Updated: Sep 28, 2009
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.
Intussusception was first described by Barbette in 1674, and Wilson was the first to successfully treat it surgically in 1831. In 1876, Hirschsprung first reported the technique of hydrostatic reduction,23 and, after monitoring a series of 107 cases, reported a 35% mortality rate attributable to intussusception in 1905.
Intussusception is the telescoping or invagination of a proximal portion of intestine into a more distal portion (see Images 1-2). Vascular compromise and subsequent bowel necrosis are the primary concerns with intussusception. Among patients who undergo operative reduction of intussusception, as many as 10% may require bowel resection.
The incidence of intussusception is 1.5-4 cases per 1000 live births, with a male-to-female ratio of 3:2. The greatest incidence of idiopathic intussusception is in infants aged 9-24 months. A seasonal incidence has been described, with peaks in the spring, summer, and the middle of winter. These periods correspond to peaks in the occurrence of seasonal gastroenteritis and upper respiratory tract infections.
Intussusception is ileocolic (see Image 1) in 80% of cases but may also be ileoileal (see Image 3), 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.52
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 Image 4) is the most common lead point, followed by polyps, such as with Peutz-Jeghers syndrome, and intestinal duplications.16
Other lead points described include lymphomas, lymphangiectasias,27 submucosal hemorrhage with Henoch-Schönlein purpura, trichobezoars with Rapunzel syndrome,40 caseating granulomas due to abdominal tuberculosis,34 hemangiomas, and lymphosarcomas.
Children with cystic fibrosis (CF) may present with intussusception due to inspissated meconium in the terminal ileum. While 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. When a lead point is present with postoperative intussusception, several cases have been reported after appendectomy with stump inversion.2 Other rare reported types of intussusception have included retrograde jejunojejunal intussusception following duodenal atresia repair25 and an ileoileal type resulting from blunt abdominal trauma.18
Although the vast majority of intussusception cases are idiopathic, Oshio et al (2007) in Japan reported a familial anatomical tendency that may predispose to the condition in the face of viral infection. 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.36
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 results in fluid sequestration and bleeding from the GI tract. If untreated, the bowel may perforate, resulting in sepsis.
In 1941, Ladd and Gross described the deceivingly healthy appearance of infants with intussusception.31 One rarely finds intussusception in a child who is thin, undernourished, and poorly developed. This is evident in that babies with intussusception are usually well nourished and generally above-average in physical development. This fat and healthy appearance is apt to mislead the physician in the early hours of the patient’s illness. Thus, the first visit may leave the physician with the impression that the parent is overanxious, whereas a return visit the next day shows that the child is desperately ill.
Disorders characterized by bowel obstruction, colicky abdominal pain, blood in the stool, an intra-abdominal mass, or a combination of these should be considered in the differential diagnoses of intussusception. These include gastroenteritis, appendicitis, Meckel diverticulum, malrotation with midgut volvulus, or incarcerated hernia.
Most infants with intussusception have a history of intermittent severe cramping or colicky abdominal pain, occurring every 5-30 minutes. During these attacks, the infant screams and flexes at the waist, draws the legs up to the abdomen, and may appear pale. These episodes may last for only a few seconds and are separated by periods of calm normal appearance and activity. However, some infants become quite lethargic and somnolent between attacks. Early on, the infant may vomit undigested food. As attacks continue, emesis may turn bilious. Stool that appear normal in character early in the course of the illness eventually become dark red and mucoid (resembling currant jelly), a sign of intestinal ischemia and mucosal sloughing.
Ideally, the dramatic paroxysms described above would sufficiently alert the parents to seek medical attention early in the course of the illness. If this is the case, initial inspection may reveal a robust infant who appears healthy. Between attacks, the infant may appear somnolent or quite normal, and findings on examination of the abdomen may be unremarkable. During an attack or spasm, the infant suddenly appears startled or anxious and begins to scream. Upon initial inspection, the abdomen may appear scaphoid; during paroxysms, it may be rigid; and later in the course of the illness, it may become distended with signs of peritonitis. Careful palpation after an attack has subsided may reveal an ill-defined or sausage-shaped mass.
With early ileocolic intussusception, the mass is typically found in the right upper quadrant or abdomen. The right lower quadrant may seem empty upon examination, a finding known as the Dance sign. This mass may be difficult to locate in inconsolable infants because of abdominal rigidity from muscle straining. If episodes of cramping are witnessed, the careful examiner may auscultate peristaltic rushes in the area of the intussusception.
The rectal examination should commence with inspection of fecal material in the diaper. Normal-appearing stool should be tested for occult blood. The presence of mucoid or frankly bloody stool supports the diagnosis. Rarely, inspection of the anus reveals the prolapsed tip of the intussusception. A digital rectal examination should be performed routinely, looking for blood or a mass higher in the anal canal.
In lieu of concerns about rotavirus vaccine–associated intussusception, The Brighton Collaboration Intussusception Working Group established a clinical diagnosis using a mix of major and minor criteria. This stratification helps to devise a working model based on 3 levels of evidence to suggest definite, probable, and possible cases of intussusception.51
Major criteria
Minor criteria
Minor criteria include any of the following:
Stratification based on evidence levels
The relevancy of this definition has also been clinically tested in Switzerland, where it correctly identified 86 of 96 confirmed episodes, 82 of which met level 1 evidence. Regarding sensitivity in comparison to this level 1 evidence, the level 2 (probable) had 65%, while level 3 (possible) had only 30%.50
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.
Immediate surgery is indicated in unstable patients, in patients who have peritonitis, and in patients with bowel perforation during attempted enema reduction. Elevated temperature and WBC counts have also served as relative indicators for surgery. Patients requiring surgery must be aggressively resuscitated with fluids, and care must be taken to preserve body temperature preoperatively, intraoperatively, and postoperatively.
Intussusception is the telescoping or invagination of a proximal portion of intestine into a more distal portion. Intussusception may be ileoileal, colocolic, ileoileocolic, or ileocolic, which is most common (see Image 1). The primary concern with intussusception is vascular compromise and subsequent bowel necrosis. In addition to bowel obstruction, edema with venous obstruction and eventual obstruction of arterial flow leads to ischemia and eventual full-thickness necrosis of the intussuscepted bowel and mesentery.
Contraindications to enema reduction include evidence of bowel perforation and peritonitis.
Resected specimens show varying degrees of ischemia, necrosis, or both. Benign reactive lymph node hyperplasia and Peyer patch hyperplasia is common. Resected specimens should be carefully examined for potential lead points (eg, Meckel diverticulum, polyps, lymphoma).
Expeditious diagnosis and management is essential to successful outcomes in infants with intussusception. Once the diagnosis of intussusception is entertained, surgical personnel should be notified, an intravenous line inserted, and intravenous hydration started. A nasogastric tube should be inserted and placed to suction. If the patient is markedly distended or has a dilated loop of bowel, an abdominal radiograph should be obtained. Antibiotics should be administered based on clinical suspicion of peritonitis or infection (sepsis) or in patients with a markedly elevated WBC count.
If the intussusception can be reduced with an enema, the hospital stay typically lasts 3 days in the United States. However, one small retrospective study in Canada evaluated 96 patients with a successfully reduced ileocolic intussusception and determined that a short observational stay in the emergency department could be used safely in 90% of patients. This approach would prevent an admission and save on costs; however, more evidence-based criteria need to be developed before it could become common practice.
Preoperatively, intravenous crystalloid resuscitation is begun (10 mL/kg x 2, plus 1.5 x maintenance fluid). A Foley catheter is placed to guide fluid resuscitation. A nasogastric tube is placed. Broad-spectrum intravenous antibiotics are administered. Body temperature must be preserved in the operating room. A type and screen of the patient's blood should be obtained. As with any patient with a bowel obstruction, careful induction (ie, rapid sequence) of anesthesia should take place because of the risk of regurgitation and aspiration.
The abdomen and bowel are typically explored through a right lower quadrant transverse incision, although some advocate a right transverse supraumbilical or even an upper midline incision. After inspection for signs of perforation, the intussusception is identified and delivered into the wound. First, an attempt is made at manual reduction by retrograde milking of the intussusceptum. Although gentle pulling may aid in reduction, avoid vigorous pulling apart of the intussuscepted segment of bowel.
If manual reduction is unsuccessful, if a mass or pathologic lead point is present, or if perforation has occurred, segmental bowel resection is necessary. After resection, a primary anastomosis may be performed. Often, after successful manual reduction, the involved segment of bowel appears edematous, hyperemic, or ischemic. These findings do not necessarily mandate resection. An incidental appendectomy is often performed, particularly if a right lower quadrant incision was made for access to the abdomen, as it may be presumed that the patient has had an appendectomy.
Laparoscopy in the management of intussusception was initially limited to a diagnostic role. It was used to confirm unreduced bowel following an enema with prompt conversion to an open procedure. The laparoscope allowed the surgeons to avoid unnecessary open procedures in cases of spontaneous reduction following enema and enhanced the efficacy of hydrostatic or pneumatic reductions, reducing the need for an open procedure in approximately 30% of cases. Continued experience with laparoscopy and improved technology has led some centers to successfully utilize the technique for therapeutic reduction in confirmed cases of pediatric intussusception.
Several small series have been published demonstrating the laparoscopic approach as safe, effective, and cost efficient when compared to the open technique. In a retrospective analysis at the University of Michigan comparing laparoscopic treatment of intussusception to the open technique, intraoperative complications occurred only in the cases where bowel necrosis was present and resulted in a 12.5% (2/18) conversion to open rate. Postoperative complications between the open and laparoscopic groups were not significantly different in this study. The open group experienced one wound infection and one recurrence (2/25), and the laparoscopic group experienced one urinary tract infection and one recurrence (2/18).28
A major concern regarding the laparoscopic approach is the inability to reduce the intussusception using the standard retrograde fashion of the open technique. Some authors voice concern that a surgeon reducing an intussusception laparoscopically must apply pull and tension on an often friable segment of bowel, increasing the risk of bowel perforation.
Additionally, because most cases of intussusception in older children and adults are secondary to a pathologic lead point, the laparoscopic technique may not be appropriate in these patients. In order to minimize the rate of conversion to the open approach, the patient should be seen within 36 hours of the onset of symptoms and have no peritoneal signs.
The compromised tactile feedback for identifying a lead point laparoscopically and the potential need for an extended bowel resection for malignancy support an open approach in older children and adults. However, advances in laparoscopy have made intussusception reduction more safe and effective. Chui et al have developed a “Chinese fan spread” technique that utilizes an intracorporeal fulcrum to distribute the distraction forces more evenly as the surgeon does a push-pull on the intussusception. Overall, 12 of 14 intussusceptions were reduced successfully without a concurrent enema, and the 2 cases in which laparoscopic reduction failed also failed open manual reduction, thus requiring right hemicolectomy. Of the 12 reductions, 5 had lead points that were managed laparoscopically or through a transumbilical incision.
The role of laparoscopy in intussusception is evolving and will be better defined as technology progresses and experience with the minimally invasive approach to this disease grows.
Intravenous fluid resuscitation is continued and calculated, taking into consideration maintenance requirements and third-space losses. Upon resolution of ileus, diet is advanced at the discretion of the surgeon.
In older children and in cases of recurrent intussusception (after 3-4 episodes) successfully reduced with an enema, consider evaluating the patient for a lead point (eg, upper GI series, Meckel scan).
Intussusception results in bowel obstruction; thus, complications such as dehydration and aspiration from emesis can occur. Ischemia and bowel necrosis can cause bowel perforation and sepsis. Necrosis of a significant length of intestine can lead to complications associated with short bowel syndrome. Whether treated by operative or radiographic reduction, late stricture (4-8 wk) may occur within the length of intestine involved.
The overall mortality rate of intussusception is less than 1%. Recurrence rates following nonoperative reduction and surgical reduction are approximately 5% and 1%-4%, respectively.
The following criteria are associated with a higher failure rate of nonoperative reduction:
Factors significantly predictive of bowel perforation are younger age and a longer duration of symptoms. The risk of postoperative adhesive small-bowel obstruction following nonoperative reduction is 0%; for operative reduction, it has been reported in as many as 5% of patients.
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intussusception, intussusceptum, intussuscipiens, bowel obstruction, ileoileal intussusception, colocolic intussusception, ileoileocolic intussusception, ileocolic intussusception, telescoping of intestine
Michael S Irish, MD, Adjunct Clinical Assistant Professor, Department of Surgery, University of Iowa; Consulting Pediatric Surgeon, Department of Pediatric Surgery, Blank Children's Hospital and Children's Hospital Physicians Group
Michael S Irish, MD is a member of the following medical societies: International Pediatric Endosurgery Group and Sigma Xi
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Jason K Shellnut, MD, Staff Physician, Department of Surgery, Iowa Methodist Hospital
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Philip M Bovet, DO, MPH, Resident Physician in Family Medicine, University of Wisconsin Health Clinic
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Rebeccah Brown, MD, Associate Director of Trauma Services, Associate Professor, Department of Clinical Surgery and Pediatrics, Cincinnati Children's Hospital Medical Center and University of Cincinnati Hospital
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H Biemann Othersen Jr, MD, Professor of Surgery and Pediatrics, Emeritus Head, Division of Pediatric Surgery, Medical University of South Carolina
H Biemann Othersen Jr, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Pediatrics, American Association for the Surgery of Trauma, American Burn Association, American Cancer Society, American College of Surgeons, American Medical Association, American Pediatric Surgical Association, American Society for Parenteral and Enteral Nutrition, American Surgical Association, American Thoracic Society, British Association of Paediatric Surgeons, Society for Surgery of the Alimentary Tract, Society of Critical Care Medicine, South Carolina Medical Association, Southeastern Surgical Congress, Southern Medical Association, Southern Society for Pediatric Research, and Southern Thoracic Surgical Association
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Harsh Grewal, MD, FACS, FAAP, Professor of Surgery and Pediatrics, Temple University School of Medicine; Chief, Section of Pediatric Surgery, Temple University School of Medicine
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