluation of patients with intussusception.
• Leukocytosis can be an indication of gangrene if the process is advanced.
• Dehydration is depicted by electrolyte imbalances.

Imaging Studies

• After obtaining a thorough history and performing a careful physical examination, obtain plain radiographs of the abdomen with the patient in the supine and upright positions.
• • Plain radiograph findings may be normal early in the course of intussusception.
  • As the disease progresses, earliest radiographic evidence includes an absence of air in the right lower and upper quadrants and a right upper quadrant soft tissue density present in 25-60% of patients.
  • These findings are followed by an obvious pattern of small bowel obstruction, with small bowel dilatation and air-fluid levels in the small bowel only. If the distention is generalized and the air-fluid levels are also present in the colon, the findings more likely represent acute gastroenteritis than intussusception.
  • A left lateral decubitus view is also helpful. If the view exhibits air in the cecum, the presence of ileocecal intussusception is highly unlikely.
• Ultrasonography is a noninvasive modality that can aid in making the diagnosis of intussusception.
• • Hallmarks of ultrasonography include depiction of the intussusceptum and its mesentery within the intussuscipiens (target and pseudokidney signs).
  • Ultrasonography is highly operator dependent; therefore, interpret results with caution.
• CT scan has also been proposed to be useful making the diagnosis of intussusception; however, CT findings are unreliable, and use of CT carries the risks associated with intravenous contrast administration, radiation exposure, and sedation.
• The traditional and most reliable way to make the diagnosis of intussusception in children is to obtain a contrast enema (either barium or air).
• • Contrast enema is quick and reliable and has the potential to be therapeutic.
  • Exercise caution when performing contrast enema in patients older than 3 years because most patients older than 3 years have a surgical lead point in the small bowel, and the diagnostic and therapeutic yield of the enema is lower in these patients.

Histologic Findings

If a segment of intestine is resected at the time of operative reduction, intestinal obstruction with edema, congestion, lymphocytic infiltration, and transmural infarction are typical findings.

TREATMENT

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Medical Care

Tailor treatment of the child with intussusception to the stage at presentation.

• For all children, start intravenous fluid resuscitation and nasogastric decompression as soon as possible.
• The presence of peritonitis and any evidence of perforation revealed on plain radiographs are the only 2 absolute contraindications to an attempt at nonoperative reduction with a therapeutic enema. Therapeutic enemas can be hydrostatic, with either barium or water-soluble contrast, or pneumatic, with air insufflation. Therapeutic enemas can be performed under fluoroscopic or ultrasonographic guidance. The technique chosen is not important as long as the radiologist performing the enema is comfortable with the method. Preferably, the pediatric surgeon involved is present at the reduction.
• Since the description by Hirschsprung of a systematic approach to hydrostatic reduction of intussusception, the reported success rate of this nonoperative intervention has widely varied (<40% to >90%). This variability in outcome attests to the various factors involved in successful hydrostatic reduction. Among these are factors that are individual to the patient (age, duration of symptoms, presence of lead points) and others that depend on the technique used. Paramount among the latter category is the availability of a team of pediatric surgeons and radiologists with the necessary expertise, determination, and dedication. Even among pediatric radiologists, consensus has been lacking on methodologic issues, including the choice of reducing agent, the type of catheter, the role of the external manipulation of the abdomen, the use of medications, and the establishment of guidelines for pressure limits and number of attempts.
• When performing a therapeutic enema, the recommended pressure of air insufflation should not exceed 120 cm of water. When using barium or water-soluble contrast, the column of contrast should not exceed 100 cm above the level of the buttocks. An attempt is not considered successful until the reducing agent, whether air, barium, or water-soluble contrast, is observed refluxing back into the terminal ileum. Otherwise, the intussusception can remain at the ileocecal region and can resume its progression.
• The value of repeated attempts at nonoperative reduction, if the first attempt is unsuccessful, has not been determined. Some clinicians recommend taking the patient to surgical care if the first attempt fails, and other clinicians advocate 1 or 2 subsequent attempts within a few minutes to a few hours after the first attempt. Delay between the reduction attempts may place the patient in the "window" of spontaneous resolution, which has been reported with an incidence of 5-6%. In addition, the first attempt can reduce the intussusception partially, making the intussusceptum less edematous with improved venous drainage.
• Some reports have postulated that reduced edema with better venous drainage is one reason the success rate of hydrostatic reduction improves with administration of a second enema. If repeated attempts are unsuccessful, any progress in pushing back the intussusceptum toward the ileocecal valve during operative reduction is advantageous. Delay in performing surgery because of additional attempts at nonoperative reduction has been demonstrated to have no adverse effects on the rates of success of operative reduction and morbidity in the patient.
• When therapeutic enema is successful, the results are immediate and extremely gratifying. The infant falls asleep almost immediately, and the obstruction is relieved, allowing the resumption of a normal diet. A short period of overnight observation usually is warranted before discharge. The recurrence rate of intussusception after nonoperative reduction is usually less than 10% but has been reported as high as 21%. Most intussusceptions recur within 72 hours of the initial event; however, recurrences have been reported up to 36 months later. More than one recurrence suggests the presence of a lead point. A recurrence usually is 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, contemplate surgical exploration.
• Therapeutic enema is of no value in patients with small bowel–to–small bowel intussusception, which usually occurs in older patients who have other associated diseases (eg, HSP, hemophilia, Peutz-Jeghers syndrome, malignancies).

Surgical Care

If nonoperative reduction is unsuccessful or if obvious perforation is present, promptly refer the infant for surgical care.

• Traditional entry into the abdomen is through a right paraumbilical incision. Deliver the intussusception into the wound and attempt nonoperative reduction. Milking the intussusceptum out of the intussuscipiens is important. Sustain gentle manual pressure rather than pulling out the intussusceptum to avoid risk of iatrogenic perforation. If operative reduction is successful, appendectomy is often performed if the blood supply of the appendix is compromised. A cecopexy is not necessary. Risk of recurrence of the intussusception after operative reduction is less than 5%.
• If manual reduction is not possible or perforation is present, perform a segmental resection with an end-to-end anastomosis. A diligent search for any lead points is warranted, especially if the patient is older than 2-3 years.
• Recently, laparoscopy has been added to the surgical armamentarium in the treatment of intussusception. Laparoscopy can be performed in all cases of intussusception. Reduction of the intussusception, confirmation of radiologic reduction, and detection of lead points have all been reported.

Consultations

Involve a pediatric surgeon as early as possible to help coordinate the care and resuscitation of the infant. The availability of a pediatric radiologist enhances the chances of successful nonoperative reduction.

Diet

A few hours after nonoperative reduction, start the infant on a regular age-appropriate diet as tolerated. If operative reduction was performed, advance the diet as with any postoperative patient.

Activity

The only limitations on activity after the treatment of intussusception are those imposed by the postoperative state.

MEDICATION

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Drug therapy is not currently a component of the standard of care for intussusception.

FOLLOW-UP

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Further Inpatient Care

• With toleration of diet, patients treated with nonoperative reduction usually are discharged 12-18 hours after the therapeutic enema. After operative reduction, postoperative progress dictates the length of stay.

Further Outpatient Care

• Patients treated with nonoperative reduction usually do not require any specific follow-up care unless problems exist.
• Postoperatively, patients require 1-2 visits to the pediatric surgeon to check on the progress of healing.

In/Out Patient Meds

• Medications are limited to those used for pain control after laparotomy. Patients with HSP or hemophilia and intussusception require standard therapy for the individual disease.

Transfer

• Transfer patients in whom intussusception is suspected to a facility at which surgeons and radiologists are present who are experienced in therapeutic techniques.

Deterrence/Prevention

• No measures are preventive for idiopathic intussusception.

Complications

• Complications rarely occur when the diagnosis is prompt, and they 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)

Prognosis

• Prognosis is usually excellent.

Patient Education

• Educate parents and caregivers of a patient treated with nonoperative reduction in regard to the risks and signs and symptoms of recurrence so that the initiation of care is not delayed.
• For excellent patient education resources, visit eMedicine's Esophagus, Stomach, and Intestine Center. Also, see eMedicine's patient education articles, Abdominal Pain in Children, Colic, Rectal Bleeding, and Barium Enema.

MISCELLANEOUS

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Special Concerns

• Intussusception can be associated with various medical conditions or situations, which are usually seen in patients older than 2-3 years. The intussusception is usually located from the small bowel to the small bowel; therefore, therapeutic enemas are less helpful and are usually unsuccessful.
• • 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 intussusception in the small bowel. Elucidating the cause of the pain is essential in any child in whom HSP is suspected.
    • Treating the pain of vasculitis with corticosteroids results in dramatic response.
    • Patients with intussusception require surgery, rather than an enema, since the intussusception is usually in the small bowel.
    • Obtain plain radiographs of the abdomen with the patient in the supine and upright positions to identify the small bowel obstruction present with intussusception. If radiograph findings are normal, assume the patient has vasculitis and treat with steroids.
  • Hemophilia and other coagulation disorders: Patients with hemophilia and other coagulation disorders can develop submucosal hematomas, which can cause pain and lead to intussusception. Differential diagnosis includes retroperitoneal hemorrhage in addition to other usual causes of abdominal pain. Radiographs of the abdomen should reveal a small bowel obstruction pattern if intussusception is present. In the absence of intussusception, treatment is supportive with correction of the coagulopathy.
  • Postoperative intussusception: Intussusception is a rare complication in the postoperative period. Complicating 0.08-0.5% of laparotomies, intussusception can occur regardless of the site of the operation. Intussusception is assumed to occur because of a difference in activity between segments of the intestine recovering from an ileus, which creates the intussusception. Intussusception is suggested in any postoperative patient who has a sudden onset of a small bowel obstruction after a period of recovery from an ileus, usually within the first 2 weeks after surgery. 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 reduce the intussusception and remove the tip of the catheter.
  • Cystic fibrosis: A rare complication of cystic fibrosis, intussusception occurs in approximately 1% of patients. 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. Most of these patients require operative reduction.
  • Neonatal intussusception: Intussusception in the first month of life is rare. Most of these patients are found to have a surgical lead point; therefore, enemas are rarely successful and are potentially dangerous.

MULTIMEDIA

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Media file 1:  Abdominal radiograph shows small bowel dilatation and paucity of gas in the right lower and upper quadrants.
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Media type:  Radiograph

Media file 2:  Air contrast enema shows intussusception in the cecum.
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Media type:  Radiograph

Media file 3:  Barium enema shows intussusception in the descending colon.
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Media type:  Radiograph

Media file 4:  CT scan reveals the classic ying-yang sign of an intussusceptum inside an intussuscipiens.
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Media type:  CT

Media file 5:  Abdominal ultrasonography reveals the classic target sign of an intussusceptum inside an intussuscipiens.
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Media type:  Ultrasound

REFERENCES

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Article Last Updated: Apr 25, 2008