Enterocolitis Associated with Hirschsprung's Disease

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

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II. Pathophysiology

  1. Typically occurs after definitive Hirschsprung's pull-through repair (1 to 20 months post-operatively)

III. Risk Factors

  1. Down Syndrome

IV. 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

V. 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

VI. 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

VII. References

  1. Coran (2000) Am J Surg 180:382-7 [PubMed]
  2. Frykman (2012) Semin Pediatr Surg 21(4): 328-35 [PubMed]

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