Intussusception

Overview

Definition

Intussusception is telescoping of the proximal part (intussusceptum) into a distal part (intussuscipiens) of the intestine.

Epidemiology

• Incidence Incidence The number of new cases of a given disease during a given period in a specified population. It also is used for the rate at which new events occur in a defined population. It is differentiated from prevalence, which refers to all cases in the population at a given time. Measures of Disease Frequency: 26–38 per every 100,000 live births
• Most common cause of bowel obstruction Bowel obstruction Any impairment, arrest, or reversal of the normal flow of intestinal contents toward the anal canal. Ascaris/Ascariasis in the 6–36-month age group
• 60% of cases within 1st year of life
• Boys > girls (ratio of 3:2)
• Most prevalent around viral season

Etiology

• Idiopathic Idiopathic Dermatomyositis (75%–80% of cases) with no identifiable lead point:
  • Most common in children
  • Rare in adults
• Infection (causes Peyer’s patch Patch Nonpalpable lesion > 1 cm in diameter Generalized and Localized Rashes enlargement):
  • Upper respiratory tract infection (30%)
  • Bacterial enteritis Enteritis Inflammation of any segment of the small intestine. Lactose Intolerance
  • Recent rotavirus Rotavirus A genus of Reoviridae, causing acute gastroenteritis in birds and mammals, including humans. Transmission is horizontal and by environmental contamination. Seven species (rotaviruses A through G) are recognized. Rotavirus immunization or infection
• Underlying pathology:
  • Meckel’s diverticulum Diverticulum A pouch or sac opening from the colon. Diverticular Disease (most common)
  • Henoch-Schönlein purpura Henoch-Schönlein Purpura Henoch-Schönlein purpura (HSP), also known as immunoglobulin A vasculitis, is an autoimmune small-vessel vasculitis that typically presents as a tetrad of abdominal pain, arthralgia, hematuria, and purpuric rash. Henoch-Schönlein Purpura (causes thickening of the mucosa)
  • Lymphoma Lymphoma A general term for various neoplastic diseases of the lymphoid tissue. Imaging of the Mediastinum
  • Intestinal polyps or tumors

Pathophysiology and Clinical Presentation

Pathophysiology

• Intussusception is considered idiopathic Idiopathic Dermatomyositis if it does not involve a lead point.
• Idiopathic Idiopathic Dermatomyositis intussusception is most common in the pediatric population.
• Lead point: a lesion that gets trapped during peristalsis Peristalsis A movement, caused by sequential muscle contraction, that pushes the contents of the intestines or other tubular organs in one direction. Gastrointestinal Motility, dragging that segment into the distal part of the intestine:
  • Tumor Tumor Inflammation/polyp
  • Meckel’s diverticulum Diverticulum A pouch or sac opening from the colon. Diverticular Disease
  • Duplication cyst
  • Vascular malformation
  • Hematoma
• Pathologic lead point should be suspected if:
  • Multiple episodes of intussusception
  • In adults and children < 6 months or > 3 years of age
• Based on anatomic location, intussusception can be:
  • Ileocecal/ileocolic (most common, accounts for 85%–90% of cases)
  • Ileoileal
  • Ileocolic
  • Jejuno-ileal
  • Jejuno-jejunal
  • Colo-colic
• Telescoping of bowel into itself → obstruction and impaired lymphatic drainage
• Increasing pressure in intussusceptum bowel wall → impairment of venous and lymphatic drainage → vascular compromise
• Ischemia Ischemia A hypoperfusion of the blood through an organ or tissue caused by a pathologic constriction or obstruction of its blood vessels, or an absence of blood circulation. Ischemic Cell Damage of intussusceptum mucosa → bowel mucosa infarcts and sloughs off → bloody stools
• Transmural necrosis Necrosis The death of cells in an organ or tissue due to disease, injury or failure of the blood supply. Ischemic Cell Damage and perforation Perforation A pathological hole in an organ, blood vessel or other soft part of the body, occurring in the absence of external force. Esophagitis may occur with prolonged ischemia Ischemia A hypoperfusion of the blood through an organ or tissue caused by a pathologic constriction or obstruction of its blood vessels, or an absence of blood circulation. Ischemic Cell Damage

Clinical presentation

Toddlers/infants:

• Sudden onset, cramping, severe intermittent abdominal pain Abdominal Pain Acute Abdomen
• Drawing up legs toward abdomen
• Inconsolable crying
• Episodes occur every 15–20 minutes and become more frequent over time.
• Vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia:
  • Non-bilious first
  • After a pain Pain An unpleasant sensation induced by noxious stimuli which are detected by nerve endings of nociceptive neurons. Pain: Types and Pathways episode
  • Can become bilious as obstruction worsens
• Grossly bloody stool (50% of cases)
• Classic triad (only present in 15% of cases):
  • Abdominal pain Abdominal Pain Acute Abdomen
  • Sausage-shaped mass Mass Three-dimensional lesion that occupies a space within the breast Imaging of the Breast in the RUQ
  • Currant-jelly stool:
    • Blood mixed with mucus
    • Suggests mucosal necrosis Necrosis The death of cells in an organ or tissue due to disease, injury or failure of the blood supply. Ischemic Cell Damage and sloughing
    • Late presentation

Adults/older children:

• Intermittent abdominal pain Abdominal Pain Acute Abdomen is most common symptom.
• Other symptoms of bowel obstruction Bowel obstruction Any impairment, arrest, or reversal of the normal flow of intestinal contents toward the anal canal. Ascaris/Ascariasis may occur:
  • Nausea Nausea An unpleasant sensation in the stomach usually accompanied by the urge to vomit. Common causes are early pregnancy, sea and motion sickness, emotional stress, intense pain, food poisoning, and various enteroviruses. Antiemetics/ vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia
  • Constipation Constipation Constipation is common and may be due to a variety of causes. Constipation is generally defined as bowel movement frequency < 3 times per week. Patients who are constipated often strain to pass hard stools. The condition is classified as primary (also known as idiopathic or functional constipation) or secondary, and as acute or chronic. Constipation
• Symptoms of underlying process (e.g., malignancy Malignancy Hemothorax, infection):
  • Fever Fever Fever is defined as a measured body temperature of at least 38°C (100.4°F). Fever is caused by circulating endogenous and/or exogenous pyrogens that increase levels of prostaglandin E2 in the hypothalamus. Fever is commonly associated with chills, rigors, sweating, and flushing of the skin. Fever
  • Weight loss Weight loss Decrease in existing body weight. Bariatric Surgery

Diagnosis

Pediatric intussusception

History:

• Episodes of crying, legs drawn toward abdomen
• Episodes of lethargy Lethargy A general state of sluggishness, listless, or uninterested, with being tired, and having difficulty concentrating and doing simple tasks. It may be related to depression or drug addiction. Hyponatremia
• Bloody stools
• Vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia
• Fever Fever Fever is defined as a measured body temperature of at least 38°C (100.4°F). Fever is caused by circulating endogenous and/or exogenous pyrogens that increase levels of prostaglandin E2 in the hypothalamus. Fever is commonly associated with chills, rigors, sweating, and flushing of the skin. Fever
• Sick contacts
• Potential food poisoning Food poisoning Acute illnesses, usually affecting the gastrointestinal tract, brought on by consuming contaminated food or beverages. Most of these diseases are infectious, caused by a variety of bacteria, viruses, or parasites that can be foodborne. Sometimes the diseases are caused by harmful toxins from the microbes or other chemicals present in the food. Especially in the latter case, the condition is often called food poisoning. Clostridia/toxin exposure

Physical exam:

• General appearance:
  • Pallor
  • Lethargy Lethargy A general state of sluggishness, listless, or uninterested, with being tired, and having difficulty concentrating and doing simple tasks. It may be related to depression or drug addiction. Hyponatremia
• Palpation Palpation Application of fingers with light pressure to the surface of the body to determine consistency of parts beneath in physical diagnosis; includes palpation for determining the outlines of organs. Dermatologic Examination:
  • Sausage-shaped mass Mass Three-dimensional lesion that occupies a space within the breast Imaging of the Breast in the RUQ
  • Dance’s sign: scaphoid (empty) RLQ
  • May or may not have focal abdominal tenderness
  • Guarding, rebound: usually late signs associated with bowel ischemia Bowel ischemia Mesenteric ischemia is a rare, life-threatening condition caused by inadequate blood flow through the mesenteric vessels, which results in ischemia and necrosis of the intestinal wall. Mesenteric ischemia can be either acute or chronic. Mesenteric Ischemia
• Auscultation: high-pitched bowel sounds (indicative of obstruction)

Imaging:

• Abdominal ultrasound:
  • Best initial test
  • Target sign Target sign Pseudomembranous Colitis: the invaginated portion of the bowel appears as a ring on a target (outer bowel wall)
  • Pseudokidney sign: the appearance of the intussuscepted segment of bowel, which mimics a kidney
• Abdominal X-ray X-ray Penetrating electromagnetic radiation emitted when the inner orbital electrons of an atom are excited and release radiant energy. X-ray wavelengths range from 1 pm to 10 nm. Hard x-rays are the higher energy, shorter wavelength x-rays. Soft x-rays or grenz rays are less energetic and longer in wavelength. The short wavelength end of the x-ray spectrum overlaps the gamma rays wavelength range. The distinction between gamma rays and x-rays is based on their radiation source. Pulmonary Function Tests: 
  • Not very sensitive or specific test
  • Crescent Crescent Rapidly Progressive Glomerulonephritis sign: soft-tissue density projecting into colonic lumen
  • Signs of small bowel obstruction Bowel obstruction Any impairment, arrest, or reversal of the normal flow of intestinal contents toward the anal canal. Ascaris/Ascariasis:
    • Dilated loop
    • Absence of gas in the colon Colon The large intestines constitute the last portion of the digestive system. The large intestine consists of the cecum, appendix, colon (with ascending, transverse, descending, and sigmoid segments), rectum, and anal canal. The primary function of the colon is to remove water and compact the stool prior to expulsion from the body via the rectum and anal canal. Colon, Cecum, and Appendix: Anatomy
  • Pneumoperitoneum Pneumoperitoneum A condition with trapped gas or air in the peritoneal cavity, usually secondary to perforation of the internal organs such as the lung and the gastrointestinal tract, or to recent surgery. Pneumoperitoneum may be purposely introduced to aid radiological examination. Perforated Viscus if perforation Perforation A pathological hole in an organ, blood vessel or other soft part of the body, occurring in the absence of external force. Esophagitis has occurred
• Abdominal CT: 
  • Performed only if other modalities yield unclear diagnosis
  • Usually identifies an underlying pathology (lead point) if present
• Contrast or pneumatic enema:
  • Confirmatory
  • Therapeutic as well as diagnostic for ileocecal intussusception
  • Performed using ultrasound or fluoroscopy Fluoroscopy Production of an image when x-rays strike a fluorescent screen. X-rays
  • Procedure: Air/contrast is injected into the intestines to create pressure, which ejects the trapped part of the bowel out of the distal bowel.

Intussusception in adults

History:

• Will reveal symptoms of bowel obstruction Bowel obstruction Any impairment, arrest, or reversal of the normal flow of intestinal contents toward the anal canal. Ascaris/Ascariasis
• Can be acute in onset or slow, insidious, and intermittent

Physical exam:

• Abdominal distention Abdominal distention Megacolon
• Focal or diffuse tenderness
• Peritonitis Peritonitis Inflammation of the peritoneum lining the abdominal cavity as the result of infectious, autoimmune, or chemical processes. Primary peritonitis is due to infection of the peritoneal cavity via hematogenous or lymphatic spread and without intra-abdominal source. Secondary peritonitis arises from the abdominal cavity itself through rupture or abscess of intra-abdominal organs. Penetrating Abdominal Injury is a late presentation indicative of ischemia Ischemia A hypoperfusion of the blood through an organ or tissue caused by a pathologic constriction or obstruction of its blood vessels, or an absence of blood circulation. Ischemic Cell Damage/ perforation Perforation A pathological hole in an organ, blood vessel or other soft part of the body, occurring in the absence of external force. Esophagitis.

Imaging:

• Abdominal X-ray X-ray Penetrating electromagnetic radiation emitted when the inner orbital electrons of an atom are excited and release radiant energy. X-ray wavelengths range from 1 pm to 10 nm. Hard x-rays are the higher energy, shorter wavelength x-rays. Soft x-rays or grenz rays are less energetic and longer in wavelength. The short wavelength end of the x-ray spectrum overlaps the gamma rays wavelength range. The distinction between gamma rays and x-rays is based on their radiation source. Pulmonary Function Tests:
  • Nonspecific
  • Can show obstructive pattern:
    • Dilated small bowel Dilated Small Bowel Imaging of the Intestines loops
    • Paucity of colonic gas
    • Pneumoperitoneum Pneumoperitoneum A condition with trapped gas or air in the peritoneal cavity, usually secondary to perforation of the internal organs such as the lung and the gastrointestinal tract, or to recent surgery. Pneumoperitoneum may be purposely introduced to aid radiological examination. Perforated Viscus if perforation Perforation A pathological hole in an organ, blood vessel or other soft part of the body, occurring in the absence of external force. Esophagitis occurred
• Abdominal CT scan:
  • Small bowel obstruction Bowel obstruction Any impairment, arrest, or reversal of the normal flow of intestinal contents toward the anal canal. Ascaris/Ascariasis:
    • Dilated small bowel Dilated Small Bowel Imaging of the Intestines loops
    • Collapsed distal small bowel Small bowel The small intestine is the longest part of the GI tract, extending from the pyloric orifice of the stomach to the ileocecal junction. The small intestine is the major organ responsible for chemical digestion and absorption of nutrients. It is divided into 3 segments: the duodenum, the jejunum, and the ileum. Small Intestine: Anatomy and colon Colon The large intestines constitute the last portion of the digestive system. The large intestine consists of the cecum, appendix, colon (with ascending, transverse, descending, and sigmoid segments), rectum, and anal canal. The primary function of the colon is to remove water and compact the stool prior to expulsion from the body via the rectum and anal canal. Colon, Cecum, and Appendix: Anatomy
  • Bowel wall thickening
  • Target sign Target sign Pseudomembranous Colitis, sausage-shaped mass Mass Three-dimensional lesion that occupies a space within the breast Imaging of the Breast

Management

Pediatric intussusception

Initial management: 

• Nothing by mouth
• Nasogastric decompression
• Fluid resuscitation Resuscitation The restoration to life or consciousness of one apparently dead. . Neonatal Respiratory Distress Syndrome

Nonsurgical reduction:

• 1st-line therapy for:
  • Ileocecal intussusception
  • Stable patients Stable Patients Blunt Chest Trauma without peritonitis Peritonitis Inflammation of the peritoneum lining the abdominal cavity as the result of infectious, autoimmune, or chemical processes. Primary peritonitis is due to infection of the peritoneal cavity via hematogenous or lymphatic spread and without intra-abdominal source. Secondary peritonitis arises from the abdominal cavity itself through rupture or abscess of intra-abdominal organs. Penetrating Abdominal Injury/evidence of perforation Perforation A pathological hole in an organ, blood vessel or other soft part of the body, occurring in the absence of external force. Esophagitis/ ischemia Ischemia A hypoperfusion of the blood through an organ or tissue caused by a pathologic constriction or obstruction of its blood vessels, or an absence of blood circulation. Ischemic Cell Damage
• Accomplished with contrast or pneumatic enema
• Success rate: 80%–95% in experienced centers
• If reduction is partial, a repeat reduction can be attempted.
• Recurrence rate is 10%–20% (50% of those within first 72 hours).
• A repeat reduction can be performed for a stable patient.

Surgical management:

• Indications:
  • Unstable patient, peritonitis Peritonitis Inflammation of the peritoneum lining the abdominal cavity as the result of infectious, autoimmune, or chemical processes. Primary peritonitis is due to infection of the peritoneal cavity via hematogenous or lymphatic spread and without intra-abdominal source. Secondary peritonitis arises from the abdominal cavity itself through rupture or abscess of intra-abdominal organs. Penetrating Abdominal Injury; evidence of perforation Perforation A pathological hole in an organ, blood vessel or other soft part of the body, occurring in the absence of external force. Esophagitis/ ischemia Ischemia A hypoperfusion of the blood through an organ or tissue caused by a pathologic constriction or obstruction of its blood vessels, or an absence of blood circulation. Ischemic Cell Damage
  • Completely unsuccessful reduction attempt with enema
  • A persistent filling defect after reduction, suggesting a tumor Tumor Inflammation
  • For a small bowel-to-small bowel intussusception:
    • Some will resolve spontaneously.
    • If intussusception does not resolve, surgery is usually indicated.
    • Reduction with enema is usually unsuccessful.
    • Also, higher probability Probability Probability is a mathematical tool used to study randomness and provide predictions about the likelihood of something happening. There are several basic rules of probability that can be used to help determine the probability of multiple events happening together, separately, or sequentially. Basics of Probability of a lead point/underlying pathology
• Procedures:
  • Hutchinson maneuver: 
    • Manual reduction of the intussusception
    • Bowel may appear edematous/inflamed, but if viable, does not need to be resected.
    • If there is no lead point identified, only manual reduction is needed.
    • Concomitant appendectomy Appendectomy Appendectomy is an invasive surgical procedure performed with the goal of resecting and extracting the vermiform appendix through either an open or a laparoscopic approach. The most common indication is acute appendicitis. Appendectomy is sometimes performed.
    • Can be performed open or laparoscopically
  • Bowel resection:
    • For bowel perforation Bowel perforation Perforated viscus or GI perforation represents a condition in which the integrity of the GI wall is lost with subsequent leakage of enteric contents into the peritoneal cavity, resulting in peritonitis. The causes of perforated viscus include trauma, bowel ischemia, infections, or ulcerative conditions, all of which ultimately lead to a full-thickness disruption of the intestinal wall. Perforated Viscus
    • For nonviable bowel: permanent dullness or no detectable pulsation
    • Pathologic lead point identified (Meckel’s diverticulum Diverticulum A pouch or sac opening from the colon. Diverticular Disease, tumor Tumor Inflammation)

Adult intussusception

General considerations:

• Asymptomatic intussusception without obstruction:
  • Usually an incidental finding on CT scan
  • Will resolve spontaneously and does not require an intervention
• Intussusception associated with bowel obstruction Bowel obstruction Any impairment, arrest, or reversal of the normal flow of intestinal contents toward the anal canal. Ascaris/Ascariasis:
  • Usually associated with pathologic lead point (> 92% of cases)
  • Can involve small bowel Small bowel The small intestine is the longest part of the GI tract, extending from the pyloric orifice of the stomach to the ileocecal junction. The small intestine is the major organ responsible for chemical digestion and absorption of nutrients. It is divided into 3 segments: the duodenum, the jejunum, and the ileum. Small Intestine: Anatomy or colon Colon The large intestines constitute the last portion of the digestive system. The large intestine consists of the cecum, appendix, colon (with ascending, transverse, descending, and sigmoid segments), rectum, and anal canal. The primary function of the colon is to remove water and compact the stool prior to expulsion from the body via the rectum and anal canal. Colon, Cecum, and Appendix: Anatomy
  • Requires surgery
  • General approach is the same as for any bowel obstruction Bowel obstruction Any impairment, arrest, or reversal of the normal flow of intestinal contents toward the anal canal. Ascaris/Ascariasis.

Initial/supportive management:

• Nothing by mouth
• Nasogastric decompression
• Intravenous fluids Intravenous Fluids Intravenous fluids are one of the most common interventions administered in medicine to approximate physiologic bodily fluids. Intravenous fluids are divided into 2 categories: crystalloid and colloid solutions. Intravenous fluids have a wide variety of indications, including intravascular volume expansion, electrolyte manipulation, and maintenance fluids. Intravenous Fluids, electrolyte correction

Surgery:

• Procedure depends on underlying cause
• Usually involves bowel/pathologic lead point resection ( tumor Tumor Inflammation, Meckel’s diverticulum Diverticulum A pouch or sac opening from the colon. Diverticular Disease)

Differential Diagnosis

• Volvulus Volvulus A volvulus is the twisting or axial rotation of a portion of the bowel around its mesentery. The most common site of volvulus in adults is the colon; most frequently the sigmoid volvulus. Patients typically present with symptoms of bowel obstruction such as abdominal pain, distension, vomiting, and constipation/obstipation. Volvulus: small bowel obstruction Bowel obstruction Any impairment, arrest, or reversal of the normal flow of intestinal contents toward the anal canal. Ascaris/Ascariasis secondary to intestinal malrotation Malrotation Pediatric Gastrointestinal Abnormalities in children. Patients Patients Individuals participating in the health care system for the purpose of receiving therapeutic, diagnostic, or preventive procedures. Clinician–Patient Relationship have a similar presentation to intussusception, with vomiting Vomiting The forcible expulsion of the contents of the stomach through the mouth. Hypokalemia and drawing in of the knees. Diagnosis can be established with ultrasound or upper GI series Upper Gi Series Imaging of the Intestines. This condition requires emergent surgery for bowel detorsion.
• Strangulated inguinal hernia Hernia Protrusion of tissue, structure, or part of an organ through the bone, muscular tissue, or the membrane by which it is normally contained. Hernia may involve tissues such as the abdominal wall or the respiratory diaphragm. Hernias may be internal, external, congenital, or acquired. Abdominal Hernias: a nonreducible inguinal hernia Hernia Protrusion of tissue, structure, or part of an organ through the bone, muscular tissue, or the membrane by which it is normally contained. Hernia may involve tissues such as the abdominal wall or the respiratory diaphragm. Hernias may be internal, external, congenital, or acquired. Abdominal Hernias containing a loop of bowel with compromised blood supply. Presents as irreducible inguinal mass Mass Three-dimensional lesion that occupies a space within the breast Imaging of the Breast that is usually tender and associated with symptoms of small bowel obstruction Bowel obstruction Any impairment, arrest, or reversal of the normal flow of intestinal contents toward the anal canal. Ascaris/Ascariasis. Diagnosis is usually established with physical exam. Management involves emergent surgical hernia Hernia Protrusion of tissue, structure, or part of an organ through the bone, muscular tissue, or the membrane by which it is normally contained. Hernia may involve tissues such as the abdominal wall or the respiratory diaphragm. Hernias may be internal, external, congenital, or acquired. Abdominal Hernias repair.
• Gastroenteritis Gastroenteritis Gastroenteritis is inflammation of the stomach and intestines, commonly caused by infections from bacteria, viruses, or parasites. Transmission may be foodborne, fecal-oral, or through animal contact. Common clinical features include abdominal pain, diarrhea, vomiting, fever, and dehydration. Gastroenteritis: an acute viral infection of the GI tract that presents with diarrhea Diarrhea Diarrhea is defined as ≥ 3 watery or loose stools in a 24-hour period. There are a multitude of etiologies, which can be classified based on the underlying mechanism of disease. The duration of symptoms (acute or chronic) and characteristics of the stools (e.g., watery, bloody, steatorrheic, mucoid) can help guide further diagnostic evaluation. Diarrhea and abdominal pain Abdominal Pain Acute Abdomen. Watery diarrhea Watery diarrhea Rotavirus is usually a prominent feature, and diagnosis is established clinically. Management is usually supportive and involves oral or intravenous hydration.
• Colic: acute episodes of abdominal pain Abdominal Pain Acute Abdomen in infants associated with crying and irritability. Can be confused with intussusception, but the infant is healthy and thriving, with a normal exam.
• Appendicitis Appendicitis Appendicitis is the acute inflammation of the vermiform appendix and the most common abdominal surgical emergency globally. The condition has a lifetime risk of 8%. Characteristic features include periumbilical abdominal pain that migrates to the right lower quadrant, fever, anorexia, nausea, and vomiting. Appendicitis: acute inflammation Acute Inflammation Inflammation of the appendix Appendix A worm-like blind tube extension from the cecum. Colon, Cecum, and Appendix: Anatomy. In young children, presents with abdominal pain Abdominal Pain Acute Abdomen and crying spells. Right lower quadrant Right lower quadrant Anterior Abdominal Wall: Anatomy is usually exquisitely tender on physical exam with no palpable mass Mass Three-dimensional lesion that occupies a space within the breast Imaging of the Breast. Diagnosis is established based on exam and imaging studies, usually ultrasound in children. Management is appendectomy Appendectomy Appendectomy is an invasive surgical procedure performed with the goal of resecting and extracting the vermiform appendix through either an open or a laparoscopic approach. The most common indication is acute appendicitis. Appendectomy.