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