Peds

Intestinal Malrotation

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Intestinal Malrotation

  • See Also
  • Epidemiology
  1. Incidence: 1 in 500 live births
    1. Half the Incidence of Pyloric Stenosis
  2. Typically diagnosed in infants and children
    1. First month of life presentation in 60-85% of cases
    2. Diagnosis by age 1 year in 90% of cases
  • Pathophysiology
  1. Congenital Anomaly
  2. Duodenojejunal junction with abnormal position
  • Signs
  1. Presents as Midgut Volvulus in 75% of cases
    1. Of these, 75% present in the first month (most in first week)
    2. Presents as an infant with acute Bilious Emesis and Abdominal Distention
  1. Pyloric Stenosis
  2. Duodenal Atresia
  • Imaging
  1. Malrotation: Upper gastrointestinal series
    1. False positive and false negatives in 15% of cases
    2. Lower Test Specificity in older children and adults (compared with infants and young children)
  2. Acute Midgut Volvulus: Abdominal Xray
    1. Often non-specific
    2. Dilated Stomach and proximal duodenum with intervening pylorus (Double Bubble Sign)
      1. Double-Bubble is also seen in duodenal atresia (typically diagnosed in newborn nursery)
  • Risk Factors
  • Associated Syndromes
  1. Familial Intestinal Malrotation
  2. Marfan Syndrome
  3. Prune Belly Syndrome
  4. Chromosomal abnormalities
    1. Trisomy 13
    2. Trisomy 18
    3. Trisomy 21
  5. Other conditions (e.g. Cat-eye, Cantrell, Cornelia de Lange, Coffin-Siris, Heterotaxy, and Apple-Peel atresia)
  • Associated Conditions
  1. Kidney and ureter absence
  2. Biliary atresia
  3. Congenital Diaphragmatic Hernia
  4. Duodenal atresia or Small Bowel stenosis
  5. Gastroschisis
  6. Hirschprung Disease
  7. Imperforate anus
  8. Intussusception
  9. Meckel Diverticulum
  10. Omphalocele
  11. Pyloric Stenosis
  1. Emergent Surgical Intervention
    1. Mesenteric Infarction and necrosis starts within first 3 hours of onset
  • Complications
  1. Midgut Volvulus (75%)
    1. Consider in any toxic appearing infant with Bilious Emesis
    2. Differential Diagnosis includes Pyloric Stenosis, duodenal atresia
  2. Small Bowel Obstruction
  3. Malabsorption