Intussusception, Pediatric

The invagination or telescoping of a proximal portion of bowel (the intussusceptum) into a distal segment of bowel (the intussuscipiens)

Can be unremitting (80%) or transient (20%)

85% are ileocolic; ileoileal and colocolic types also occur.

Telescoping of the bowel occurs over a "lead point"¯-a lesion or defect in the bowel wall.

Telescoping of the bowel causes diminished venous blood flow and bowel wall edema, which can result in ischemia and obstruction.

Over time, arterial blood flow is inhibited and infarction of the bowel wall occurs, which results in hemorrhage.

If untreated, possible perforation and death

Bowel necrosis can occur within 48-72 hours after onset.

Clinical presentation can vary but usually includes the following:

"Paroxysms of pain"¯: episodes of calmness interspersed with fussiness

Bilious vomiting

"Currant jelly stools"¯ which represent mucosal sloughing

Epidemiology

1-4/1,000 live births

Male-to-female ratio: 2:1

Generally occurs in patients 3 months to 3 years of age

Peak age: from 5 to 10 months

More common in winter months

The most common abdominal emergency of early childhood and second most common cause of abdominal pain next to constipation

Increased incidence in children who received the RotaShield rotavirus vaccine (no longer available). Currently available vaccines (RotaTeq or Rotarix) have not been shown to increase the risk.

Etiology

Children <3 years: usually idiopathic (95%) or due to an enlarged Peyer patch (from viral infection)

Children ≥3 years: higher incidence of a pathologic lead point (4%): Meckel diverticulum, polyps, and lymphomas are most common. Other common etiologies include Henoch-Sch ¶nlein purpura (HSP), Peutz-Jeghers syndrome, intestinal duplications, inflammatory bowel disease, and other tumors.

Postoperative(1%): can occur in children who have had large retroperitoneal tumors removed

Diagnosis

History

Common to have recent history (prodrome) of a viral upper respiratory illness or gastroenteritis

Classical presentation involves the sudden onset of severe intermittent (colicky) abdominal pain characterized by drawing the legs up to the abdomen and crying.

Subsequently, the child may appear asymptomatic between paroxysms of pain that recur in <24 hours.

Classical presentation only occurs in 25-30% of patients.

Young infants may appear listless or lethargic during episodes.

Nonbilious emesis initially, becomes bilious with progressive obstruction

Currant jelly stools (sloughed mucosa, blood, and mucous) appear in 50% of cases and should be considered a late finding suggestive of a longer course of the disease prior to presentation.

Physical Exam

Lethargic with intermittent colicky abdominal pain

Palpable abdominal mass, usually in right upper quadrant, tubular or "sausage"¯ shaped

Dance sign: absence of bowel contents in right lower quadrant

Abdominal distention

Rectal exam: blood-tinged mucous or currant jelly stool

Symptoms and signs of peritonitis

Differential Diagnosis

Infectious

Gastroenteritis

Enterocolitis

Parasites

Immunologic

HSP

Gastrointestinal

GER or GERD

Food allergy

Inflammatory bowel disease

Celiac disease

Miscellaneous

Appendicitis

Peutz-Jeghers syndrome

Obstruction

Adhesions

Hernias

Volvulus

Stricture

Foreign body

Polyp

Tumor

Diagnostic Tests & Interpretation

Lab CBC, electrolytes Imaging

Abdominal x-ray

May show paucity of gas in right lower quadrant, air-fluid levels

"Meniscus sign"¯ is indicative of a mass in the colon (25-45%).

Abdominal ultrasound

Primary diagnostic modality

Highly sensitive and specific with experienced radiologist

"Target sign"¯ with presence of several concentric rings of bowel

Can sometimes indicate the pathologic lead point

Contrast enema

Diagnostic and therapeutic with reduction often achieved (95%)

Air enema (good chance of success, low risk of perforation,) preferred over barium enema (more effective but higher risk of perforation)

Alert

Only 25-30% of patients present with the classical triad of abdominal pain, vomiting, and currant jelly stool, so high clinical suspicion is necessary.

Patients should always have an IV placed and be undergoing fluid resuscitation prior to imaging or procedures.

Treatment

General Measures

Prompt recognition and reduction is imperative.

Spontaneous reduction occurs in 5-20%.

IV insertion, fluid therapy, and surgical consultation should be obtained once diagnosis is entertained.

Broad-spectrum antibiotics if perforation, peritonitis, or bacterial translocation due to a compromised gastrointestinal mucosa is suspected

Radiologic, pneumatic reduction with air contrast enema is mainstay of therapy.

Absolute contraindications to reduction by enema:

Peritonitis

Shock

Perforation

Multiple attempts may be made at reduction if initially unsuccessful or patient transferred to tertiary care facility after failed attempt.

Perforation during reduction occurs in <1% of cases, mostly in the transverse colon, and requires immediate operative intervention.

Surgery/Other Procedures

If patient presents with generalized peritonitis and/or signs of perforation, they should immediately go to the OR.

Unsuccessful attempts at pneumatic reduction

Open surgery involves milking the intussusceptum out of the intussuscipiens and rarely involves resection.

Laparoscopic surgery has gained more traction with better outcomes, cosmesis, and shorter length of stay.

Ongoing Care

Follow-up

Some centers may discharge home immediately or observe after nonoperative reduction.

Diets can be advanced quickly.

Education

Parents should be counseled that a 10% recurrence risk exists after nonsurgical reduction in the first 24 hours.

Recurrences may require repeat pneumatic reduction and/or ultimate operative intervention.

Prognosis

Timely diagnosis results in a highly favorable prognosis with no effects on the bowel.

Recurrences or failed reduction in children >3 years old may require workup for pathologic lead point.

Complications

Bowel necrosis secondary to local ischemia

Gastrointestinal bleeding

Bowel perforation

Sepsis, shock, death

Additional Reading

Daneman A, Navarro O. Intussusception. Part 1: a review of diagnostic approaches. Pediatr Radiol. 2003;33(2):79-85. [View Abstract]

Daneman A, Navarro O. Intussusception. Part 2: an update on the evolution of management. Pediatr Radiol. 2004;34(2):97-108. [View Abstract]

McCollough M, Sharieff GQ. Abdominal surgical emergencies in infants and young children. Emerg Med Clin North Am. 2003;21(4):909-935. [View Abstract]

Pepper VK, Stanfill AB, Pearl RH. Diagnosis and management of pediatric appendicitis, intussusception, and Meckel diverticulum. Surg Clin North Am. 2012;92(3):505-539. [View Abstract]

Vesikari T, Matson DO, Dennehy P, et al. Safety and efficacy of a pentavalent human-bovine (WC3) reassortant rotavirus vaccine. N Engl J Med. 2006;354(1):23-33. [View Abstract]

Codes

ICD09

560.0 Intussusception

ICD10

K56.1 Intussusception

SNOMED

49723003 Intussusception of intestine (disorder)

17186003 Ileocolic intussusception

FAQ

Q: Can my child have a recurrent intussusception?

A: Yes, if your child has had a nonsurgical reduction via air (or barium) enema. However, the risk is considered low (<10%). If the lead point has been removed surgically, recurrence is very unlikely. The greatest risk for recurrence is in the first 24 hours after reduction.

Q: What are common ages for presentation with intussusception?

A: 3 months to 3 years is the age range associated with the greatest risk of intussusception, but it may occur at any age. The prevalence of intussusception being a sign of an underlying pathologic condition rises with age.

Q: Could an infant have intussusception and not be crying in pain?

A: Yes. Infants often present without the classical manifestations of intussusception. It is critical to have a high index of suspicion in any infant presenting with acute onset of emesis, especially bilious emesis, and lethargy.