Updated: 9/27/2022

Meconium Ileus

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  • Snapshot
    • A 2-day-old boy is brought to the emergency department by his mother after he became inconsolable with abdominal distention and has not vomited or passed his first feces. He was born to a 27-year-old woman via spontaneous vaginal delivery without any perinatal complications. The mother was not able to attend prenatal care visits to receive appropriate screening. An abdominal radiograph is performed, which shows dilated loops of bowel without any evidence for perforation. A hyperosmotic contrast enema radiography is performed and confirms meconium ileus. A sweat test is subsequently done to work-up for cystic fibrosis.
  • Introduction
    • Clinical definition
      • neonatal intestinal obstruction secondary to
        • meconium within the lumen of the bowel that has become thickened
  • Epidemiology
    • Incidence
      • can occur in approximately 20% of patients with cystic fibrosis
    • Risk factors
      • family history of
        • cystic fibrosis
        • meconium ileus
      • low birth weight
  • ETIOLOGY
    • Pathogenesis
      • accumulated thickened meconium leads to obstruction in the bowel lumen resulting in
        • dilation of the proximal ileum wall and
          • narrowing of the distal intestine
    • Genetics
      • inheritance pattern
        • cystic fibrosis is an autosomal recessive disease
      • mutations
        • gene
          • CF transmembrane conductance regulator (CFTR) gene
            • delta F508 is the most common mutation
              • there is a deletion of one amino acid (phenylalanine) in the 508 position of this gene
        • protein encoded for
          • CFTR protein
    • Associated conditions
      • cystic fibrosis
  • Presentation
    • Symptoms
      • failure to rectally pass meconium
    • Physical exam
      • abdominal distension
      • palpable bowel loops
      • may have visible peristaltic waves
  • Imaging
    • Radiographs
      • views
        • abdominal
      • findings
        • dilated loops of bowel proximal to the obstruction
        • narrowed loops of bowel distal to the obstruction
        • may see abdominal calcifications
    • Contrast enema
      • indications
        • performed to confirm and treat meconium ileus when there is no evidence of perforation
      • views
        • abdominal
      • findings
        • dilated bowel
        • colon may appear as a microcolon
  • Studies
    • Labs
      • sweat test after 48 hours of age in order to
        • work up for cystic fibrosis
  • Differential Diagnosis
    • Intestinal atresia
    • Hirschsprung disease
    • Volvulus
  • Treatment
    • Non-operative
      • hyperosmolar enema (e.g., Gastrografin)
        • indication
          • this is a non-operative approach to treat meconium ileus as this leads to
            • breakdown of the thickened meconium which clears the obstruction
          • this is typically performed in simple meconium ileus but
            • can be used in complicated meconium ileus
    • Operative
      • resection with enterostomy or primary anastomosis
        • indication
          • can be used in cases of simple meconium ileus that does not clear with hyperosmolar enema
          • this is typically performed in complicated meconium ileus
  • Complications
    • Complications
      • intussusception
      • segmental volvulus
      • intestinal perforation
      • meconium peritonitis
  • Prognosis
    • There is a low mortality risk after appropriate treatment of meconium ileus
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