List of Abbreviations
ABT - antibiotic therapy
AP - anatomical cause
IBD - inflammatory bowel disease
USHC - ultrasonographic colonoscopy (ultrasound examination of the large intestine)
GI - gastrointestinal tract
II - intestinal intussusception
ICI - ileocecal intussusception
ABG - acid-base balance
LD - laparoscopic reduction
MSE - medical and social expert evaluation
CBC - complete blood count
UA - urinalysis
ABO - acute bowel obstruction
PBE - pneumatic barium enema (air contrast enema - radiological examination of the large intestine)
RCT - randomized controlled trial
SCT – spiral computed tomography
Ultrasound — ultrasound examination
CDI - color Doppler imaging
Terms and Definitions
Anatomical cause – a space-occupying gastrointestinal lesion acting as a lead point for bowel invagination
Bezoar - a gastrointestinal foreign body (calculus) formed from organic (hair, plant matter, etc.) and inorganic material. Most frequently found in the stomach
Henoch-Schönlein purpura – a systemic hemorrhagic vasculitis primarily affecting small vessels
Hematochezia - blood in the stool
Hydrostatic reduction – reduction of intussusception by creating pressure within the bowel through fluid administration
Hydro-echocolonoscopy - ultrasound-guided reduction of intussusception by creating pressure within the bowel through fluid administration via the anus
Meckel's diverticulum – a congenital gastrointestinal malformation presenting as a local outpouching of the ileal wall resulting from incomplete obliteration of the vitelline (omphalomesenteric) duct located 10-100 cm from the ileocecal junction, frequently containing heterotopic gastric and/or pancreatic tissue
Burkitt lymphoma – a B-cell lymphoma
Nonspecific mesadenitis – reactive inflammation of mesenteric lymph nodes caused by nonspecific, typically viral infections
Peyer's patch – an aggregation of lymphoid follicles in the distal ileum and proximal large intestine
Pneumostatic reduction - reduction of intussusception by creating pressure within the bowel through air insufflation
Pneumo-enema - fluoroscopy-guided reduction of intussusception by creating pressure within the bowel through air insufflation via the anus Condition – alterations in the body arising from the impact of pathogenic and/or physiological factors, requiring medical care
Surgical treatment – a method of treating a disease by dividing and uniting tissues during a surgical operation
Peutz-Jeghers syndrome – a genetic hamartomatous gastrointestinal polyposis.
Severity of condition – a composite assessment of vital bodily functions
Kloiber's sign (cup) – a horizontal fluid level with a dome-shaped radiolucency above it, resembling an inverted cup.
1. Brief information on the disease or condition
1.1 Definition of the disease or condition
Intussusception (II) – the invagination of one segment of the bowel into the lumen of another.
II is one of the most common causes of acquired acute bowel obstruction in young children and represents a combined form of intestinal obstruction (obstructive and strangulatory) [1-10]. Three cylinders (external, middle, internal), a head, and a neck are distinguished within the intussusceptum.
1.2 Etiology and pathogenesis of the disease or condition (group of diseases or conditions)
Intussusception in children is typically idiopathic, but may also be provoked by anatomical causes (space-occupying lesions) acting as a lead point for bowel invagination. II can also be transient and occur postoperatively following abdominal surgeries.
According to the World Health Organization, most researchers worldwide view intussusception in young children as idiopathic, citing a lack of sufficient evidence to consider viral or bacterial infection as the etiological factor [1]. The primary risk factor for idiopathic intussusception (86-95%) in young children is nonspecific mesadenitis associated with a prior viral infection [1, 7-11]. Pronounced lymphoid hyperplasia presenting as terminal ileitis and enlarged Peyer's patches may act as a lead point for invagination [7-14]. The introduction of complementary feeding is considered the main factor provoking intestinal invagination in infants [2-10]. Intestinal (viral and bacterial) infections [2-10, 15,16] as well as rotavirus vaccination [8, 17, 18] can also lead to the development of intussusception. An important role in the occurrence of intussusception in young children is played by the immaturity of the regulatory and enzymatic systems of the gastrointestinal tract and the anatomical features of the ileocecal region: a mobile cecum, and underdevelopment of the valve apparatus of the ileocecal valve [2, 3, 5, 6, 9]. The main aspect of the pathogenesis of intestinal invagination in these cases is dyscoordination of intestinal motility with a predominance of circular muscle layer contractions [2-10].
Pathological intussusception (4-14%) is associated with the presence of a lead point for invagination [2-14] and can occur at any age. In infants and young children, the frequency of anatomical causes (AC) ranges around 5-7% and increases in older children and adolescents [2-14]. The role of the lead point of invagination is played by ACs, as well as foreign bodies, helminthic infestations, bezoars, diffuse changes in the intestinal wall associated with hemorrhages in Henoch-Schönlein purpura, or inflammatory changes in IBD and necrotizing enterocolitis. Intussusception is most frequently provoked by Meckel's diverticulum, polyps (juvenile polyp, Peutz-Jeghers syndrome), enlarged Peyer's patches, and Burkitt lymphoma [2-14]. Rare causes include vascular malformations, gastrointestinal duplication, and adhesions [2-14].
The disease occurs as a result of the invagination of the proximal bowel segment into the lumen of the distal segment along the direction of intestinal peristalsis (luminal obstruction), and extremely rarely in reverse. The mesentery of the proximal section also invaginates into the lumen of the distal bowel, becoming compressed, which results in venous stasis and edema of the intestinal wall, followed by progressive impairment of blood supply (strangulation) and adhesion of the peritoneal surfaces of the intussusceptum cylinders. Further isoperistaltic advancement of the intussusceptum exacerbates circulatory disorders in the bowel. Ischemic changes develop most rapidly in the neck of the intussusceptum.
Thus, the main etiopathogenetic factor in both infants and children over one year of age is dyscoordination of intestinal peristalsis, but in infants the provocative factor is alimentary, whereas in older children it is nonspecific mesadenitis. Anatomical causes can provoke II in children of any age.
1.3 Epidemiology of the Disease or Condition
The epidemiology of II depends on ethnicity, geographical location, and the economic and social development of the country. According to a 2013 review (Jiang J. et al.) involving 44,454 patients from all continents, the incidence of II in infants under one year of age is 74 (9-328) cases per 100,000 children under 1 year of age [19]. Regions with a high incidence include Asian countries (China, South Korea, Vietnam, Japan). For instance, in Vietnam, the incidence of II in infants under one year is 302 cases per 100,000 children [17]. Europe and North America can be classified as regions with a moderate incidence level. According to WHO data, the incidence of II in Europe ranges from 1.1 to 4.3 cases per 1,000 live births, and in North American countries from 0.5 to 2.3 cases per 1,000 live births [1]. Furthermore, incidence rates in European countries vary significantly: from 20 cases in Finland to 60-66 per 100,000 children in Germany, Denmark, and England; the incidence of II in North America is 33-49 cases per 100,000 infants under one year [19]. Russia can also be classified as a country with a moderate incidence level. According to V.V. Podkamenev et al., the incidence of II in Russia can be judged by publications based on hospital data [9]. Thus, from 7 to 45 children with II are hospitalized annually in various clinics in Russia [9]. Pediatric hospitals in Europe and North America admit between 5 and 35 children per year [10].
A low incidence rate has been observed in Muslim countries. According to WHO data, the incidence of intussusception in Kuwait is 0.5 per 1,000 live births, and in Saudi Arabia, it is less than one case per year [1]. According to a review by Jiang J. et al., Bangladesh is among the regions with the lowest incidence of intussusception—9 cases per 100,000 children under one year of age [19]. A publication from Israel reported that among children with intussusception, Jews outnumbered Arabs by more than 2 times [20].
The disease occurs most frequently in boys, with a peak in infancy (4–9 months) [1-11, 19-21]. Overall, intussusception is most common in infants and young children: 60% of cases occur between 6 and 12 months of age, 90% under the age of 3 years, and only 10% in older children and adolescents [8]. Infants under 3 months of age account for less than 1% of the overall incidence of intussusception [8]. There are isolated reports of intussusception in newborns in the literature [7-10, 22, 23], as well as publications on rare clinical cases of intrauterine intussusception manifesting as gastrointestinal atresia at various levels in newborns [9, 24].
Publications after 2000 note both an annual decrease in the overall incidence of intussusception and a decrease in its frequency among infants in their first year of life. Over 20 years, the incidence of intussusception in Denmark has decreased by 55% [20]. According to Canadian researchers, the proportion of patients in their first year of life has decreased by 42% [21]. According to V.V. Podkamenev et al., infants under one year of age accounted for 77.6% of cases in the 1980s, whereas today they account for only 6.5% [9]. Other Russian authors also point to a shift in the age range toward an increasing number of intussusception cases in children over one year old [11, 25, 26].
Seasonal variations in the occurrence of intussusception are described inconsistently across various publications [1]. Earlier publications indicate a correlation with the dry summer season or the rainy season in tropical and subtropical regions, as well as a correlation with the months experiencing peak hospitalizations of children with acute gastroenteritis and respiratory infections [1]. According to a review by Jiang J. et al., which included 14,234 patients, no statistically significant differences in the frequency of intussusception cases across the months of the year were found [19].
An epidemiological feature of intussusception is the predominance of boys over girls [1-12, 19-21]. On average, the male-to-female ratio ranges from 2:1 to 3:1 [2]. According to WHO data, publications from Africa report a male-to-female ratio reaching 8:1, and in India, 9:1 [1].
Mortality is virtually nonexistent in developed countries [1]. In most European hospitals, the treatment of intussusception is not associated with mortality [1]. In developing countries, mortality persists. In African countries, mortality from intussusception among hospitalized children averages 9.4% (2–25%) [1], whereas this figure is 0.2% (0–6%) for Asian countries, 0.6% (0–1) for Central and South America, 0.8% (0–5%) for the Mediterranean, 0.1% (0–1) for Europe, 0.4% (0–1) for North America, and 0% (0–0.1%) for Oceania [19]. On average, mortality in developed countries is 0.3% (0–0.6%) [18], while mortality in developing countries reaches 10–20% [19].
1.4 Features of Coding the Disease or Condition
K56.1 - Intussusception
1.5 Classification of the disease or condition
Classification issues regarding the disease are discussed in detail in publications by domestic and foreign authors and in manuals from the second half of the last century [3-7]. The disease stages described in Russian guidelines (initial signs, pronounced clinical manifestations, incipient complications, severe complications) reflect its pathogenesis and the potential development of complications due to intestinal necrosis. However, time frames are highly conventional and cannot serve as a definitive criterion for systematization. The most crucial aspects are diagnosing complications and assessing the severity and stability of the patient's condition. From this perspective, a distinction should be made between uncomplicated and complicated intussusception.
In the practical work of a surgeon, the classification based on the localization of the intestinal intussusceptum, the clinical course of the disease, and the presence of complications is of primary importance.
By localization:
Ileocecal
Small bowel
Colonic
Rare forms
By course:
Acute
Chronic
Recurrent
Transient
Complications:
Intestinal necrosis
Intestinal perforation
Peritonitis
Small bowel obstruction
Depending on the localization, ileocecal, small bowel, and colonic intussusception are distinguished [2-10]. Most frequently (90-95%) in children, ileocecal intussusception develops, which can be ileocolic (the small intestine invaginates through the ileocecal valve into the ascending colon) and cecocolic (the dome of the cecum invaginates into the cecum and ascending colon, sometimes pulling the ileum along without invaginating through the ileocecal valve) [2-10]. Ileocolic intussusception is most common in children (78-85%) [2-11]. Diagnosing the type of intussusception during conservative treatment is practically impossible. Isolated invagination of the small intestine into the small intestine and the large intestine into the large intestine occurs relatively rarely in children (5–10%) [2-11], and, as a rule, has a provoking anatomical cause. Rare forms of intestinal invagination include isolated invagination of the appendix, Meckel's diverticulum, multi-cylinder intussusception, as well as retrograde, multiple, and postoperative intussusception [2-11]. Postoperative intussusception extremely rarely develops in the postoperative period after traumatic abdominal surgeries and, as a rule, is associated with the development of adhesions. Cases of ileocecal intussusception have been described, but small bowel intussusception is most common in the first two weeks after surgery [8].
According to its clinical course, intussusception in children most frequently has an acute character—the disease develops suddenly against the background of relative well-being and requires emergency care. Chronic intussusception in children is diagnosed extremely rarely. In cases of chronic intussusception, complete obstruction of the intestinal lumen does not occur, and circulatory disorders with necrosis of the intestinal wall do not develop. The most frequent cause of chronic intussusception is colonic intussusception against the background of polyps (Peutz-Jeghers syndrome). Spontaneous reduction of intussusception is well known, since the movement of food through the intestine occurs due to the rhythmic contraction and relaxation of the circular and longitudinal muscles of the intestinal wall, including the formation of micro-intussusceptions that spontaneously reduce. Transient intussusception may be accompanied by clinical symptoms, but more often it is asymptomatic and detected by ultrasound. Transient intussusceptions are most characteristic of the small intestine and are diagnosed during ultrasound as short-lived intestinal invaginations of insignificant length with subsequent reduction during the examination [8]. As a rule, transient intussusception is registered against the background of enteritis of various etiologies and does not require special treatment. Treatment of the underlying disease under the supervision of a pediatrician is necessary. Recurrent intussusception refers to the course of the disease with a relapse of intestinal invagination after its conservative or surgical elimination. According to various authors, recurrent intussusception accounts for 4.5 to 18.4% [9].
1.6 Clinical presentation of the disease or condition
The clinical picture of intussusception depends on the form of invagination and the duration of the disease. The typical picture of intussusception described in most guidelines refers to ileocecal intussusception [2-12, 25-32]. In most cases, intestinal invagination begins suddenly, amidst complete health. The child becomes restless, cries, draws legs up to the abdomen (monkey sign), and refuses to eat. The face acquires a suffering expression. The bout of restlessness ends as suddenly as it begins, but after a short period of time it repeats again; vomiting joins, which has a reflex character and is associated with strangulation of the mesentery of the invaginated segment of the intestine. At later stages of the disease, vomiting is caused by intestinal obstruction. Body temperature is most often normal, but may be elevated. In the first hours of the disease, normal stool may occur due to the emptying of the distal intestine. After some time, mucus mixed with blood—"raspberry jelly"—is passed from the rectum instead of feces. Stool in the form of "raspberry jelly" is one of the most important pathognomonic symptoms of intussusception [2-12, 25-31]. Upon abdominal palpation, usually in the right mesogastrium, a tumor-like mass can be determined in the form of a smooth, slightly mobile, slightly painful soft-elastic consistency roll,
Thus, the typical clinical picture of ileocecal intussusception distinguishes the main triad of symptoms:
Pain, manifested by bouts of restlessness with pain-free intervals, is the primary symptom present in all patients and is the main reason for seeking medical care. The pain is ischemic in nature and occurs due to strangulation of the intestinal mesentery, and as a rule, is colicky in character, which is caused by the peristalsis of the intestinal tube: when the peristaltic wave reaches the segment of the intestine with invagination, pain occurs.
«Raspberry jelly» stool—mucus stained with blood due to diapedesis of erythrocytes into the intestinal lumen. In English-language literature, this symptom is frequently described as a red «currant jelly» stool [7,8,27-29].
Pathological mass—a space-occupying lesion felt upon abdominal palpation as an elongated, smooth, moderately mobile mass with a soft-elastic consistency.
Additional symptoms of ileocolic intussusception include vomiting and Dance's sign. In rare cases of extensive intussusception, prolapse of the intussusceptum through the anus may occur.
Vomiting can be single or recurrent. Vomiting at the onset of the disease is caused by a viscero-visceral reflex, but in cases of intussusception necrosis with peritonitis, it may be a manifestation of small bowel obstruction.
Dance's sign (the «empty right iliac fossa» sign) is caused by the displacement of the cecum into the ascending and colon.
The classical triad of symptoms undoubtedly allows for the diagnosis of intussusception. However, according to recent publications, it is present in no more than 20–30% of cases [8, 12, 26-29]. A typical clinical picture is characteristic of ileocolic intussusception in children under 1 year of age [2-11, 27-32]. In children over one year old, as well as in other types of intussusception, the clinical picture is blurred, which introduces certain difficulties in diagnosing the disease. The potential for the rapid development of structural and ischemic changes in the intestinal wall and abdominal complications (peritonitis, obstruction) with severe pathophysiological disorders in the young patient dictates the need for timely diagnosis and treatment.
2. Diagnostics, medical indications and contraindications for diagnostic methods
Diagnostic criteria:
anamnestic data indicating the time of symptom onset, preceding acute illnesses (viral and intestinal infections), dietary disturbances and introduction of complementary foods, patient vaccination, and a history of intussusception
physical examination findings characteristic of intussusception (see Section 1.6)
instrumental examination data
laboratory test results
It is recommended to perform all evaluations in all patients hospitalized with suspected intussusception in order to establish a diagnosis [2-15, 19-21, 27-32]
GCR BLoE 3Level of Evidence B (Level of Confidence of Evidence – 3)
2.1 Complaints and Anamnesis
It is recommended to inquire about the disease duration and the nature of abdominal pain in all patients (or their parents) in order to establish a diagnosis [2-11, 27-32]
GRC CLEL 4Grade of Recommendation C (Level of Evidence – 4)
Comments: Abdominal pain begins acutely, against a background of complete well-being, is colicky in nature, and is accompanied by child restlessness alternating with pain-free («silent») intervals. Over time, the intervals between pain episodes decrease while the duration of the episodes increases.
It is recommended to ascertain the presence of intussusception risk factors (introduction of complementary foods, dietary errors, acute illnesses within the past month, vaccination) in all patients (or their parents) in order to establish a diagnosis [2-21,23-30]
GCR BLoE 3Level of Evidence B (Level of Confidence of Evidence – 3)
Comments: The primary risk factor for idiopathic intussusception in infants under one year of age is the introduction of complementary foods. In young children, idiopathic intussusception is typically associated with the development of nonspecific mesenteric lymphadenitis following viral infections and may also develop against the background of acute gastroenteritis. There are indications of a link between rotavirus vaccination and the development of intussusception [17,18].
It is recommended to inquire about a history of intussusception and the treatment method used in all patients (or their parents) in order to establish a diagnosis [9, 12, 33-42]
GRC CLEL 4Grade of Recommendation C (Level of Evidence – 4)
Comments: The incidence of recurrent intestinal intussusception in children ranges from 4.5% to 20% [9,12,33-42]. Surgical treatment is associated with a lower risk of recurrent intussusception. The primary causes of recurrent intussusception are lymphoid hyperplasia and anatomical factors [12].
It is recommended to check for the presence of blood in the child's stool and the time of onset of this symptom in all patients (or their parents) in order to establish a diagnosis [2-15, 19-21, 25-32].
GCR BLoE 3Level of Evidence B (Level of Confidence of Evidence – 3)
Comments: «Raspberry jelly» stool is a pathognomonic symptom of ileocolic intussusception in children and occurs in 50–60% of cases [2-12, 25-32]
It is recommended to inquire about additional symptoms of the disease (vomiting, fever, diarrhea, delayed stool, etc.) and the time of symptom onset in all patients (or their parents) in order to establish a diagnosis [2-15, 25-32]
GRC CLEL 4Grade of Recommendation C (Level of Evidence – 4)
Comments: A detailed characterization of symptoms allows for the differential diagnosis of abdominal pain syndrome.
2.2 Physical Examination
Physical examination may reveal pathognomonic symptoms of intussusception. Abdominal palpation reveals pain and a pathological mass in the right mesogastrium. However, child restlessness and active muscular defense frequently preclude palpation of the intussusceptum. Digital rectal examination typically reveals an empty rectal ampulla; the head of the intussusceptum may also be palpated if located low, or a tumor-like mass can be detected via bimanual palpation. An enema helps determine the presence and characteristics of the stool. Obtaining «raspberry jelly» stool supports the diagnosis of intussusception.
It is recommended that all patients with suspected intussusception undergo a general physical examination by a pediatric surgeon and have their severity of condition assessed (level of consciousness, respiration, circulation: general thermometry, heart rate measurement, peripheral blood pressure measurement, respiratory rate measurement, pulse oximetry) to determine further management tactics [2-15, 25-32]
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: in intussusception, the child's general condition is usually only mildly affected. In cases of bowel necrosis, perforation, peritonitis, small bowel obstruction, or sepsis, the child's condition may be critical.
It is recommended that all patients with suspected intussusception undergo abdominal palpation (palpation for large intestine disorders, palpation for small intestine disorders) to identify symptoms of the disease [2-15,25-32].
GRC CLEL 4Grade of Recommendation C (Level of Evidence – 4)
Comments: Abdominal palpation may reveal localized tenderness and muscle guarding over the intussusceptum; in some cases, the intussusceptum itself can be felt as an elongated, moderately mobile, soft-elastic mass, typically in the right mesogastrium, along with Dance's sign in ileocolic intussusception. In small bowel intussusception, the intussusceptum is detected less frequently; it is more mobile, smaller, and localized in the periumbilical region. In colonic intussusception, the palpable mass is most often located in the left hypochondrium or left iliac region. With the development of peritonitis, positive peritoneal signs are elicited.
It is recommended that all patients with suspected intussusception undergo pulmonary and abdominal auscultation to identify symptoms of the disease [2-15,25-32].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: Pulmonary auscultation helps rule out pulmonary pathology that may mimic an «acute abdomen». Abdominal auscultation assesses bowel sounds and detects pathological bruits in the development of small bowel obstruction.
2.3 Laboratory Diagnostics
When intestinal intussusception is suspected, a standard laboratory workup for abdominal syndrome should be performed.
It is recommended that all patients with suspected intussusception undergo a complete blood count (CBC) for diagnostic purposes [2-15,25-32].
Level of Evidence CGrade of Recommendation 5Level of Clinical Effectiveness C (level of evidence – 5)
Comments: An elevated leukocyte count may indicate bowel necrosis with an inflammatory process in the abdominal cavity. A decrease in hemoglobin and erythrocyte levels may indicate pre-existing anemia, as «raspberry jelly» stool does not lead to a drop in red blood cell parameters. An elevated hematocrit, hemoglobin, and erythrocyte count may indicate hypovolemia.
It is recommended to perform a urinalysis in all patients with suspected intussusception for diagnostic purposes [2-15,25-32].
Level of Evidence CGrade of Recommendation 5Level of Clinical Effectiveness C (level of evidence – 5)
Comments: Urinalysis aids in the differential diagnosis of abdominal pain syndrome.
It is recommended to perform acid-base balance and blood gas testing in patients with a complicated course of intussusception whose general condition is rated as «severe» for diagnostic purposes [2-15,25-32].
Level of Evidence CGrade of Recommendation 5Level of Clinical Effectiveness C (level of evidence – 5)
Comments: Possible abnormalities are signs of hypovolemia and the development of a critical condition. An elevated lactate level may indicate the presence of bowel necrosis.
It is recommended to perform a general biochemical blood test in patients with a complicated course of intussusception whose general condition is rated as «severe» for diagnostic purposes [2-15,25-32].
Level of Evidence CGrade of Recommendation 5Level of Clinical Effectiveness C (level of evidence – 5)
Comments: A general therapeutic biochemical blood analysis allows for the assessment of the blood protein pool and organ failure, as well as the level of C-reactive protein as a marker of the infectious process in the development of peritonitis.
It is recommended to perform a coagulogram (screening test of the hemostatic system) in patients with a complicated course of intussusception whose general condition is assessed as "severe" for diagnostic purposes [2-15,25-32].
Level of Evidence CGrade of Recommendation 5Level of Clinical Effectiveness C (level of evidence – 5)
Comments: Assessment of APTT, fibrinogen, and INR levels allows for the diagnosis of hypocoagulation and disseminated intravascular coagulation (DIC) syndrome in a complicated course of intussusception, development of peritonitis, or sepsis.
2.4 Instrumental Diagnostics
Imaging methods play a key role in the diagnosis and treatment of intussusception. High diagnostic accuracy, the absence of ionizing radiation, and patient comfort make ultrasound the preferred imaging modality for diagnosing intussusception. Abdominal X-ray has a low diagnostic value regarding intussusception. The diagnostic value of an abdominal X-ray is only 25% [41, 42]. Barium enema (X-ray examination of the colon) with radiopaque agents containing barium sulfate** in many countries around the world, as well as air-contrast enema (X-ray examination of the colon) in our country, have been the standard for diagnosing intussusception for many years. The advantage of diagnosing intussusception using enema is the ability to immediately proceed to a therapeutic procedure. Spiral computed tomography (SCT) is usually not used for diagnosing intussusception. Nevertheless, it is an effective imaging tool for many abdominal diseases, which can be useful in patients with atypical complaints, including for diagnosing anatomical causes provoking intussusception [8, 43]. A number of authors recommend performing colonoscopy to diagnose intraluminal lead points (Burkitt's lymphoma, juvenile polyp, polyps in Peutz-Jeghers syndrome) as well as performing a step-by-step biopsy, which allows for differential diagnosis between inflammatory bowel disease, lymphoid nodular hyperplasia, and a neoplastic process (Burkitt's lymphoma) [12].
It is recommended that all patients with suspected intussusception undergo an abdominal ultrasound (ultrasound examination of the small, large, sigmoid, and rectum; ultrasound detection of free fluid in the abdominal cavity) to establish the diagnosis [2, 7-12, 44, 46-57].
GCR ALEL 1Grade of Recommendations A (Level of Evidence – 1)
Comments: Ultrasound is the primary diagnostic method for intussusception in children, with a sensitivity and specificity of 90-100%. The sonographic appearance of intussusception was first described by D.L. Weissberg in 1977. Since then, numerous studies have appeared describing such signs of intussusception as the "target", "donut", or "bull's-eye" sign with multiple rings in cross-section, and the "pseudokidney", "layered cake", or "fork" sign in longitudinal section [46-57]. The advantages of the method include the ability to evaluate any segment of the intestine, determine the viability of the intestinal wall, and identify additional inclusions that may act as the lead point of intussusception. In addition, this method is safe and does not expose the child's body to ionizing radiation, making it possible to use it repeatedly for dynamic monitoring. The diagnostic value of ultrasound in suspected intussusception in children has been confirmed by numerous studies [46-57].
It is recommended that all patients with suspected intussusception undergo an abdominal X-ray when ultrasound is not feasible and for diagnosing complications of intussusception (perforation, small bowel obstruction) [2-15, 25-32, 42-44].
GCR BLoE 3Level of Evidence B (Level of Confidence of Evidence – 3)
Comments: Abdominal X-ray can be useful in diagnosing ileocolic intussusception. Intussusception can be diagnosed by the pathognomonic crescent sign, which is the presence of a curvilinear mass along the course of the colon on the radiograph. However, the diagnostic value of radiography for intussusception does not exceed 25% [43, 44]. In addition, signs of small bowel obstruction may be present: air-fluid levels in the small intestine (Kloiber's sign) and absence of gas in the large intestine [44]. Abdominal X-ray can also be used to diagnose free air when bowel perforation is suspected and in unclear clinical presentations to identify other acute abdominal conditions [2, 7, 8-10, 43].
It is recommended that all patients with suspected intussusception undergo an enema (pneumatic or using a contrast agent) when ultrasound is not feasible to establish the diagnosis [2-10, 25, 27-30, 43, 44].
GCR BLoE 3Level of Evidence B (Level of Confidence of Evidence – 3)
Comments: Enema has been the standard for diagnosing ileocecal intussusception for many years. The classic signs of intussusception are the "crescent" and "fork" signs. These symptoms occur as a result of the contrast agent filling the space between the colon wall and the intussuscepted ileum. The advantage of enema is the ability to immediately proceed to a therapeutic procedure.
It is recommended that patients with suspected intussusception and an unclear clinical presentation undergo spiral computed tomography (SCT) (Spiral computed tomography of the abdominal organs with intravenous bolus contrast enhancement, multiplanar and three-dimensional reconstruction) if equipment and a specialist are available, for the purpose of differential diagnosis of the causes of abdominal syndrome [2, 7, 8-10, 45].
GRC CLEL 4Grade of Recommendation C (Level of Evidence – 4)
Comments: SCT has 100% sensitivity for diagnosing intussusception and can be useful for diagnosing anatomical causes of intussusception. SCT is an effective imaging tool for many abdominal diseases.
2.5 Other Procedures
When intussusception is suspected, differential diagnosis within the framework of acute abdominal syndrome should be performed, ruling out diseases accompanied by cramping pain, vomiting, hematochezia, and the presence of an abdominal mass. If necessary, consultations with specialist physicians and additional instrumental examinations prescribed by specialists are recommended.
It is recommended that all patients with intussusception undergo an initial examination (evaluation, consultation) by a pediatrician in case of an unclear diagnosis for the purpose of differential diagnosis of the causes of abdominal syndrome and follow-up after conservative treatment of intussusception [2-15, 25-32].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: Differential diagnosis is most often performed with viral diarrhea, dysentery, Henoch-Schönlein purpura, gastrointestinal bleeding from Meckel's diverticulum ulcers, and gastrointestinal bleeding from colonic polyps. It should be noted that all these conditions can act as risk factors for intussusception. Rotavirus vaccination can also become a factor provoking intussusception in infants.
It is recommended that all patients hospitalized in the ICU after surgical treatment of intussusception undergo multi-parameter monitoring [2-15].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: All patients requiring treatment in the ICU must undergo multi-parameter monitoring, including measurement of heart rate, measurement of peripheral blood pressure, measurement of respiratory rate, pulse oximetry, electrocardiography, echocardiography, and comprehensive abdominal ultrasound.
3. Treatment, including drug and non-drug therapies, diet therapy, and pain management
Approaches to the treatment of intussusception vary across countries. In developing countries, surgical treatment is most frequently resorted to. In developed countries, the treatment strategy depends on the type of intussusception and the patient's condition. In cases of idiopathic intussusception without complications, conservative treatment is indicated. If conservative treatment is ineffective, a lead point is diagnosed, complications develop, or the patient's condition is unstable, surgical treatment is performed.
3.1 Conservative treatment methods
Two main methods are used for non-operative reduction of the intussusceptum: pneumostatic and hydrostatic reduction (release of the bowel invaginated into another). Imaging methods for dynamic control of reduction are fluoroscopy and ultrasound. Moreover, both pneumostatic and hydrostatic reduction are performed under either fluoroscopic or ultrasound guidance.
It is recommended that all patients with uncomplicated ileocolic intussusception undergo conservative (pneumostatic or hydrostatic) reduction (release of the bowel invaginated into another) [2-15, 25-32,56-71].
GCR ALEL 1Grade of Recommendations A (Level of Evidence – 1)
Comments: The main principle of conservative reduction of intussusception is to increase intraluminal pressure in the colon to push the intussuscepted ileum back through the ileocecal valve. According to a meta-analysis published in 2020, which included 20,734 patients (from 85 observational and 5 randomized clinical trials (RCTs) worldwide), the efficacy of pneumatic reduction was 81% (95% CI, 78–84%), and that of hydrostatic enema under ultrasound guidance was 82% (95% CI, 79–85%), with an identical perforation rate of 1% (95% CI, 1–2%) [42]. The success rate of fluoroscopy-guided hydrostatic enema was 67% (95% CI, 60–73%), and for ultrasound-guided pneumatic enema, it was 93% (95% CI, 90–95%), with perforation rates of 2% (95% CI, 1–3%) and 1% (95% CI, 0–5%), respectively [41]. Fluoroscopy-guided pneumatic reduction and ultrasound-guided hydrostatic reduction are equally reliable and safe methods [42].
Pneumatic enema (air enema) is the primary method for conservative treatment of intussusception in children in our country, dating back to the mid-20th century [3–6, 9]. Modifications of the method were detailed by V.M. Portnoy (1962) [58] and L.M. Roshal (1964) [4]. The main advantage of the method is the generation of high pressure within the colonic lumen, while the disadvantage is exposure to ionizing radiation. The efficacy of the method has been confirmed by numerous studies and reaches 88–99% [4, 10, 11, 44, 59, 60].
Air is introduced into the rectum using a Richardson's bulb, and its advancement is monitored via fluoroscopy (contrast enema/lower GI series). The air pressure in the colon should not exceed 60–80 mm Hg to avoid bowel injury. When the intussusceptum is reduced, air enters the small intestine, and the pressure on the manometer decreases. If necessary, no more than 3 attempts are performed.
Ultrasound-guided hydrostatic reduction – hydro-echocolonoscopy (HEC). This method has been used in many countries worldwide since the early 1980s [61]. In Russia, the method is used in selected clinics [61–64]. The main advantage of the method is the absence of ionizing radiation. Also noteworthy is the ability to assess blood flow in the intussusceptum and diagnose pathological echogenic formations acting as a lead point. The efficacy and safety of the method have been confirmed by numerous studies and are comparable to pneumatic enema [44, 50, 59–67].
It is recommended to perform HEC in the operating room under multi-component endotracheal anesthesia with relaxation of the anterior abdominal wall muscles. Sodium chloride** solution is injected into the rectum at a constant pressure of 80–100 cm H2O. Fluid advancement is monitored sonographically. The criteria for successful reduction are visual resolution of the intussusceptum, entry of fluid into the terminal ileum, and the appearance of the characteristic "pseudokidney" or "honeycomb" sign. If necessary, no more than 3 attempts are performed.
Fluoroscopy-guided hydrostatic reduction. Fluoroscopy-guided hydrostatic enema was long the primary method used in many countries worldwide. Barium sulfate-containing radiopaque agents were the first contrast agents used for this procedure, but their use has steadily declined due to the higher risk of peritonitis in the event of perforation [60]. Currently, water-soluble contrast agents are predominantly used in the United States, with only 20% of radiologists utilizing this method, compared to 78% using pneumatic enema [60]. A large number of studies have compared the efficacy and safety of these methods over years of use, including an extensive 2015 meta-analysis involving more than 32,000 cases. The authors concluded that pneumatic reduction is superior to hydrostatic reduction in efficacy (85.6% vs. 65.0%) with no differences in perforation rates [59]. According to the 2020 meta-analysis, the pooled efficacy of the method was the lowest of all at 67% (95% CI, 60–73%) [44].
Ultrasound-guided pneumatic reduction theoretically represents the best approach for conservative reduction. The method does not require ionizing radiation and provides high intraluminal pressure. The procedure was first described in 1990 by T. Todani et al. [68], but it has not yet become widespread. The disadvantages of the method include more challenging visualization of the intussuscepted bowel during the procedure and, consequently, more difficult confirmation of successful reduction, as well as more difficult diagnosis of bowel perforation [68–71]. According to D. Plut et al., the pooled efficacy of the method was 93% (95% CI, 90–95%), with a perforation rate of 1% (95% CI, 0–5%) [44]. There are no publications in domestic literature reporting the use of this method for the conservative treatment of intussusception.
Sedation and general anesthesia. The use of sedation and general anesthesia during conservative treatment of intussusception varies significantly across different institutions [73-78]. The benefit of sedation and general anesthesia in the conservative management of intussusception was not confirmed in a 2020 meta-analysis [44]. However, many researchers believe that the use of general anesthesia, which ensures sleep, relaxation of the anterior abdominal wall muscles, and patient psychological comfort, can increase the efficacy of conservative treatment [12, 73-78].
3.2 Surgical treatment methods
Surgical treatment of intussusception is determined by the form and etiology of the invagination. Conservative treatment is ineffective in small bowel intussusception. Considering the etiology (polyps, neoplasms), surgical treatment is also indicated for colonic intussusception. The described forms of intussusception in children are extremely rare. Therefore, the diagnostic and treatment algorithm for intussusception specifically addresses ileocolic intussusception.
In ileocolic intussusception, the need for and frequency of surgical treatment are determined by the frequency of lead points triggering the invagination and the rate of complications (perforation, peritonitis), which in most cases are also associated with lead points. According to literature data, the frequency of anatomical causes in ileocolic intussusception ranges from 4 to 14% [2-14]. The rate of bowel resection performed for ileocolic intussusception is 3-8% [7,8-12]. Such a frequency of lead points and bowel resections implies that 100% efficacy of conservative treatment cannot be expected or pursued. Another argument for the necessity of surgical treatment is recurrent intussusception, which is often associated with lead points. The incidence of recurrent intussusception varies from 6 to 16% according to various researchers [33-42]. Several researchers note that the frequency of lead points in recurrent intussusception is higher than in the general population. However, there is no consensus on the number of attempts at conservative reduction in recurrent intussusception. Surgical treatment is recommended if a lead point is suspected in recurrent intussusception [12-14].
It is recommended that all patients with intussusception scheduled for surgical treatment undergo antibiotic prophylaxis with broad-spectrum systemic antibacterial agents to prevent surgical site infections [79-83].
GCR CLEL 5Grade of Recommendation C (Level of Evidence – 5)
Comments: The same drugs are used for antibiotic prophylaxis as for empirical antibacterial therapy. According to a 2021 systematic review, the authors concluded that antibiotic prophylaxis before conservative treatment of ileocolic intussusception is not mandatory, as no difference in the course of the disease was observed, and no bacteremia was detected in children who did not receive systemic antibacterial drugs before conservative reduction [90].
It is recommended that all patients with complicated intussusception (peritonitis, small bowel obstruction) undergo preoperative preparation in an intensive care unit to normalize water and electrolyte disturbances and stabilize the patient's condition [82, 83].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: Preoperative preparation aims to correct pronounced metabolic and water-electrolyte disorders, which are manifestations of endotoxicosis in the development of peritonitis and small bowel obstruction. The duration and scope of preoperative preparation are determined by a concilium of an anesthesiologist-reanimatologist and a pediatric surgeon [2,7,88,89].
It is recommended that all patients with ileocolic intussusception, in cases of ineffective conservative reduction, development of complications (bowel perforation, peritonitis, small bowel obstruction), as well as upon diagnosis or suspicion of a lead point, undergo surgical treatment via laparoscopic or laparotomic approach to perform reduction [2-15, 23-30, 54-78, 84-94].
GCR ALEL 1Grade of Recommendations A (Level of Evidence – 1)
Comments:
Laparoscopy. The first report on the use of laparoscopy for the reduction of intussusception in two children in our country was published in 1980 by E.I. Finkelson et al. In 1996, P.M. Cuckow et al. (England) published a case report of successful laparoscopic reduction after two failed attempts at pneumatic reduction in a 10-month-old boy [85]. To date, the efficacy of laparoscopic reduction has been confirmed by numerous studies [85-95]. Laparoscopy can be the primary surgical treatment method in specialized centers with adequate experience in laparoscopic procedures [93]. According to a 2013 systematic review by N. Apelt et al. including 276 patients, the efficacy of laparoscopic reduction (releasing the bowel telescoped into another (intussusception)) was 71% [93]. Conversion is typically performed when laparoscopic reduction is impossible and manual reduction is required, or when bowel resection is necessary due to necrosis. Endosurgical treatment can also be successful when bowel resection is necessary, provided there is sufficient experience [82]. Laparoscopic reduction (releasing the bowel telescoped into another (intussusception)) is performed using bowel traction with instruments. Laparoscopic surgery has an advantage over laparotomy when comparing the duration of hospital stay (4.0 vs. 7.0 days, p<0.01) [93]. With successful laparoscopic reduction (releasing the bowel telescoped into another (intussusception)), intraluminal lead points such as polyps, Burkitt lymphoma, etc., may go undiagnosed. Following successful laparoscopic reduction (releasing the bowel telescoped into another (intussusception)) and the exclusion of intra-abdominal lead points in recurrent intussusception, a postoperative colonoscopy is recommended for timely diagnosis of intraluminal lead points [12].
Laparotomy is the traditional approach for surgical treatment. Laparotomy is performed when conservative treatment fails in non-specialized centers lacking sufficient experience in endosurgical procedures, as well as in cases where laparoscopic reduction fails and manual reduction is required. Reduction is carried out by "squeezing" the inner cylinder of the intussusceptum (Hutchinson maneuver). In case of necrosis of the disintussuscepted bowel in the absence of peritonitis, its resection with the formation of an enteroenteric anastomosis is recommended. Most frequently, resection of the ileocecal junction or ileum is performed; if reduction is impossible, resection of the intussusceptum within healthy tissue is performed. For ileal resection, an end-to-end anastomosis is recommended, while for resection of the ileocecal junction or intussusceptum, an end-to-side anastomosis [2, 5-14, 23, 27-29, 57]. In bowel necrosis complicated by peritonitis, surgical tactics depend on the child's general condition. In severe, "unstable" conditions, the formation of an anastomosis should be avoided, and an ileostomy should be brought out as the first stage of the surgical intervention. Formation of an anastomosis is possible upon stable clinical stabilization of the patient.
Currently, based on a 2021 systematic review by Lorraine I. Kelley-Quon et al., there is no evidence to determine the superiority of laparoscopic surgery over laparotomy regarding recurrence rates or postoperative complications. However, a shorter hospital stay was noted with laparoscopic treatment [81].
It is recommended that all patients with critical intussusception and hemodynamic instability undergo surgical treatment via laparotomy to prevent intraoperative complications [8, 9, 82, 83].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: In case of hemodynamic instability, which may occur with the development of severe complications (peritonitis, small bowel obstruction), the capnoperitoneum required for laparoscopic intervention can contribute to critical hemodynamic disorders [82, 83]. Uniform and significant dilation of the small bowel loops in small bowel obstruction prevents effective reduction and poses a risk of intestinal wall injury.
It is recommended that all patients with complicated intussusception and peritonitis undergo intraoperative collection of peritoneal exudate for bacteriological examination of the peritoneal fluid for aerobic and facultatively anaerobic opportunistic pathogens and determination of antimicrobial susceptibility [79, 80].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: In the event of a complicated postoperative course, microbiological monitoring is performed (microbiological (culture) blood test for sterility, microbiological (culture) examination of mucus from the tonsils and posterior pharyngeal wall for aerobic and facultatively anaerobic microorganisms, and in cases of repeated surgeries, bacteriological examination of the peritoneal fluid for aerobic and facultatively anaerobic opportunistic pathogens and determination of antimicrobial susceptibility).
It is not recommended to perform concomitant appendectomy during the surgical treatment of intussusception [81, 86, 94-95].
GCR BLEL 3Grade of Recommendation B (Level of Evidence – 3)
Comments: The role of appendectomy in the surgical treatment of intussusception remains controversial. Some surgeons advocate for appendiceal removal because the appendix may serve as a lead point for recurrent bowel intussusception [94, 95]. According to A. Wang et al., the total cost of hospitalization was higher when appendectomy was performed; readmission rates for recurrent intussusception were similar in both groups (appendectomy group: 5.5% vs. non-appendectomy group: 6.7%, p = 0.34) at one-year follow-up, whereas 10 patients (1.3%) in the appendectomy group and only 2 patients (0.35%) in the non-appendectomy group presented with adhesive small bowel obstruction, p = 0.06 [96]. Several authors recommend removing the appendix only in cases of ischemia or inflammation [81, 86, 97]. According to a 2021 systematic review, evidence supporting prophylactic appendectomy during the surgical treatment of intussusception is insufficient; appendectomy may be performed if the vermiform appendix is inflamed or ischemic [81].
3.3 Postoperative management
It is not recommended to prescribe specialized treatment to patients with uncomplicated intussusception after conservative or laparoscopic reduction (dislodgement of the intussuscepted bowel segment) [2–15, 81, 97].
GGR BLEL 3Grade of Recommendation B (Level of Evidence 3)
Comments: Following reduction (dislodgement of the intussuscepted bowel segment), special attention should be paid to the principles of rational feeding and adherence to an age-appropriate diet in children. Analgesics, medications for functional gastrointestinal disorders, and cleansing enemas may be prescribed symptomatically.
It is recommended to admit all patients with complicated intussusception to the intensive care unit for intensive therapy following surgical treatment [2, 5, 7, 89, 90].
Level of Evidence 5Grade of Recommendation C (Level of Evidence 5)
Comments: The development of complications (necrosis, bowel perforation, peritonitis, small bowel obstruction) triggers a cascade of diverse pathological processes that threaten the development of organ dysfunction, sepsis, and multiple organ dysfunction syndrome (MODS). The patient's critical condition, as well as the risk of its development, necessitates intensive care and multi-parameter monitoring in an intensive care unit setting. The mainstays of intensive therapy include: correction of fluid, electrolyte, and metabolic disturbances, analgesia, antibacterial therapy, nutritional support, and restoration of gastrointestinal function. To normalize gastrointestinal function and stimulate reparative processes as part of comprehensive intensive care, several authors recommend using epidural anesthesia and hyperbaric oxygenation.
It is recommended to prescribe infusion therapy to all patients after surgical treatment of complicated intussusception in order to correct metabolic, fluid, and electrolyte disturbances [2, 7, 62, 93].
LeR CLoE 5Level of Recommendation C (Level of Evidence — 5)
Comments: The infusion therapy regimen includes electrolyte solutions, blood substitutes, and perfusion solutions (B05) as indicated.
It is recommended to prescribe parenteral nutrition to all patients after surgical treatment of complicated intussusception to maintain their nutritional status [2, 7, 82, 83].
LeR CLoE 5Level of Recommendation C (Level of Evidence — 5)
Comments: Parenteral nutrition products (B05BA) are generally prescribed from the 1st day of the postoperative period, after relief of shock reactions and stabilization of the patient's hemodynamics, until the transition to enteral nutrition in the volume of physiological requirement.
It is recommended to perform early enteral therapy in all patients after surgical treatment of intussusception complicated by peritonitis and small bowel obstruction, with the aim of restoring gastrointestinal function and preventing bacterial translocation. [82, 83]
LeR CLoE 5Level of Recommendation C (Level of Evidence — 5)
Comments: The main stages of early enteral therapy are decompression of the upper gastrointestinal tract (stomach, initial parts of the jejunum) using a nasogastric or double-lumen nasointestinal tube, and administration of an isotonic saline electrolyte solution into the stomach and/or small intestine with a gradual transition to special nutrient formulas (oligomeric, polymeric) as gastrointestinal function recovers.
It is recommended to prescribe therapy with broad-spectrum antibacterial drugs (ATX – antibacterials for systemic use) for therapeutic purposes in all patients after surgical treatment of complicated intussusception to reduce postoperative infectious complications [79, 80]
LeR CLoE 5Level of Recommendation C (Level of Evidence — 5)
Comments: In the postoperative period, empirical antibiotic therapy is performed until the results of microbiological examination of the exudate are obtained. If postoperative complications develop (intra-abdominal abscesses, ongoing peritonitis, sepsis), antibiotic therapy is adjusted in accordance with the results of microbiological studies (purulent focus, blood, patient's mucous membranes) in the postoperative period.
4. Medical Rehabilitation and Sanatorium-Resort Treatment
It is not recommended to provide medical rehabilitation to patients after successful conservative treatment of uncomplicated intussusception [2-11, 28, 29, 81]
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: Children after successful conservative treatment of idiopathic intussusception do not need medical rehabilitation.
It is recommended that all patients after surgical treatment of complicated intussusception consult a rehabilitation physician (medical rehabilitation services for a patient with a digestive system disease) to determine the medical rehabilitation program [2-10,98]
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: In the postoperative period, therapeutic physical training (therapeutic physical training for diseases of the small and large intestines, therapeutic physical training for respiratory diseases), massage (massage for diseases of the small and large intestines and general medical massage) are indicated for the prevention of postoperative complications. The choice of physical therapy depends on the type and scope of surgical intervention and the course of the postoperative period. To improve reparative processes and prevent postoperative infectious complications, decimeter-wave (DMV) electromagnetic radiation therapy, magnetic field therapy, low-intensity laser therapy (intravenous laser blood irradiation) are used; for the normalization of intestinal motility – gastrointestinal electromyostimulation.
5. Prevention and Dispensary Observation
It is recommended that all children after successful conservative or laparoscopic disintussusception undergo follow-up by a pediatrician for 1 year (dispensary appointment (examination, consultation) of a pediatrician) after discharge, at 1, 3, 6 months, and 1 year [2-10].
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: Considering the etiology of idiopathic intussusception, the main burden of preventing recurrences of idiopathic intussusception falls on the pediatrician. In infants, dietary control and educational work on rational feeding are necessary. Considering the leading role of lymphoid hyperplasia in the etiopathogenesis of idiopathic intussusception in young children, the prevention of viral diseases and hardening of the child come to the fore in this group of children. It is necessary to inform parents about the possibility of disease recurrence and the need for emergency hospitalization in a surgical hospital if a recurrence occurs
It is recommended to exempt all patients after surgical treatment of intussusception from increased physical exertion for a period of 1 to 3 months, depending on the scope of the surgical intervention and the course of the postoperative period [2-10].
Level of Evidence CGrade of Recommendation 5Level of Evidence C (reliability of evidence – 5)
It is recommended that all children with an intestinal stoma after surgical treatment of complicated intussusception undergo medical and social expertise (MSE) and follow-up by a pediatric surgeon (dispensary appointment (examination, consultation) of a pediatric surgeon) [2-10,98]
GDR CLE 5Grade of recommendation C (level of evidence – 5)
Comments: The individual rehabilitation program (IRP) during disability registration shall include a record that the patient needs ostomy pouches (one-piece closed ostomy pouch for intestinal stoma, or multi-component closed ostomy pouch for intestinal stoma, or one-piece open-type ostomy pouch for intestinal stoma, or multi-component open-type ostomy pouch for intestinal stoma) and care products for them.
6. Organization of Medical Care
Indications for hospitalization in a medical organization:
If intestinal intussusception is suspected, emergency hospitalization in a pediatric surgical hospital is indicated [99].
Indications for patient discharge from a medical organization
General condition of the child: satisfactory.
Absence of complaints
No changes upon physical and laboratory examination
Presence of spontaneous stool without pathological impurities
Absence of pathological echo formations according to abdominal ultrasound (ultrasound examination of the small and large intestines)
Recommended hospitalization periods:
Conservative treatment – 1–3 days.
Surgical treatment – 5–10 days.
7. Additional Information
Historical background
The first publications regarding intussusception appeared in the 17th century (Paul Barbette, 1674, Amsterdam; Johann C. Peyer, 1677, Switzerland) [99]. In 1732, the first detailed description of the disease was given by John Hunter (England) in 1793 [101]. The first successful operation for intestinal intussusception in 1784 was performed by the British surgeon A. Nuck on a 50-year-old man, and only in 1871 did J. Hutchinson successfully operate on a two-year-old child [102]. Despite publications on the successful surgical treatment of intussusception, an expectant management strategy prevailed in the 18th and 19th centuries, and mortality from intussusception was about 90% [103]. The idea of using air introduced into the rectum for the mechanical expansion of the intussusceptum was first expressed by John Hunter. The first successful pneumatic disintussusception in a child was performed by Samuel Mitchell (England) in 1836 [104]. In 1876, the Danish pediatrician Harald Hirschsprung published a paper on the positive effect of saline enemas in children with intussusception [105]. In 1913, W. E. Ladd first published an X-ray contrast image of an intussusceptum [106]. Following the publication of a paper by the prominent American pediatric surgeon M. Ravich in 1948, the technique of hydrostatic disintussusception using a barium suspension under fluoroscopic control became the "gold standard" in the treatment of intussusception in children in many countries for many years [104]. In the 1970s, ultrasound replaced X-ray examination for diagnosis and monitoring of intussusception reduction. In our country, the main method of diagnosis and simultaneous conservative treatment was pneumatic disintussusception under fluoroscopic control (pneumatic enema). Modifications of the method were developed in detail by V. M. Portnoy (1962) [58] and L. M. Roshal (1964) [4]. Infants underwent conservative reduction of intussusception via pneumatic enema, while children older than one year and those with a disease duration of more than 12 hours underwent laparotomy [3-6]. Later, laparoscopic disintussusception replaced laparotomy in most cases of surgical treatment [85-95].
Criteria for Evaluating the Quality of Medical Care
№ | Quality criteria | Evaluation of compliance | |
|---|---|---|---|
1. | Examination by a pediatric surgeon was performed | Yes/No | |
2. | Diagnostic abdominal ultrasound was performed ultrasound examination of the small, large, sigmoid, and rectum, ultrasound detection of free fluid in the abdominal cavity) if equipment and a specialist are available | Yes/no | |
3. | Laboratory examination upon admission was performed (complete blood count, urinalysis) | Yes/No | |
4. | Acid-base balance and blood gas analysis were performed in the clinical and laboratory presentation of disease complications (intestinal perforation, peritonitis, small bowel obstruction). | Yes/No | |
5. | Preoperative preparation was performed in the clinical and laboratory presentation of disease complications (intestinal perforation, peritonitis, small bowel obstruction). | Yes/No | |
6. | After surgical treatment of a disease with a complicated course (intestinal necrosis, intestinal perforation, peritonitis, small bowel obstruction), broad-spectrum antibacterial drugs (ATX – antibacterials for systemic use) were prescribed | Yes/No | |
7. | Surgical treatment was performed in case of ineffectiveness of conservative disintussusception and upon diagnosis of complications (intestinal necrosis, intestinal perforation, peritonitis, small bowel obstruction) | Yes/No | |
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Appendix A1. Working Group Composition
Mikhail Aleksandrovich Akselrov, Associate Professor, MD, PhD, Head of the Department of Pediatric Surgery, Tyumen State Medical University of the Russian Ministry of Health, Member of the Russian Association of Pediatric Surgeons, Tyumen, Russia. No conflict of interest.
Bairov Vladimir Gireevich, MD, Professor, Professor of the Department of Faculty Surgery with Clinic, Almazov National Medical Research Centre of the Russian Ministry of Health, Chief Freelance Pediatric Surgeon of the Northwestern Federal District, member of the Russian Association of Pediatric Surgeons, Saint Petersburg, Russia. No conflict of interest.
Barova Natalya Kaplanovna, Associate Professor, PhD in Medicine, Head of the Department of Pediatric Surgical Diseases, Kuban State Medical University of the Russian Ministry of Health, Honored Healthcare Worker of Kuban, Honored Healthcare Worker of Adygea, Head of Surgical Department No. 1, Children's Regional Clinical Hospital of the Ministry of Health of Krasnodar Krai, Chief Freelance Pediatric Surgeon of the Ministry of Health of Krasnodar Krai, member of the Russian Association of Pediatric Surgeons, Krasnodar, Russia. No conflict of interest.
Barskaya Margarita Aleksandrovna, MD, Professor, Professor of the Department of Surgical Diseases of Children and Adults, Samara State Medical University of the Russian Ministry of Health, Chief Freelance Pediatric Surgeon of the Ministry of Health of Samara Oblast, member of the Russian Association of Pediatric Surgeons, Samara, Russia. No conflict of interest.
Golikov Denis Evgenievich, Researcher at the Department of Polytrauma, Research Institute of Emergency Pediatric Surgery and Traumatology of the Moscow Healthcare Department, member of the Russian Association of Pediatric Surgeons, Moscow, Russia. No conflict of interest.
Gorelik Aleksandr Lvovich, Researcher at the Department of Polytrauma, Research Institute of Emergency Pediatric Surgery and Traumatology of the Moscow Healthcare Department, member of the Russian Association of Pediatric Surgeons, Moscow, Russia. No conflict of interest.
Zhurilo Ivan Petrovich – MD, Professor, Professor of the Department of Pediatric Surgical Disciplines and Innovative Technologies in Pediatrics, Orel State University named after I.S. Turgenev, member of the Russian Association of Pediatric Surgeons, Orel, Russia. No conflict of interest.
Karaseva Olga Vitalievna, MD, Deputy Director for Research, Head of the Department of Polytrauma, Anesthesiology and Intensive Care, Research Institute of Emergency Pediatric Surgery and Traumatology of the Moscow Healthcare Department, Chief Freelance Pediatric Specialist in Polytrauma of the Moscow Healthcare Department, member of the Russian Association of Pediatric Surgeons, Moscow, Russia. No conflict of interest.
Nalivkin Aleksandr Evgenievich, MD, Professor, Head of the Pediatric Surgery Course at the Department of Surgery, Moscow Regional Research Clinical Institute named after M.F. Vladimirsky, Moscow, Russia. No conflict of interest.
Poddubny Igor Vitalevich, MD, Professor, Head of the Department of Pediatric Surgery, A.I. Evdokimov Moscow State University of Medicine and Dentistry of the Russian Ministry of Health, member of the Russian Association of Pediatric Surgeons, Moscow, Russia. No conflict of interest.
Svarich Vyacheslav Gavrilovich, MD, Associate Professor, Professor of the Department of Surgery, Medical Institute of Pitirim Sorokin Syktyvkar State University, Deputy Chief Medical Officer for Surgery at the Republican Children's Clinical Hospital, Chief Pediatric Surgeon of the Ministry of Health of the Republic of Karelia, Honored Doctor of the Russian Federation, President of the Komi Republican Association of Doctors, member of the Russian Association of Pediatric Surgeons. No conflict of interest.
Sokolov Yury Yurievich, MD, Professor, Head of the Department of Pediatric Surgery named after Academician S.Ya. Doletzky, Russian Medical Academy of Continuous Professional Education of the Russian Ministry of Health, member of the Russian Association of Pediatric Surgeons. No conflict of interest.
Timofeeva Anna Vyacheslavovna, Researcher at the Department of Polytrauma, Research Institute of Emergency Pediatric Surgery and Traumatology of the Moscow Healthcare Department, member of the Russian Association of Pediatric Surgeons. No conflict of interest.
Kharitonova Anastasia Yurievna, PhD in Medicine, Head of the Endoscopy Department, Research Institute of Emergency Pediatric Surgery and Traumatology of the Moscow Healthcare Department, member of the Russian Association of Pediatric Surgeons, member of the Russian Society for Gastrointestinal Endoscopy. No conflict of interest.
Tsap Natalia Aleksandrovna – MD, Professor, Head of the Department of Pediatric Surgery, Ural State Medical University of the Russian Ministry of Health, Chief Freelance Pediatric Surgeon of the Ministry of Health of Sverdlovsk Oblast; Chief Pediatric Surgeon Specialist for the Ural Federal District, member of the Russian Association of Pediatric Surgeons. No conflict of interest.
Appendix A2. Methodology for Developing Clinical Guidelines
Target audience for these clinical guidelines:
Inpatient and outpatient pediatric surgeons
Pediatricians
Radiologists
Endoscopists
Anesthesiologists-intensivists
Students, residents, PhD students, continuing professional education trainees
Table 1. Scale for assessing levels of reliable evidence (LRE) for diagnostic methods (diagnostic interventions)
LoE | Definition |
|---|---|
1 | Systematic reviews of studies with reference standard control or systematic reviews of randomized controlled trials with meta-analysis |
2 | Individual studies with reference standard control or individual randomized controlled trials and systematic reviews of studies of any design, except randomized controlled trials, with meta-analysis |
3 | Studies without consecutive reference standard control or studies with a reference standard not independent of the test method, or non-randomized comparative studies, including cohort studies |
4 | Non-comparative studies, case report |
5 | There is only a rationale for the mechanism of action or expert opinion |
Table 2.Levels of Evidence (LoE) grading scale for prevention, treatment and rehabilitation methods (preventive, therapeutic, and rehabilitative interventions)
LoE | Definition |
|---|---|
1 | Systematic review of RCTs with meta-analysis |
2 | Individual RCTs and systematic reviews of studies of any design except RCTs, with meta-analysis |
3 | Non-randomized comparative studies, including cohort studies |
4 | Non-comparative studies, case reports or case series, case-control studies |
5 | There is only a rationale for the mechanism of action of the intervention (preclinical studies) or expert opinion |
Table 3. Scale for grading grades of recommendation (GR) for prevention, diagnostic, treatment, and rehabilitation methods (preventive, diagnostic, therapeutic, and rehabilitative interventions)
GoR | Definition |
|---|---|
A | Strong recommendation (all considered efficacy criteria (outcomes) are important, all studies are of high or acceptable methodological quality, and their conclusions on the outcomes of interest are consistent) |
B | Conditional recommendation (not all considered efficacy criteria (outcomes) are important, not all studies are of high or acceptable methodological quality, and/or their conclusions on the outcomes of interest are inconsistent) |
C | Weak recommendation (lack of evidence of proper quality; all considered efficacy criteria [outcomes] are unimportant, all studies have low methodological quality, and their conclusions regarding outcomes of interest are inconsistent) |
Procedure for updating clinical guidelines.
The mechanism for updating clinical guidelines provides for their systematic revision at least once every three years, as well as upon the emergence of new evidence-based medicine data on the diagnosis, treatment, prevention, and rehabilitation of specific diseases, or in the presence of justified additions/comments to previously approved clinical guidelines, but no more frequently than once every 6 months.
Appendix A3. Reference Materials
These clinical guidelines were developed taking into account the following regulatory legal documents:
Order of the Ministry of Health of the Russian Federation No. 562n dated October 31, 2012, "On Approval of the Procedure for Providing Medical Care in the Profile 'Pediatric Surgery'"
Order of the Ministry of Health of the Russian Federation No. 203n dated May 10, 2017, "On Approval of Criteria for Assessing the Quality of Medical Care".
Appendix B. Physician Action Algorithms

Appendix C. Patient Information
Intussusception is the invagination of one segment of the intestine into the lumen of another.
The condition most commonly occurs in infants and young children (under 3 years of age). In most cases, the exact causes of the disease are unknown. The main risk factor for intussusception in infants is the introduction of complementary foods. It most frequently affects infants aged 4–9 months. Intussusception may also occur in children following rotavirus vaccination. Precipitating factors in young children include previous viral infections, as well as the acute course of intestinal infections. In rarer cases, intestinal intussusception is associated with underlying anatomical causes (polyps, tumors, gastrointestinal malformations). In any case, the telescoping of one intestinal segment into another occurs against the background of impaired, typically increased, bowel motility.
In most cases, the disease begins suddenly against a background of complete health. The child becomes restless, cries, draws up their legs, and refuses to eat. The bout of restlessness ends as suddenly as it begins, but recurs after a short interval; vomiting joins, and the child's general condition worsens. Body temperature is most often normal, but may be elevated. In the first hours of the disease, stools may be normal; after some time, instead of fecal matter, 'raspberry jelly' stools pass from the rectum.
Due to the strangulation of the intestine during intussusception, necrosis may occur, leading to the development of a severe inflammatory process in the abdominal cavity. Intussusception requires the most rapid elimination possible, for which the child must be hospitalized in a surgical hospital.
If you suspect intestinal intussusception in your child, you must seek medical attention immediately.
The main method of treating intestinal intussusception is conservative treatment, which is possible if the child's condition is satisfactory. Conservative treatment consists of the mechanical reduction of the intussusceptum using fluid or air introduced into the intestine via the anus. Air or liquid enemas are performed under fluoroscopic or ultrasound guidance, which allows monitoring of the movement of air or fluid through the intestine and the reduction of the invaginated bowel. Conservative reduction of the intussusceptum is the most favorable outcome of the disease. In this case, the child requires hospital observation for 2–3 days to rule out potential recurrence of the disease, after which they can be discharged home.
In cases of severe condition of the child upon late hospitalization, or if conservative treatment is ineffective, the child undergoes surgical treatment to eliminate the intussusception—laparoscopy or laparotomy. In the most severe cases with necrosis of the strangulated intestine, resection with the formation of an enteroenteric anastomosis is performed; in extremely severe conditions, an intestinal stoma is formed. Following surgery, the child may spend several days in the intensive care unit before being transferred to the surgical ward.
After discharge, the child requires follow-up by a pediatrician to ensure proper feeding, hardening (conditioning), prevention of viral and intestinal infections, including vaccine prophylaxis.
Parents must remember the possibility of disease recurrence at any age and seek medical attention immediately should it occur.
Appendix G. Assessment Scales, Questionnaires, and Other Evaluation Tools
Not applicable.