Intussusception in children

Seiji Kitagawa, MD; Mohamad Miqdady, MD Up to date on line 16.1 This topic last updated: Fevereiro 4, 2008 Modificado: Jefferson P Piva

INTRODUCTION Intussusception, the invagination into of a part is in the of the most intestine childhood, second intestinal in adults, itself,

Intussusception slight male

appears

to

have with

a a

predominance,

male:female ratio of approximately 3:2. PATHOGENESIS Intussusception occurs most often near the ileocecal junction (ileo-colic), although ileo-ileocolic, jejuno-jejunal, jejuno-ileal, also or colo-colic intussusception have

common abdominal emergency in early particularly common children cause of younger than 2 years of age, and the most obstruction and the after pyloric is

stenosis [1]. Intussusception is unusual diagnosis commonly overlooked. In the majority of cases in adults, a pathologic cause is identified [2]. In contrast, the majority of cases in children are idiopathic. EPIDEMIOLOGY Intussusception is

been described. The proximal segment of bowel telescopes into the distal segment, dragging the associated mesentery with it. This leads to the development of venous and lymphatic congestion with resulting intestinal edema, which can ultimately lead to ischemia, perforation and peritonitis. A lead point can be identified in up to 25% are of children to with be ileo-colic idiopathic, body of intussusception. The remaining 75% considered an although increasing

the most common cause of intestinal obstruction in infants between 6 and 36 months of age. Approximately 60 percent of children are younger than 1 year old, and 80 percent are younger than 2. Intussusception is rare before 3 months and after 6 years of age. In a population-wide survey in Switzerland, the yearly mean incidence of intussusception was 38, 31, and 26 cases per 100,000 live births in the first, second, and third year of life, respectively, then fell to less than half that rate in older age groups [3]. Most episodes occur in otherwise healthy and well-nourished children.

evidence suggests that viral triggers may play a role in some cases: The incidence of intussusception has a seasonal coinciding [3,4] . Intussusception has been associated with some forms of rotavirus vaccine. An early form of the vaccine (RRV-TV: Rotashield) was removed from the variation, with with seasonal peaks viral

gastroenteritis in some populations

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market because of a 22-fold increase in intussusception among vaccinated infants. The vaccine currently used in the United States (PRV: Rotateq) appears to have rates of intussusception that are similar to expected background rates. However, there may be additional cases of intussusception that have not been reported. Providers should be alert for cases of intussusception that may be associated with rotavirus vaccine, and report all suspected cases to the Vaccine Adverse event of Reporting patients (upper otitis System (VAERS). Approximately experience respiratory 30% viral tract illness infection,

potentially acts as a lead point for ileocolic intussusception [5]. A role for corticosteroids of this in the prevention of recurrence has been proposed in light possible association with lymphoid hyperplasia [10,11]. A lead point is recognized more

commonly in children older than five years [1,4,12]. A variety of pathologic conditions associated with intussusception, including small bowel lymphoma [13-15], Meckel diverticulum [16], duplication cysts [17,18] , polyps [19], vascular malformations [20] , inverted appendiceal stumps [21,22] , parasites [23,24], (eg, Ascaris lumbricoides) purpura Henoch-Schnlein

media, flu-like symptoms) before the onset of intussusception. A strong association with adenovirus infection has been shown in a variety of populations. In 30 to 40 % of cases, there is evidence of recent infection with enteric and non-enteric species of adenovirus [5-9]. In a prospective examining a case-control variety of study potential

[25], and cystic fibrosis [26], have been described. The mechanisms leading to

intussusception differ depending upon the specific cause. As examples: Small bowel intussusception may occur after an episode of HenochSchnlein purpura, with a bowel wall hematoma acting as the lead point. In these cases, intussusception typically occurs after resolution of the abdominal pain. In patients with cystic fibrosis, thick inspissated stool may act as the lead point [26] . Patients secondary excessive with to celiac disease may and wall

infectious triggers for intussusception in Vietnam and Australia, infection with adenovirus, as the strongest predictor of intussusception in both populations [9]. Viral infections, including enteric

adenovirus, can accentuate lymphatic tissue in the intestinal tract, resulting in hypertrophy of Peyer patches in the lymphoid-rich terminal ileum, which

develop small bowel intussusception dysmotility or bowel secretion

weakness [27,28].

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Patients

with

Crohn

disease

may

the painful episodes, the child may behave relatively normally and be free of pain. As a result, initial symptoms can be confused with gastroenteritis [30]. As symptoms progress, increasing lethargy develops. In up to 70 % of cases, the stool contains gross or occult blood [31]. The stool may be a mixture of blood and mucous, giving it the appearance of currant side of jelly. A sausage-shaped However, the abdominal mass may be felt in the right abdomen. sausage classically described triad of pain, a palpable shaped abdominal mass, and currant-jelly stool is seen in less than 15 percent of patients at the time of presentation [30,32] . As many as 20% of young infants have no obvious pain. Approximately one-third of patients does not pass blood or mucus or develop an abdominal mass. Occasionally, the initial presenting sign may be lethargy alone, or without altered pain, consciousness suggest an

develop intussusception because of inflammation and stricture formation [29] . Postoperative: Bowel intussusception (usually jejuno-jejunal) also has been described in the postoperative setting where it is an uncommon but insidious cause of intestinal obstruction. It usually occurs within two weeks of a laparotomy and is caused by adhesions. The patient typically does well for several days and may even resume oral intake before developing symptoms of mechanical obstruction. The diagnosis can be difficult to establish because it may be confused with postoperative paralytic ileus. The ultrasonography and computed tomography (CT) scanning may be helpful. Diagnostic contrast studies are not as helpful in postoperative intussusception because most cases occur in the small intestine, CLINICAL develop the MANIFESTATIONS: sudden severe, abdominal onset of pain,

Patients with intussusception typically intermittent, progressive crampy,

rectal bleeding, or other symptoms that intra-abdominal process [33-35]. In most cases, this clinical presentation occurred in infants, and was often confused with sepsis. Thus, intussusception should be considered in the evaluation of lethargy or altered consciousness, especially in infants. DIAGNOSIS AND NONSURGICAL high index of

accompanied by inconsolable crying and drawing up of the legs toward the abdomen. The episodes usually occur at 15 to 20 minute intervals. They become more frequent and more severe over time. Vomiting may follow episodes of abdominal pain. Initially emesis is nonbilious, but it may become bilious as the obstruction progresses. Between

TREATMENT A

suspicion coupled with early diagnosis

Invaginao intestinal

of

intussusception

may

obviate the

suggests that bowel perforation has occurred. A target sign, consisting of two concentric on the A radiolucent the right circles kidney superimposed and report within [36].

need for surgical intervention. For patients in whom the diagnosis is unclear at presentation, initial workup may include abdominal ultrasound or abdominal plain films, provided that these studies do not significantly delay the definitive diagnosis and treatment of intussusception. Patients with a typical presentation, in whom there is a high index of suspicion for intussusception, studies can may proceed the directly to an enema contrast study. These establish diagnosis of ileocolic intussusception, and frequently also are therapeutic. Before obtaining a contrast study, the patient should be stabilized and resuscitated with intravenous fluids; the stomach should be decompressed with a nasogastric tube. The patient should be considered fit for surgery, in case surgery is necessary, before he or she undergoes radiographic imaging. The surgical team should be notified before attempted reduction because there is a risk of perforation and because surgical intervention may be necessary if contrast enema fails to reduce the intussusception. Abdominal plain films Plain

representing peritoneal fat surrounding intussusception, density appeared in 26% of 94 patients in one soft-tissue projecting into the gas of the large bowel (intussusception image) is called the "crescent sign". The presence of air in the ascending colon can help to exclude intussusception in cases with a low clinical suspicion of the disease. In a retrospective study from a single center, plain radiographs with three views (supine, lateral, and prone) were used to screen patients with suspected intussusception [37]. The presence of air in the ascending colon on at least two of these views had high sensitivity for excluding intussusception in this patient population with a low clinical suspicion of disease (sensitivity 96.3 percent, 95% CI 89.2-100). However, the sensitivity of plain radiographs may be considerably lower in different clinical settings or when fewer views are analyzed. Therefore, we do not recommend relying on plain radiography to exclude intussusception if there is a significant clinical suspicion of the disease. Ultrasonography Ultrasonography also can be useful; its sensitivity and specificity approach 100 percent in the

radiographs of the abdomen may be helpful because they may show frank intestinal obstruction or massively distended loops of bowel with absence of colonic gas. Although rarely seen, the presence of pneumoperitoneum

Invaginao intestinal

hands

of

an

experienced

water ileum.

and bubbles

in the terminal

ultrasonographer [38-40] . The classic ultrasound image of intussusception is a "bull's eye" or "coiled spring" lesion representing layers of perfusion in the of the intestine intussusceptum within the intestine. In addition, a lack detected with color duplex imaging may indicate the development of ischemia. An advantage of ultrasonography is that it can diagnose the rare ileoileal intussusception and identify the lead point of intussusception in approximately two-thirds of cases in which underlying pathology exists [41]. Ultrasonography may also be useful in predicting which children are most likely to need surgical intervention for small bowel intussusception. In a small retrospective study, cases in which the intussusceptum was less than 3.5 cm were more likely to resolve spontaneously during or shortly after the ultrasonographic examination as compared to cases with a longer intussusceptum [42] . Reduction under ultrasonographic

Contrast of

studies The

standard is a

procedure for diagnosis and treatment ileocolonic intussusception contrast (air or radiopaque) enema [46]. Broad-spectrum antibiotics may be risk administered of before attempting with these nonoperative reduction since there is a perforation procedures. As compared or with ileocolonic jejunojejunal)

intussusception, small bowel (ileoileal, jejunoileal, intussusception is less likely to be reducible by contrast enema [47,48]. Intussusceptum length as measured by ultrasonography may be helpful in determining which patients may be managed expectantly, and which are likely to require surgical intervention. Barium and water-soluble barium has

contrast Traditionally,

been the preferred contrast agent in most North American and European centers [49-51]. Before instilling barium, a water-soluble contrast enema should be considered, particularly if there is a high risk of perforation. Water-soluble agents reduce the risk of electrolyte disturbances and peritonitis in patients in whom perforation has occurred. Barium enema reduction of

control without fluoroscopy has been described [43,44] . Signs of successful reduction include the disappearance of the intussusception and the retrograde flow of the water-soluble contrast into the small intestine. Ultrasound guided water enema also has been used [45]. Signs of successful reduction with water include the disappearance of the intussusception and the appearance of

intussusception can be performed if there is no evidence of perforation. The

Invaginao intestinal

standard method of reduction is to place a reservoir of barium 1 meter above the patient so that constant hydrostatic pressure is generated. With experience (and depending upon the clinical status may of the patient), a a physician undertake more

valve. However, benign and malignant lesions result, cannot a be distinguished alone. or As study by a radiologic examination repeat

even

laparotomy may indicate if any concern of a benign or malignant lead point exists [53]. After the successful reduction of an ileo-colic temperature release bacterial 12 to of intussusception, higher endotoxin, than a 38C or

aggressive reduction. In a typical ileo-colic intussusception, the barium column appears defect. as an The intraluminal-filling

(100.4F) often is noted because of the cytokines, The translocation. 24 hours for patient

intussusception can be found in any part of the large bowel, even the rectum. Occasionally, some barium may coat the outer surface of the intussuscipiens, resulting in a coiled spring pattern. Successful reduction is indicated by the free flow of contrast into the small bowel. Reduction is complete only when a good portion of the distal ileum is filled with barium, thus excluding ileoileal intussusception. Other indications of successful reduction include relief of symptoms and disappearance of the abdominal mass. A characteristic sound also may be appreciated with auscultation. In occasional patients, the contrast material does not reflux freely into the small bowel even with a complete reduction, but no filling defect in the cecum is present apart from the ileocecal valve and symptoms and signs of these patients resolve. A post-reduction filling defect in the cecum commonly is seen, probably the result of residual edema in the ileocecal

should be admitted to the hospital for observation. usually has is had as Nasogastric returned passage Feedings tolerated. Air an contrast Air alternative to the reduction contrast The and of then suction the a

maintained until bowel function has patient bowel are movement.

advanced

techniques have gained popularity as hydrostatic Foley methods into used [54-57]. the to

technique begins with insertion of a catheter is rectum. the Fluoroscopy assess

presence of bowel gas in the abdomen. Air is then instilled, initially by hand pump, until the diagnosis is established and the intussusceptum is pushed back gently [49,50] . An electric pump is connected if the intussusceptum stops moving despite the use of the hand pump. Both of these pumps must have a pressure

Invaginao intestinal

release system so that the pressure remains between 80 and 120 mmHg. Carbon dioxide can be used instead of air. It has the advantage of being absorbed less rapidly from than the air, gut, is associated with less discomfort, and is dangerous which potentially could cause an air embolism (although air embolisms have not been reported). Reflux of air into the terminal ileum and the disappearance of the mass at the ileocecal valve indicate reduction. An advantage of this method is the ease with which the intussusception can be reduced with less radiation exposure and cost, and the relatively harmless nature of air in the peritoneal cavity, compared with that of other contrast materials. Risk and complications The main risk of hydrostatic or pneumatic reduction is perforation of the bowel, which occurs in 1% or fewer patients [58-60]. The perforation usually occurs on colon, the and distal side of where the the intussusception, often in the transverse commonly intussusception was first demonstrated by radiographic studies [61,62] . The risk of perforation is greatest in infants younger than 6 months of age who have had symptoms for three days or longer and in patients with evidence of small bowel obstruction [63]. Nonoperative reduction should not be attempted in patients with prolonged

symptoms or any signs of peritoneal irritation or free peritoneal air. Success techniques is rate Nonoperative successful in

reduction using barium or air contrast approximately in 60 to 90% of patients with ileo-colic intussusception [3,6466]. Success is more likely to be achieved in patients with idiopathic intussusception (ie, no identifiable lead point), although in it also can with be a accomplished patients

recognized lead point [53]. Successful radiographic reduction is more likely if, during reduction, the small bowel is visualized before the appendix [67]. lleo-ileo-colic intussusception may be more difficult to reduce because the barium often percolates along the loops of small bowel in the colon, reducing the effective pressure of the enema. In addition, success is less likely to be achieved in infants younger than 1 year of age (particularly younger than 3 months), in children older than 5 years of age, and when plain films show signs of intestinal obstruction [30,58,64] . Although some authors have noted a reduced likelihood of reduction when symptoms have been present for longer than 48 hours [30,58,66,68] , others have found no such correlation [65] . In these high-risk cases, the barium study should still be performed to confirm the diagnosis and attempt therapy, but a pediatric surgeon should be readily available in the imaging department.

Invaginao intestinal

Recurrence [30,69,70].

after Recurrence

successful is not

ileocecal valve. Thus, when a filling defect appears to be associated with edema in the area, an ultrasonographic or repeat contrast study should be performed provided that symptoms have resolved. Broad-spectrum intravenous antibiotics should be given before surgery. Manual reduction at operation is attempted in most cases, but resection with primary anastomosis needs to be performed if manual reduction is not possible or if a lead point is seen.

nonoperative reduction is close to 10% necessarily an indication for surgery. Each recurrence should be handled as if it were the first episode, provided that each is successfully reduced [71]. SURGERY Surgery is indicated when nonoperative reduction is incomplete or when a persistent filling defect, indicating a mass lesion, is noted [71] . As noted above, a residual filling defect may be seen because of edema of the

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