Intussusception (General Pediatric Surgery of Abdomen)
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Intussusception is one of the most common causes of acute bowel obstruction in children and occurs in 1–4 in 2000 children worldwide. This condition was initially associated with high morbidity and mortality; however, with the advent of better imaging with ultrasound and knowledge in diagnosis, the clinical management has dramatically improved clinical outcomes. Due to the essential knowledge of allowing adequate aggressive fluid resuscitation at presentation, earlier diagnosis, and prompt attempt at reduction, this common condition is being treated more successfully. Non-operative interventions, including ultrasound-guided pneumatic reduction and hydrostatic reduction, have become the mainstay of treatment and have dramatically reduced the need for surgical intervention. In cases where surgery is indicated, laparoscopy provides a useful less invasive technique to treat as well as aid in diagnosis. This once fatal phenomenon is now becoming more easily and successfully managed in pediatric surgical centers around the world.
KeywordsIntussusception Ileocolic Ileoileal Lead point Ultrasound Pneumatic reduction Hydrostatic Peyer’s patches Laparoscopic reduction
Intussusception is the telescoping of one portion of bowel into another. The word intussusception is derived from the Latin words intus (within) and suscipere (to receive) (Hamby et al. 1996). It was first identified as a disease in the 1600s by Barbette in Amsterdam (Barbette 1674), but it was not until 1793 that Hunter described the condition of intussusception in detail (Hunter 1793). Management of intussusception was initially considered to be surgical, with Jonathan Hutchinson (1873) in 1873 describing the first successful operation for intussusception in a 2-year-old child. The possibility of conservative management was confirmed 3 years later by Harald Hirschsprung (1876), who employed hydrostatic reduction in the treatment for intussusception. Early historical records, however, have also indicated toward the use of pneumatic reduction via hand bellows through the anus, first tried at the time of Hippocrates (McAlister 1998). However, the first documented description of pneumatic reduction in the literature was only found in the 1800s (McDermott 1994). With the advent of fluoroscopic-based techniques in medicine, the use of barium enemas to reduce intussusceptions was also introduced, with Ravitch popularizing the use of this method in the United States (Ravitch 1959).
In 1864, the Scottish surgeon Greig (Hirschsprung 1876; McAlister 1998) was the first to lay down strict criteria for the clinical diagnosis of intussusception. He claimed that he successfully reduced four out of five actual pediatric intussusceptions with hand bellows: “Contrary to our expectations the air passed readily into the bowel and seemed to give the child great relief” (Greig 1864).
Intussusception occurs in 1–4 in 1000 live births in the United Kingdom (Sinha and Davenport 2010) with a worldwide incidence of 1–4 in 2000 among infants and children. It occurs mainly between the ages of 3 months and 3 years; however, idiopathic intussusception can occur at any age. In 75% of cases, intussusceptions occur within the first 2 years of life, whereas 90% occur within the first 3 years of age. Over 40% of cases of intussusception are encountered within 3–9 months of life (Ein and Stephens 1971). As well as occurring in term infants and toddlers, intussusception has been described in premature infants and in utero and may lead to small bowel atresia, namely, ileal atresia (Pueyo et al. 2009). The implication of intussusception resulting in small bowel atresia has also been reported in term newborns (Saxena and Van Tuil 2008). Perinatal intussusception (0.3%), on the other hand, is more likely caused by a pathological lead point such as that found in older patients (Avansino et al. 2003).
The male to female ratio is approximately 3:2 and there is no racial variation. The incidence of intussusception peaks during epidemics of respiratory infections and gastroenteritis. The rotavirus vaccine (RotaShield™) has been postulated to cause intussusception (Bines 2005). The risk was reported to be between 1 in 10, 000 and 1 in 32, 000, with the highest risk being within the first 3 to 14 days after the first dose of RotaShield™, especially in children older than 3 months of age.
Intussusception is one of the most frequent causes of acute bowel obstruction in toddlers and infants (Ashcraft et al. 2005), and clinical deterioration can be quick. Therefore, it highlights the importance of expedited recognition and hence treatment of this condition. A combined effort between the pediatrician, pediatric surgeon, and pediatric radiologist is of utmost importance in recognizing the condition and managing it under the hospital protocols.
Intussusception involves plugging of bowel segment into the adjacent segment. The invaginated proximal bowel (the inner and middle walls) is termed the “intussusceptum,” and the distal portion of the bowel (the outer walls) is termed the “intussuscipiens.” With regard to the localization of intussusception within the gastrointestinal tract, it has been observed that more than 80% of intussusceptions are of the “ileocolic” type, although other forms such as ileoileal types (~10%) or caecocolic-, colocolic- (~10%), jejunojejunal types can also occur. An intussusception can occur due to an identifiable lesion which acts like a “pathological lead point” and causes the proximal bowel to invaginate into the distal bowel during normal peristalsis.
The cause of intussusception may be primary or secondary. Primary or idiopathic etiology for intussusception is more common as it involves an array of factors that could result in inflammation of gut lymphatic tissues (such as inflamed Peyer’s patches) that could trigger the process of invagination. These include upper respiratory tract infections (e.g., adenovirus, rotavirus) and gastroenteritis infections which cause hypertrophy of Peyer’s patches in the intestine. These protrude into the lumen and act as the lead point causing the initial invagination. In fact, during surgery of intussusception in the selective cases requiring operative management, careful examination of the pathology demonstrates marked hypertrophy of the lymphoid tissue of the intestinal wall at the leading edge of the intussusceptum (Stringer et al. 1992).
Whereas the primary causes of intussusception are correlated to viral infections, secondary causes (~5%) of intussusception are deemed to be a result of specific anatomical lead points. The incidence of secondary intussusception is reported to range between 2% and 12% in the literature (Meier et al. 1996). For some unknown reason, the incidence of anatomical lead points has been found to increase with age (Blakelock and Beasley 1998).
Meckel’s diverticulum (most common)
Polyps (Peutz-Jeghers syndrome rarely, familial adenomatous polyposis)
Intramural hematoma (Henoch-Schonlein purpura, blunt abdominal trauma)
Ectopic pancreas or gastric mucosa
Irrespective of the primary or secondary “triggering factor,” as the proximal bowel invaginates into the distal bowel, the mesentery of the bowel is compressed, leading to venous obstruction and edema of the bowel wall. If the process is not reversed in a reasonable amount of time, arterial insufficiency and then bowel wall necrosis will ultimately ensure. Although spontaneous reduction can occur as observed in small bowel intussusceptions, the natural history of this condition is to progress; and if the condition is not recognized and treated early, the progression of necrosis can lead to sepsis and even fatality. The morbidity and mortality of this condition have dramatically decreased over the last few decades due to a better understanding of the condition, optimal investigation techniques for detection of the pathology, and the success of non-operative interventions (Kaiser et al. 2007).
This is seen in children of age 9–12 years with cystic fibrosis. It is due to inspissated secretions and thick fecal matter in the intestinal lumen that acts as a lead point to produce intussusception. These can occur repeatedly, and reductions may be required on multiple occasions (Stringer et al. 1992).
Children with intussusception present with intermittent colicky abdominal cramps associated with pulling up of the legs. These occur in children who may have previously been absolutely free of symptoms and comfortable, and commonly following an episode of viral infection. At the time of presentation, most of these children may look lethargic. The abdominal cramp may occur in a periodic fashion with episodes often stopping as quickly as they had started. Presentation may also be associated with breath holding, writhing around, and vomiting as well. The vomiting may initially be undigested food, which may become bilious after further bouts. In between attacks, the child may be well or lethargic. The constrained bowel may become ischemic, leading to mucus production from venous and lymphatic production to eventual sloughing off of the mucosa from ischemia causing the classic “red-currant jelly” stool in these children.
During episodes of cramping, the right lower quadrant may appear flat or empty, and the right upper quadrant may have a sausage-shaped mass palpable (Dance’s sign) (Jean Baptiste Dance 1832). This is due to the progression of the cecum and ileocecal portion of the intussusception into the right or transverse colon. The mass may be palpable anywhere in the abdomen. Occult blood per rectum, as aforementioned, is a late sign. There may be signs of dehydration or septicemia, causing tachycardia and fever. Sometimes the children may present with septic shock, so it is important to consider this as a differential diagnosis in children presenting to emergency rooms with shock.
Progression of the disease or a delay in diagnosis may lead to the intussuscepting mass to progress along the GI tract. It can cause prolapse of the intussusceptum through the anus. This indicates severe compromise of the blood supply and ischemia of the gut. It can be mistaken for a rectal prolapse; however, rectal prolapse is not associated with vomiting or sepsis. One can examine with a gloved finger which can pass between the prolapsing bowel and the anus, whereas this is not possible in a true rectal prolapse (Torres and McCafferty 2010).
Screening ultrasound in experienced hands is highly accurate and decreases the number of unnecessary contrast enema reduction and hence also decreases the exposure to ionizing radiation (Edwards et al. 2017). Ultrasound can differentiate between small bowel intussusception and ileocolic intussusception. It may show evidence of free intra-abdominal fluid, and if used with color Doppler, it can comment on bowel vascularity in terms of wall edema (from ischemic compromise) and peristalsis and hence indicate if surgery is more appropriate rather than image-guided reduction if there is compromised bowel involved (Saxena and Hollwarth 2007).
Computed tomography (CT) investigations can be done in older children where there are irregular features or the diagnosis is not clear. There may be associated pathology (such as lymphoma) which needs to be investigated. This decision to perform a CT should be in keeping with the clinical features and condition of the child. The CT may show an intraluminal mass with a characteristic layered appearance within the mass or even evidence of intramural hematomas.
This can be diagnostic as well as therapeutic and will be discussed in more detail in the management section.
Good intravenous access, adequate fluid resuscitation, the insertion of a nasogastric tube, and broad-spectrum antibiotics are the first-line management in all cases. In the absence of peritonitis or perforation, the gold-standard method of treatment is radiological-guided reduction either pneumatic or hydrostatic (Ko et al. 2007; Tareen et al. 2011). The older the child, the longer the history, and certain features such as the presence of blood per rectum make enema reduction less successful, and operative approach may be needed instead (Daneman and Navarro 2004).
Hydrostatic reduction of intussusception was first described in 1926 (Hipsley 1926). These reductions were performed under anesthesia and using saline solution. The fundamental implications of this technique however were incorporated into the modern methods of hydrostatic and pneumatic reduction.
In the present form of hydrostatic reduction, which was the main treatment up until the mid-1980s (Kaiser et al. 2007), a lubricated straight or Foley catheter is inserted into the rectum and held in place by firmly holding the buttocks together to create a tight seal. The catheter balloon is not normally inflated here. The contrast material is then run into the rectum from a height of 3 ft above the patient. Constant hydrostatic pressure is continued as long as reduction is occurring. Filling of the bowel is observed fluoroscopically. If there is no progress, the contrast is drained, and the procedure can be repeated up to two further times. Usually, reduction is seen up until the ileocecal valve, which is followed then by a delay at that point until a free flow of contrast is seen into the distal small bowel. A successful reduction depends on observation of the free flow of contrast into the distal small bowel. The advantages of this technique compared to surgical intervention are decreased morbidity, cost, and length of hospital stay. However, if this method is the preferred option of treatment, owing to the risk of bowel perforation, a water-soluble isotonic contrast is a better alternative to barium.
However, there is a risk of perforation leading to a pneumoperitoneum. The use of a needle puncture to decompress can help stabilize the patient when proceeding to theatre for laparotomy. Perforation occurs usually in ischemic bowel. The success rates are variable 50–90%, but most units should aim to achieve a success rate of 65–70% (Guo et al. 1986; Ondhia et al. 2019); this is documented by seeing free flow of air in the distal small bowel. However, there can be false-positive results if there is poor visualization of the lead points or of the reduction itself (Maoate and Beasley 1998).
The process can be repeated again after 3 h if reduction is incomplete, but only if the child is stable. If there is no progress, then the child should proceed to theatre for surgical reduction. Non-operative reduction is not useful in ileoileal intussusceptions and is relatively contraindicated in older atypical presentations with a secondary pathology, in which surgical intervention is required. It is worth considering that the incidence of a lead point increases with age (Bines 2005).
After successful reduction, the child should be observed for 24 h and initially kept nil by mouth and on IV fluids. There is a possibility of recurrence of 5–10% in the first 72 h, and hence the family should be informed about this (Niramis et al. 2010).
A surgical approach is indicated in children who have had a perforation or incomplete hydrostatic or pneumatic reduction, or in children with signs of shock and peritonitis at initial examination. It is also considered in children who present atypically or with features of secondary causes. Again the child should have fluid resuscitation, broad-spectrum antibiotics, and nasogastric decompression prior to theatre.
Laparoscopy is a growing alternative in many centers and can also be quite advantageous as a diagnostic tool if there is doubt over the adequacy of reduction after enema. The bowel handling may not be easy, and “pulling” rather than “pushing” of the bowel has been advocated here.
Recurrence has been described from 2% to 20% with about 1/3 occurring in the first 24 h and the majority within the first 6 months (Daneman et al. 1998). Guo et al. studied risk factors for recurrent intussusception in 191 children and found that in children older than 1 year of age, symptom duration (≤12 h), the absence of vomiting, location of mass in the right abdomen, and pathological lead points were significantly predictive of recurrent infection (Guo et al. 2017). Patients tend to have fewer symptoms with recurrent intussusception and may only present with discomfort and irritability, so it should be highly suspected in these patients. Recurrences are usually less likely to occur after surgical reduction or resection.
Small Bowel Intussusception
Ileocolic intussusceptions remain the most common form of intussusception, but small bowel intussusceptions have been reported to range from 1.68% to 17% (Kornecki et al. 2000) with predominance for ileoileal intussusception (80%) followed by the jejunojejunal type (20%) (Saxena et al. 2007). As aforementioned, the symptoms of intussusception are similar regardless of the site of pathology and may cause diagnostic confusion. Symptoms of small bowel intussusception may mimic that of acute gastroenteritis. Abdominal ultrasound is extremely useful in differentiating between ileocolic and ileoileal intussusception. It can also comment on the proximity of the intussusception, in relation to the ileocecal valve, as well as vascularity of the bowel and any lead points. This therefore determines the ongoing management and clinical course of the condition (Guo et al. 2017). Most small bowel intussusceptions can reduce spontaneously and do not need any active intervention or management (Kornecki et al. 2000). It has been postulated to be due to the increased motility of the small bowel, thereby causing spontaneous reduction (Saxena and Hollwarth 2007). Successful conservative treatment has been associated with early presentation and diagnosis. Pneumatic reduction was largely regarded as unsuccessful in small bowel intussusception due to the impedance caused by the ileocecal valve as the limiting factor (Koh et al. 2006); however, it has been reported that pneumatic reduction can be successful in small bowel intussusception if the intussusception is ileoileal and close to the ileocecal valve (Saxena et al. 2007). In patients where the small bowel intussusception has not reduced spontaneously, ultrasound with color Doppler is useful to localize the site and evaluate the bowel. If the site of small bowel intussusception is close to the ileocecal area and there is good bowel viability, then a pneumatic reduction can be successful. However, if the site of small bowel intussusception is high, or there is compromised bowel, then a surgical approach is recommended.
One of the most important criteria for the management of intussusception is the timing of presentation. This depends on availability and access to quality healthcare, increased awareness, and knowledge in referring hospitals of the salient features and initial management of intussusception. Patients with ileocolic intussusception may present earlier as their symptoms may be more pronounced or they present more unwell. Symptoms of small bowel intussusception may mimic acute gastroenteritis and hence delay diagnosis. In fact, delayed presentation is directly associated with an increase in surgical intervention and bowel resections (Shapkina et al. 2006). Ileocolic intussusception is less likely to reduce spontaneously as the pathology most commonly starts at the ileocecal region, with edema at the ileocecal valve being the limiting factor. Successful pneumatic reduction in ileocolic intussusception has been reported to be as high as 91% in some centers (Saxena and Hollwarth 2007), provided the presentation is not delayed and the ultrasound confirms viable bowel. Most studies in literature include both small bowel and ileocolic intussusception in their data and do not differentiate features or outcomes between them. A study by Saxena et al. in 2007 (Saxena and Hollwarth 2007) compared the difference between patients with small bowel and ileocolic intussusception. Spontaneous reduction occurred in 13.3% of ileocolic and 64.3% of small bowel intussusception. Pneumatic reduction was successful in 91% of ileocolic compared with 85.7% of small bowel intussusception. Primary surgical intervention was required in 6% of patients with ileocolic intussusception in whom 2.4% required bowel resection and in 10.7% of patients with small bowel intussusception, all of whom necessitated bowel resection.
Conclusion and Future Directions
In conclusion, the efficacy of intussusception treatment in terms of closed reduction, surgery, and outcomes depends on a combination of factors: the timing of presentation, the type of intussusception (ileocolic versus small bowel), the presence of pathological lead points, ultrasound/Doppler findings, and expertise in reduction techniques to avoid complications.
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