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Updated: Sep 27 2022


  • Snapshot
    • A 10-day-old baby boy is brought to the emergency room for repeated episodes of vomiting. His mother describes the vomit as green and yellow. On physical exam, the physician has difficulty at first locating the heartbeat, but finally hears a heartbeat on the right side of the chest. Suspicious, he sends the baby for abdominal radiography and upper GI series. Imaging reveals dilated bowel loops and a “birds beak” appearance of bowel.
  • Introduction
    • Malrotation 
      • errors during development
      • increases risk of midgut volvulus
        • 1/3 of children with malrotation develop volvulus
    • Midgut volvulus
      • abnormal rotation of bowel due to pathologic adhesions fixating the bowel around itself
        • twists around SMA
        • compromises blood flow to bowel
      • majority in ileum
      • increased risk of bowel necrosis and perforation
    • Cecal volvulus
      • common in young, active patients
      • "coffee bean" sign
    • Associated conditions
      • gastroschisis
      • omphalocele
      • situs inversus
      • cardiovascular defects
      • Hirschsprung disease
      • heterotaxy
    • Malrotation with midgut volvulus is a surgical emergency
  • Epidemiology
    • Most common in newborns
    • Males > females
    • 80% of cases are newborn
    • Elderly population can have malrotation, but volvulus is less often
  • Presentation
    • Symptoms
      • neonates (majority of patients)
        • bilious vomiting within first week of life
        • colicky pain
      • older patients
        • nonbilious vomiting
        • crampy abdominal pain
        • change in bowel pattern
        • nausea
    • Physical exam
      • abdominal distention
      • abdominal tenderness
      • acute abdomen
      • shock
  • Imaging
    • Abdominal radiography
      • multiple air fluid levels
      • dilated loops of bowel with loss of haustra
      • normal radiography does not exclude disease
    • Upper GI imaging with barium enema
      • “bird beak” appearance at site of rotation
      • “coffee bean” appearance of bowel
      • failure of duodenum to cross midline = malrotation
  • Differential Diagnosis
    • Intussusception
    • Intestinal atresia
  • Treatment
    • Malrotation without volvulus
      • elective Ladd procedure
    • Malrotation with volvulus (symptomatic or acute abdomen)
      • emergent endoscopic decompression
      • emergent surgical decompression
    • Prevention
      • if patient has heterotaxy, screen for malrotation
      • elective Ladd procedure if malrotation without volvulus
  • Complications
    • Bowel necrosis and perforation
    • Sepsis
  • Prognosis
    • Most with resolution of symptoms after surgery
    • Good prognosis
      • mortality rate 3-9%, worse with
        • premature neonates
        • bowel necrosis
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