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Enterocolitis Associated with Hirschsprung's Disease

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Enterocolitis Associated with Hirschsprung's Disease, Hirschsprung-Related Enterocolitis, Hirschprung Associated Enterocolitis, HAEC

  • See Also
  1. Hirschsprung's Disease
  • Pathophysiology
  1. Typically occurs after definitive Hirschsprung's pull-through repair (1 to 20 months post-operatively)
  • Risk Factors
  1. Down Syndrome
  • Symptoms
  1. Early
    1. Abdominal Distention
    2. Foul-smelling watery stools or Diarrhea
    3. Lethargy
    4. Failure to Thrive
  2. Late
    1. Emesis or Hematochezia
    2. Fever
    3. Sepsis
    4. High mortality rate
  • Imaging
  1. Abdominal XRay
    1. Dilated bowel loops with air-fluid levels
    2. Rectosigmoid cut-off sign
    3. Pneumotosis intestinalis
    4. Intestinal perforation findings may be present
  • Management
  1. Acute stabilization
    1. Intravenous Fluids
    2. Nasogastric suction
    3. Broad spectrum IV Antibiotics
  2. Rectal irrigation 3-4 times daily
    1. Normal Saline infused in Rectum via Rubber catheter
    2. Insert in steps
      1. Total infused: 10-15 cc/kg
      2. Wait for 10-15 cc to leak out of Rectum
        1. Then insert cathetr further
  3. Disposition
    1. Hospital observation for serial abdominal exams is typically recommended even in mild cases
  • References
  1. Coran (2000) Am J Surg 180:382-7 [PubMed]
  2. Frykman (2012) Semin Pediatr Surg 21(4): 328-35 [PubMed]