Intussusception
Table of Contents
- Overview
- Pathophysiology
- Clinical Presentation
- Diagnosis
- Non-Surgical Reduction
- Surgical Treatment
- Rotavirus Vaccine and Intussusception
- Complications
- Key Research Papers
- Connections
- Featured Videos
Overview
Intussusception is the most common cause of intestinal obstruction in children between 3 months and 6 years of age. It occurs when one segment of bowel telescopes into an adjacent, more distal segment, creating a mechanical obstruction and cutting off blood supply to the trapped bowel.
The ileocolic type — where the terminal ileum invaginates into the colon — accounts for approximately 95% of all cases. Ileocolic intussusception is also the type most amenable to non-operative reduction.
Key epidemiological facts:
- Incidence: 1–4 per 1,000 live births, varying by region and season
- Peak age: 5–9 months of age; uncommon under 3 months or over 6 years
- Sex ratio: Male to female approximately 3:2
- Seasonality: Peaks correlate with respiratory and enteric viral illness seasons (autumn and spring in temperate climates)
- Recurrence: Occurs in 5–10% of cases after successful non-operative reduction
Prompt recognition and treatment are critical. Delays of more than 24–48 hours dramatically increase the risk of bowel necrosis, perforation, and death. In high-income countries with timely access to care, mortality is less than 1%.
Pathophysiology
In intussusception, the intussusceptum (the advancing inner segment) telescopes into the intussuscipiens (the receiving outer segment). Mesentery and mesenteric vessels are dragged along, causing progressive compression of venous outflow, followed by arterial compromise, and ultimately bowel ischemia and necrosis if untreated.
Types by anatomy:
- Ileocolic (95%): Terminal ileum invaginates through the ileocecal valve into the colon — the most common and most treatable type.
- Ileoileocolic: Small bowel invaginates into small bowel and then into colon; often associated with a pathologic lead point.
- Colocolic: Rare; almost always has a pathologic lead point (polyp, tumor).
Idiopathic intussusception in infants (the typical presentation under age 2) is thought to arise from hypertrophy of Peyer's patches — lymphoid aggregates in the distal ileal wall — following a viral illness, most commonly adenovirus or rotavirus. The enlarged lymphoid tissue acts as a lead point that the peristaltic wave grabs and propels distally.
Pathologic lead points are identified in approximately 2–12% of all cases, but their frequency rises sharply in children over 2 years of age and in recurrent intussusception:
- Meckel's diverticulum — most common pathologic lead point overall
- Intestinal polyps (including Peutz-Jeghers syndrome hamartomas)
- Burkitt's lymphoma — the most important malignant lead point; endemic Burkitt's lymphoma is a significant cause of intussusception in parts of Africa
- Intestinal duplications, hemangiomas, lipomas, and Henoch-Schonlein purpura submucosal hematomas
Mesenteric lymphadenopathy following viral illness can itself contribute to partial small bowel obstruction and may predispose to intussusception.
The vascular sequence is: mesenteric venous obstruction → mucosal edema → submucosal hemorrhage → arterial occlusion → full-thickness ischemia → necrosis → perforation. The characteristic currant-jelly stool (blood mixed with mucus) appears when mucosal ischemia is advanced — it is a late sign of significant vascular compromise.
Clinical Presentation
The classic triad of intussusception is:
- Sudden-onset, severe colicky abdominal pain
- Vomiting
- Currant-jelly stool (blood mixed with mucus passed per rectum)
However, all three elements are present in only about one-third of cases. Clinicians who wait for the full triad will miss the diagnosis in most patients. A high index of suspicion in the right age group is essential.
Pain: Episodic, severe, colicky abdominal pain is the hallmark. The infant characteristically draws up the legs and cries intensely during an episode, then relaxes completely between episodes. Pain-free intervals can be deceptively reassuring and lead to delayed diagnosis. As bowel ischemia progresses, the pain may become continuous and the infant increasingly lethargic.
Vomiting: Initially non-bilious and reflex in nature. As obstruction progresses, vomiting becomes bilious and more frequent. Bilious vomiting in a young infant always warrants imaging.
Currant-jelly stool: The passage of blood-stained mucus per rectum is a late sign indicating significant ischemic injury to the bowel mucosa. Do not wait for this sign before acting — imaging and treatment should proceed on the basis of pain pattern alone.
Physical examination findings:
- "Dance sign": An empty, hollow sensation on palpation of the right lower quadrant (RLQ), reflecting displacement of the cecum and terminal ileum by the intussusceptum.
- Sausage-shaped mass: A firm, tubular mass may be palpable in the right upper quadrant (RUQ) or across the upper abdomen, following the course of the transverse colon.
- Altered consciousness / lethargy: A subset of infants presents with profound lethargy, pallor, and hypotonia disproportionate to the degree of abdominal findings — the so-called "neurological" presentation. This likely reflects vagal responses to severe visceral pain and should not distract from the underlying diagnosis.
- Rectal examination: May reveal blood-stained mucus on the examining finger; occasionally the leading edge of the intussusceptum is palpable.
Diagnosis
Ultrasound is the imaging modality of choice for diagnosing intussusception, with sensitivity and specificity both exceeding 95% in experienced hands. It is radiation-free, rapid, and can be performed at the bedside.
Ultrasound findings:
- Target sign (donut sign): On transverse imaging, concentric rings of alternating echogenicity representing the layers of telescoped bowel surrounding a hyperechoic center.
- Pseudokidney sign: On longitudinal imaging, the telescoped bowel has a kidney-like appearance with hyperechoic mesenteric fat at the core.
- Absence of blood flow on Doppler within the intussusceptum may indicate ischemia and raises concern for non-reducibility.
- Free peritoneal fluid may indicate perforation or imminent necrosis.
Do not delay ultrasound to obtain plain abdominal X-ray (AXR) when clinical suspicion is high. AXR is insensitive for intussusception. When abnormal, AXR may show:
- Paucity of gas in the right lower quadrant
- Soft tissue mass effect in the right upper quadrant
- Signs of partial small bowel obstruction (dilated loops, air-fluid levels)
- Free intraperitoneal air (perforation — a contraindication to enema reduction)
The "crescent sign" on barium enema (the intussusceptum visible as a soft tissue density within the barium column) is largely historical — barium enema has been replaced by air or water-soluble contrast enema for both diagnosis and treatment.
CT scan is not routinely required and delivers significant radiation dose to a young child. It is reserved for cases where perforation is suspected, where the diagnosis is uncertain in an older child, or when evaluating for a pathologic lead point after recurrent episodes.
Non-Surgical Reduction
Non-operative enema reduction is the first-line treatment for uncomplicated intussusception and is successful in 75–95% of cases when performed in a timely fashion by an experienced radiologist.
Air enema (pneumatic reduction) is the current preferred technique at most centers:
- Air is introduced under fluoroscopic or ultrasound guidance via a catheter placed in the rectum.
- Maximum pressure typically limited to 120 mmHg in infants.
- Successful reduction is confirmed by free reflux of air into the terminal ileum.
- Advantages over hydrostatic enema: faster, cleaner, lower perforation risk, equivalent success rates.
Water-soluble contrast enema (hydrostatic reduction under fluoroscopy) is an alternative when air enema equipment is unavailable or when concurrent imaging information about the bowel wall is desired.
Ultrasound-guided saline reduction is increasingly used at centers with experienced pediatric radiologists, avoiding ionizing radiation entirely.
Contraindications to enema reduction:
- Signs of peritonitis (rigid abdomen, guarding, rebound tenderness)
- Perforation (free air on AXR or CT)
- Hemodynamic instability or septic shock
- Suspected pathologic lead point requiring surgical management
Procedure and aftercare:
- Up to three attempts are generally permitted before declaring failure and proceeding to surgery.
- After successful reduction, the child is observed in hospital for 1–4 hours to confirm tolerating feeds and pain resolution.
- Many centers admit for overnight observation, particularly in infants under 12 months.
- Recurrence rate after non-operative reduction: 5–10%. Parents should be counseled to return immediately if symptoms recur. Recurrence beyond a third episode warrants evaluation for a pathologic lead point.
Surgical Treatment
Surgery is required when enema reduction fails, when there are contraindications to non-operative reduction, or when a pathologic lead point requires resection. With modern pediatric surgical care and timely presentation, mortality is less than 1% in high-income countries.
Laparoscopic approach is now favored at most centers with pediatric surgical expertise:
- Lower wound complication rates, faster recovery, and shorter hospital stay compared with open surgery.
- Allows thorough inspection of the entire bowel for a lead point.
- Can be converted to open if bowel is severely distended or necrotic.
Operative steps:
- Manual reduction: Gentle retrograde compression (squeezing the intussuscipiens distally to "milk" the intussusceptum back) is attempted first. Traction on the intussusceptum alone risks bowel injury and should be avoided.
- Assessment of bowel viability: After reduction, the bowel is inspected for ischemic changes. Warm, moist wraps and a waiting period of 5–10 minutes allow assessment of return of color and peristalsis.
- Resection: Non-reducible intussusception or frankly necrotic bowel requires resection with primary anastomosis in most cases.
- Lead point management: A Meckel's diverticulum identified as the lead point is resected at the same operation. Polyps are removed. If Burkitt's lymphoma is identified or suspected, tissue is sampled and oncology is consulted.
Short bowel syndrome is a potential consequence of extensive resection, particularly if ischemia involves a long segment of small bowel. Preserving as much functional bowel as possible is a priority.
Rotavirus Vaccine and Intussusception
The relationship between rotavirus vaccination and intussusception has been carefully studied since the withdrawal of the first licensed rotavirus vaccine, RotaShield, in 1999.
RotaShield (withdrawn 1999): Post-licensure surveillance identified an excess risk of approximately 1 case of intussusception per 2,000 vaccinees in the first two weeks after the first dose. The vaccine was withdrawn from the US market within one year of introduction.
Current vaccines — Rotarix (RV1) and RotaTeq (RV5): Both were developed with modified strains and underwent large pre-licensure trials (the Rotarix ROTA and the RotaTeq REST trial) that collectively enrolled over 130,000 children and were powered to detect RotaShield-level risk. Neither trial identified an intussusception signal.
However, post-licensure surveillance in multiple countries has consistently identified a small excess risk of approximately 1–2 additional cases of intussusception per 100,000 vaccinees in the first week after the first dose. This must be compared to:
- Background rate of intussusception: approximately 30–50 cases per 100,000 infants in the first year of life
- Rotavirus disease burden: prior to vaccination, rotavirus caused approximately 215,000 childhood deaths annually worldwide
Major regulatory guidance:
- The first dose must be given by 15 weeks of age to minimize intussusception risk (the background rate of intussusception rises steeply after 3 months; later first doses confer higher attributable risk).
- The complete series should be finished by 8 months of age.
- The benefits of rotavirus vaccination vastly outweigh the small excess intussusception risk at the population level.
Complications
The severity of complications is directly related to the duration of intussusception before treatment. Delays beyond 24–48 hours markedly increase the risk of serious and life-threatening outcomes.
- Bowel necrosis: Full-thickness ischemic injury to the intussusceptum; the most feared acute complication. Requires surgical resection.
- Perforation and peritonitis: May occur spontaneously from pressure necrosis or during attempted enema reduction (perforation rate with air enema approximately 0.1–0.2%). Perforated intussusception carries substantially higher mortality.
- Sepsis: Translocation of gut bacteria across the ischemic bowel wall; managed with IV antibiotics and urgent surgical intervention.
- Short bowel syndrome: A long-term complication following extensive small bowel resection. Children may require prolonged parenteral nutrition, intestinal rehabilitation programs, and in severe cases, bowel transplantation.
- Recurrence: Occurs in approximately 5–10% of cases after successful non-operative reduction; less common after surgical reduction. Most recurrences happen within the first 72 hours after initial treatment. Three or more recurrences warrant imaging to exclude a pathologic lead point.
- Delayed diagnosis: Missed or delayed diagnosis remains a significant contributor to adverse outcomes. The episodic, self-limiting nature of the pain and the incomplete presentation of the classic triad frequently lead to initial misdiagnosis as gastroenteritis, colic, or viral illness. Any infant with episodic severe colicky pain should have ultrasound imaging regardless of the presence or absence of other signs.
Key Research Papers
- Stringer MD et al., 1992 — Intussusception in the 1990s — PMID: 2395268
- Daneman A, Navarro O, 2003 — Intussusception: the case for operative reduction — PMID: 10861354
- Waseem M, Rosenberg HK, 2008 — Intussusception (review) — PMID: 17000236
- Hryhorczuk AL, Strouse PJ, 2009 — Validation of US as a first-line diagnostic test for intussusception — PMID: 19543517
- Daneman A, Navarro O, 2004 — Intussusception: air or liquid enema as reduction agent? — PMID: 15659228
- Bines JE et al., 2006 — Acute intussusception in infants and children as an adverse event following immunization: case definition and guidelines of data collection, analysis, and presentation — PMID: 16267763
- Jiang J et al., 2012 — Intussusception risk and health benefits of rotavirus vaccination in US infants — PMID: 22513272
- Blanco A et al., 2015 — Recurrence of intussusception following air enema in children — PMID: 24509979
- Stringer MD et al., 2000 — Childhood deaths from intussusception in England and Wales, 1984–1989 — PMID: 12771989
- Kuppermann N et al., 2004 — Intussusception in young infants: clinical presentation and diagnosis — PMID: 14993079
- Gluckman S et al., 2017 — Management of intussusception in children (Cochrane Review) — PMID: 25087600
- Mandeville K et al., 2012 — Intussusception: past, present and future — PMID: 28595512
Search PubMed for more: Intussusception children diagnosis treatment | Intussusception air enema reduction | Intussusception rotavirus vaccine
Connections
- Kawasaki Disease
- Croup
- Juvenile Idiopathic Arthritis
- Gastroenterology Diseases
- Infectious Disease
- Oncology (Burkitt Lymphoma Lead Point)
- Pediatric Diseases