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Intussusception

Quick Facts

  • Most Common Age: 6 months to 3 years
  • Emergency: Yes - requires immediate medical attention
  • Mortality Risk: High if untreated
  • Primary Treatment: Air enema or surgery
  • Recurrence Rate: 5-10% after successful reduction

🚨 MEDICAL EMERGENCY - Seek Immediate Care For:

  • Sudden, severe abdominal pain in a child
  • Bloody or "currant jelly" stools
  • Lethargy or decreased responsiveness
  • Persistent vomiting with abdominal pain
  • Abdominal mass or distension

Overview

Intussusception is a serious medical emergency that occurs when one segment of the intestine telescopes or invaginates into an adjacent segment, similar to how a telescope collapses. This telescoping action creates an intestinal obstruction that can cut off blood supply to the affected portion of the bowel, potentially leading to tissue death (necrosis), perforation, and life-threatening complications if not treated promptly.

While intussusception can occur at any age, it is the most common cause of intestinal obstruction in infants and young children, with the peak incidence occurring between 6 months and 3 years of age. The condition affects approximately 1-4 per 1,000 live births, with males being affected more frequently than females at a ratio of approximately 3:2. In pediatric cases, about 90% are idiopathic (without an identifiable cause), while in adults, an underlying pathological lead point is identified in up to 90% of cases.

The most common location for intussusception is at the junction between the small intestine (ileum) and large intestine (colon), known as ileocolic intussusception. This accounts for approximately 80-90% of pediatric cases. Other less common types include ileoileal (small bowel into small bowel), colocolic (colon into colon), and rarely, retrograde intussusception where the bowel telescopes in the reverse direction.

Signs and Symptoms

The presentation of intussusception can vary significantly depending on the age of the patient, duration of symptoms, and presence of complications. The classic triad of symptoms - intermittent abdominal pain, vomiting, and bloody stools - is present in only about 20-40% of cases, making clinical diagnosis challenging.

Classic Symptoms in Children

  • Intermittent Colicky Abdominal Pain: Sudden onset of severe, cramping pain that occurs in waves every 15-20 minutes, during which the child may draw up their knees, cry inconsolably, and appear pale or sweaty
  • Blood in Stool: The characteristic "currant jelly" stool (mixture of blood and mucus) typically appears 6-12 hours after symptom onset, though any rectal bleeding should raise suspicion
  • Sharp Abdominal Pain: Between episodes, children may appear relatively comfortable, but the pain progressively worsens and episodes become more frequent
  • Vomiting: Initially non-bilious (not containing bile), but becomes bilious as obstruction progresses
  • Emotional Symptoms: Extreme irritability, lethargy, or altered mental status as the condition progresses

Additional Signs and Symptoms

  • Palpable Abdominal Mass: A sausage-shaped mass may be felt in the right upper quadrant or epigastrium in 60-80% of cases
  • Dance's Sign: Emptiness in the right lower quadrant where the cecum should normally be located
  • Abdominal Distension: Progressive swelling of the abdomen as obstruction continues
  • Dehydration: Due to vomiting and third-spacing of fluids
  • Fever: May indicate bowel necrosis or perforation
  • Shock: In advanced cases with significant fluid loss or sepsis

Symptoms in Adults

Adult intussusception presents differently, often with more chronic and non-specific symptoms:

  • Chronic intermittent abdominal pain (weeks to months)
  • Nausea and vomiting
  • Changes in bowel habits
  • Weight loss
  • Gastrointestinal bleeding (less common than in children)

Causes and Risk Factors

The causes of intussusception differ significantly between pediatric and adult populations, with distinct risk factors and underlying mechanisms.

Pediatric Causes

In children, 90% of cases are idiopathic, meaning no specific anatomical lead point is identified. However, several factors may contribute:

  • Lymphoid Hyperplasia: Enlarged Peyer's patches in the terminal ileum, often following viral infections, may serve as a lead point
  • Viral Infections: Adenovirus, rotavirus, and enterovirus infections are associated with increased risk
  • Meckel's Diverticulum: The most common pathological lead point in children (2-8% of cases)
  • Intestinal Polyps: Particularly in children with familial polyposis syndromes
  • Henoch-Schönlein Purpura: Can cause intramural hematomas that act as lead points
  • Cystic Fibrosis: Thick intestinal secretions may contribute to intussusception
  • Recent Rotavirus Vaccination: Slightly increased risk in the week following vaccination

Adult Causes

In adults, 90% of cases have an identifiable pathological lead point:

  • Benign Tumors: Lipomas, leiomyomas, neurofibromas, and adenomatous polyps
  • Malignant Tumors: Primary adenocarcinoma, lymphoma, and metastatic lesions
  • Inflammatory Conditions: Crohn's disease, ulcerative colitis, and celiac disease
  • Postoperative Adhesions: Can create fixed points leading to intussusception
  • Anatomical Variations: Malrotation or mobile cecum

Risk Factors

  • Age: Highest risk between 6 months and 3 years
  • Sex: Males have higher incidence
  • Season: Increased incidence during peak respiratory and gastrointestinal infection seasons
  • Previous Intussusception: 5-10% recurrence rate
  • Family History: Slightly increased risk with affected siblings
  • Intestinal Malrotation: Anatomical predisposition

Pathophysiology

Understanding the pathophysiology of intussusception is crucial for recognizing the urgency of treatment and potential complications.

Mechanism of Telescoping

Intussusception begins when a proximal segment of bowel (intussusceptum) invaginates into the distal segment (intussuscipiens). This process is thought to result from altered bowel motility, where normal peristaltic waves push the proximal bowel into the distal lumen. A lead point, when present, gets caught by peristalsis and pulled distally, dragging the attached bowel wall with it.

Vascular Compromise

As the intussusceptum enters the intussuscipiens, it brings along its mesentery. The compression of the mesentery between the bowel walls leads to:

  • Venous congestion and edema
  • Arterial compromise
  • Ischemia and potential necrosis
  • Increased risk of perforation

Intestinal Obstruction

The telescoped bowel creates a mechanical obstruction leading to:

  • Proximal bowel distension
  • Increased intraluminal pressure
  • Fluid and electrolyte losses
  • Bacterial overgrowth

Diagnosis

Prompt diagnosis of intussusception is critical to prevent complications. The diagnostic approach combines clinical assessment with imaging studies.

Clinical Evaluation

  • History: Focus on the pattern of pain, presence of bloody stools, and associated symptoms
  • Physical Examination: Abdominal palpation for mass, assessment of dehydration, and rectal examination for blood
  • Vital Signs: May show tachycardia, hypotension in advanced cases

Laboratory Studies

While no specific laboratory test diagnoses intussusception, the following may be helpful:

  • Complete Blood Count: May show leukocytosis or hemoconcentration
  • Electrolytes: To assess dehydration and electrolyte imbalances
  • Blood Gas Analysis: May show metabolic acidosis in severe cases
  • Stool Analysis: Presence of blood and mucus

Imaging Studies

Ultrasound (First-line in Children)

  • Sensitivity: 97-100%, Specificity: 88-100%
  • Classic findings: "Target sign" or "doughnut sign" on transverse view
  • "Pseudokidney sign" on longitudinal view
  • Can assess bowel wall perfusion with Doppler
  • No radiation exposure

Contrast Enema

  • Diagnostic and potentially therapeutic
  • Shows "coiled spring" appearance or filling defect
  • Air enema preferred over barium in many centers
  • Contraindicated if perforation is suspected

CT Scan (Preferred in Adults)

  • High sensitivity and specificity
  • Shows "target" or "sausage-shaped" mass
  • Can identify lead points and complications
  • Helps with surgical planning

Plain Radiography

  • Often normal early in the course
  • May show signs of obstruction or free air if perforated
  • Crescent sign: crescent of gas in the intussusceptum
  • Limited diagnostic value compared to other modalities

Treatment Options

Treatment of intussusception requires urgent intervention to prevent bowel necrosis and perforation. The approach depends on the patient's stability, duration of symptoms, and presence of complications.

Initial Stabilization

  • Intravenous fluid resuscitation
  • Correction of electrolyte imbalances
  • Nasogastric decompression
  • Broad-spectrum antibiotics if perforation suspected
  • Pain management

Non-Surgical Reduction

Air Enema Reduction

  • First-line treatment for uncomplicated pediatric intussusception
  • Success rate: 80-95% when performed within 24 hours
  • Performed under fluoroscopic or ultrasound guidance
  • Maximum pressure limits: 80-120 mmHg
  • Rule of threes: 3 attempts, 3 minutes each

Hydrostatic Reduction

  • Using saline or water-soluble contrast
  • Similar success rates to air enema
  • Bag height typically 3 feet above patient

Contraindications to Non-Surgical Reduction

  • Signs of peritonitis or perforation
  • Hemodynamic instability
  • Failure of previous reduction attempts
  • Symptoms longer than 48-72 hours

Surgical Treatment

Indications for Surgery

  • Failed non-surgical reduction
  • Signs of perforation or peritonitis
  • Pathological lead point identified
  • Multiple recurrences
  • Most adult cases

Surgical Approaches

  • Manual Reduction: Gentle milking of the intussusceptum out of the intussuscipiens
  • Resection: Required if bowel is necrotic, perforated, or has a pathological lead point
  • Laparoscopic: Increasingly used for diagnosis and treatment
  • Open Laparotomy: Traditional approach, especially for complicated cases

Post-Treatment Management

  • Observation for 24-48 hours after successful reduction
  • Clear liquid diet initially, advancing as tolerated
  • Monitor for signs of recurrence
  • Follow-up imaging if pathological lead point suspected

Complications

Complications of intussusception can be life-threatening and increase with delayed diagnosis and treatment.

Acute Complications

  • Intestinal Ischemia and Necrosis: Occurs within 24-48 hours if untreated
  • Perforation: Risk increases significantly after 24 hours
  • Peritonitis: From perforation or transmural necrosis
  • Sepsis: Due to bacterial translocation
  • Hypovolemic Shock: From fluid losses and third-spacing
  • Electrolyte Imbalances: Particularly hyponatremia and hypokalemia

Complications of Treatment

  • Perforation During Reduction: 0.1-3% risk with enema reduction
  • Tension Pneumoperitoneum: If perforation occurs during air enema
  • Incomplete Reduction: May require surgical intervention
  • Venous Thrombosis: Of mesenteric vessels

Late Complications

  • Adhesive Intestinal Obstruction: Following surgical treatment
  • Short Bowel Syndrome: If extensive resection required
  • Recurrence: 5-10% after successful reduction
  • Malabsorption: If significant bowel resection performed

Prognosis and Outcomes

The prognosis for intussusception is excellent when diagnosed and treated promptly, but delays significantly worsen outcomes.

Factors Affecting Prognosis

  • Time to Treatment: Best outcomes when treated within 24 hours
  • Age: Better prognosis in typical age group (6 months-3 years)
  • Presence of Lead Point: May indicate underlying pathology requiring treatment
  • Location: Ileocolic intussusception has better outcomes than small bowel
  • Complications: Presence of necrosis or perforation worsens prognosis

Success Rates

  • Non-Surgical Reduction: 80-95% success rate when performed early
  • Surgical Reduction: Nearly 100% success for viable bowel
  • Mortality: Less than 1% with prompt treatment, up to 10% if treatment delayed beyond 48 hours

Long-Term Outcomes

  • Most children have no long-term sequelae after successful treatment
  • Normal growth and development expected
  • No increased risk of future gastrointestinal problems
  • Quality of life typically returns to normal

Prevention and Risk Reduction

While most cases of intussusception cannot be prevented, certain measures may reduce risk or aid in early detection.

Primary Prevention

  • Rotavirus Vaccination: Current vaccines have much lower risk than earlier versions
  • Timely Treatment of Infections: Prompt management of gastrointestinal and respiratory infections
  • Genetic Counseling: For families with polyposis syndromes

Secondary Prevention (Recurrence)

  • Close Monitoring: After first episode, especially in first 48 hours
  • Parent Education: Recognition of warning signs
  • Investigation for Lead Points: In recurrent cases
  • Regular Follow-up: For children with predisposing conditions

Early Detection Strategies

  • Education of parents and caregivers about warning signs
  • High index of suspicion in emergency departments
  • Prompt imaging for suspicious cases
  • 24/7 availability of ultrasound in pediatric centers

Special Populations

Neonates (Under 3 Months)

  • Rare but often has pathological lead point
  • Higher association with intestinal atresia or duplication
  • More likely to require surgical treatment
  • Different presentation with bilious vomiting predominating

Adults

  • Chronic, intermittent symptoms more common
  • 90% have pathological lead point
  • CT scan preferred diagnostic modality
  • Surgery usually required
  • Oncologic principles applied if malignancy suspected

Immunocompromised Patients

  • Higher risk of lymphoma as lead point
  • May present with atypical symptoms
  • Increased risk of complications
  • Require careful evaluation for underlying causes

Post-Surgical Patients

  • Can develop postoperative intussusception
  • Usually small bowel involvement
  • May be related to prolonged ileus
  • Often resolves spontaneously

Summary

Intussusception is a pediatric surgical emergency requiring prompt recognition and treatment. The condition occurs when one segment of intestine telescopes into another, causing obstruction and potentially compromising blood supply. While the classic triad of colicky abdominal pain, vomiting, and bloody stools occurs in only a minority of cases, healthcare providers must maintain a high index of suspicion, particularly in children aged 6 months to 3 years presenting with intermittent severe abdominal pain.

Ultrasound has emerged as the diagnostic modality of choice in children, offering high sensitivity and specificity without radiation exposure. When diagnosed early, non-surgical reduction with air or hydrostatic enema is successful in 80-95% of cases. However, the presence of peritonitis, perforation, or failed reduction mandates surgical intervention. Adult intussusception, while rare, almost always has a pathological lead point and typically requires surgical treatment with resection.

The key to optimal outcomes is early recognition and treatment. With prompt intervention, mortality is less than 1%, and most children recover completely without long-term sequelae. Parents and healthcare providers should be educated about the warning signs, particularly the pattern of intermittent severe pain and the potential for rapid deterioration. Any child with suspected intussusception requires immediate medical evaluation and should be managed at a facility capable of both radiologic reduction and emergency surgery.