Pediatric Intussusception Surgery

Updated: Jun 29, 2023
  • Author: Amulya K Saxena, MD, PhD, DSc, FRCS(Glasg); Chief Editor: Harsh Grewal, MD, FACS, FAAP  more...
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Overview

Practice Essentials

Intussusception, which is defined as the telescoping or invagination of a proximal portion of intestine (intussusceptum) into a more distal portion (intussuscipiens), is one of the most common causes of bowel obstruction in infants and toddlers. [1]

Intussusception was first described by Barbette in 1674, and it was first successfully treated surgically by Wilson in 1831. In 1876, Hirschsprung first reported the technique of hydrostatic reduction, [2]  and in 1905, after monitoring a series of 107 cases, he reported a 35% mortality attributable to intussusception.

Vascular compromise and subsequent bowel necrosis are the primary concerns with intussusception. Among patients who undergo operative reduction of intussusception, those with long-standing intussusceptions, mostly due to late presentation, bear the risks of resection of affected bowel.

Stable patients in whom the index of suspicion for intussusception is high but who do not have evidence of ischemic bowel, perforation, or sepsis may undergo immediate contrast enema for diagnosis and treatment of suspected intussusception; bowel perforation and peritonitis are contraindications for enema reduction. Hydrostatic reduction of intussusception has proved effective in Europe and Asia and has been increasingly used at some centers in the United States.

Immediate surgery is indicated for unstable patients, patients who have peritonitis, and patients with bowel perforation during attempted enema reduction; elevated temperature and white blood cell (WBC) counts have been relative indications for surgery.

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Anatomy

Intussusception is the telescoping or invagination of a proximal portion of intestine into a more distal portion (see the first image below). Intussusception may be ileoileal (see the second image below), colocolic, ileoileocolic, or ileocolic (the most common type; see the third image below).

Diagram illustrating anatomy of intussusception. Diagram illustrating anatomy of intussusception.
Intraoperative appearance of ileoileal intussuscep Intraoperative appearance of ileoileal intussusception.
Intraoperative appearance of ileocolic intussuscep Intraoperative appearance of ileocolic intussusception.
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Pathophysiology

Intussusception results in bowel obstruction, followed by congestion and edema with venous and lymphatic obstruction. This progresses to arterial obstruction and subsequent necrosis of the bowel. Ischemia and then necrosis result in fluid sequestration and bleeding from the gastrointestinal (GI) tract. If untreated, the bowel may perforate, resulting in sepsis.

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Etiology

Intussusception is ileocolic in 80% of cases but may also be ileoileal, colocolic, or ileoileocolic. Most infants and toddlers (95%) with the condition do not have an identifiable specific lead point. In these idiopathic cases, careful examination may reveal hypertrophied mural lymphoid tissues (Peyer patches), which are due to adenovirus or rotavirus infection. Intussusception has also been found to increase the risk of tonsillar disease (ie, chronic or acute tonsillitis) and tonsillectomy in children. [3]

A specific lead point that draws the proximal intestine and its mesentery inward and propagates it distally through peristalsis is identified in only 5% of cases and is most commonly found in cases of ileoileal intussusception. Specific lead points are more commonly found in children older than 3 years and almost always in adults with intussusception. Meckel diverticulum (see the image below) is the most common lead point, followed by polyps, such as are seen with Peutz-Jeghers syndrome, and intestinal duplications. [4]

Intraoperative appearance of ileocolic intussuscep Intraoperative appearance of ileocolic intussusception due to Meckel diverticulum.

Other lead points described include lymphomas, lymphangiectasias, [5] submucosal hemorrhage with Henoch-Schönlein purpura, trichobezoars with Rapunzel syndrome, [6] caseating granulomas due to abdominal tuberculosis, [7] hemangiomas, and lymphosarcomas. Intussusception associated with pathologic lead points may be more likely to recur. [8]

Children with cystic fibrosis (CF) may present with intussusception due to inspissated meconium in the terminal ileum. Although intussusception is generally observed as a complication in older children with CF, neonatal intussusception with meconium plug syndrome associated with CF has been reported.

Postoperative jejunoileal or ileoileal intussusception, which has no specific lead point in most cases, accounts for approximately 1% of intussusceptions in children of all ages. [9] When a lead point is present with postoperative intussusception, several cases have been reported after appendectomy with stump inversion. [10] Other rare reported types of intussusception include retrograde jejunojejunal intussusception following duodenal atresia repair [11] and an ileoileal type resulting from blunt abdominal trauma. [12]

Although the vast majority of intussusception cases are idiopathic, Oshio et al in Japan reported a familial anatomic tendency that may predispose to the condition in the face of viral infection. [13] Of 554 families who had at furthest a third-degree relative with an idiopathic case of intussusception, the authors found an incidence of approximately 7%, or 1 per 14.2 cases. The family history may help in the workup, and further genetic testing may eventually identify the gene responsible for this predisposition.

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Epidemiology

The incidence of intussusception ranges from 26 to 38 cases per 100,000 live births in the first 3 years of life. [14] Intussusception typically presents between the ages of 6 and 36 months. Approximately 60% of children with intussusception are younger than 1 year, and 80-90% are younger than 2 years. [15]  

Although intussusception is most common in infants and toddlers, it is a diagnosis that should be considered in children outside this age range as well. Approximately 10% of cases occur in children older than 5 years, 3-4% in children older than 10 years, and 1% in infants younger than 3 months. [14]

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Prognosis

The following criteria are associated with a higher failure rate for nonoperative reduction:

  • Ileoileocolic intussusception
  • Long duration of symptoms (>24 hr)
  • Raised neutrophil percentage
  • Rectal bleeding
  • Failed reduction with barium enema at another institution
  • Age older than 2 years or younger than 3 months
  • Small-bowel obstruction on radiography
  • Dehydration of greater than 5%
  • Inexperienced radiologist

Factors significantly predictive of bowel perforation are younger age and a longer duration of symptoms.

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