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Hirschsprung Disease, Emergency Medicine

Sheila McMorrow Reviewed 06/2017



  • Described in 1886 by Harold Hirschsprung as a cause of constipation in early infancy

  • Congenital aganglionosis megacolon

  • 1:5,000 live births

  • Overall mortality of Hirschsprung enterocolit...


Signs and Symptoms

Children with Hirschsprung disease are usually diagnosed by the age of 2 yr 
  • Three presentations, varying with age:

    • Neonatal

      • Abdominal distension

      • Failure of passage of meconium...


Initial Stabilization/Therapy

  • Airway, breathing, and circulation management with monitoring as appropriate

  • Initial bolus of isotonic IV fluids (20 cc/kg) for shock, dehydration, sepsis

Ed Treatment/Procedures



Admission Criteria

  • Neonates and infants presenting with bowel obstruction

  • Enterocolitis

  • Ill-appearing infants should be admitted to a neonatal/pediatric intensive care unit with avai...

Pearls and Pitfalls

  • Presentation varies with the age of the child

  • Continuum may vary from toxicity and enterocolitis to chronic constipation

  • Toxic child need stabilization, antibiotics and emergent imag...

Additional Reading

  • Amiel  J, Lyonnet  S. Hirschsprung disease, associated syndromes, and genetics: A review. J Med Genet.  2001;38:729–739.

  • Kays  DW. Surgical conditions of the neonatal intestinal...



751.3 Hirschsprung's disease and other congenital functional disorders of colon 


Q43.1 Hirschsprung's disease 


  • 204739008 Hirschsprung's disease (disorder)

  • 204741009 Short segment Hir...

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