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NICU guideline identifier

Surgery - management of Hirschsprung's Disease in the neonate

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Hirschprung's Disease is a congenital absence of normal ganglionic cells in the mesenteric plexus of the bowel, resulting in abnormal peristalsis. It may present with delayed passage of meconium (after 24 hours of birth), or may present as an enterocolitis. In older children, it may present as chronic constipation.

Pre-operative care of the infant with Hirschsprung's Disease

  1. Follow standard pre-operative care guideline and the guideline for infants with necrotising enterocolitis.
  2. Rectal temperatures may assist. 
  3. Dilatations of the rectum should be performed as directed by the surgeon, with rectal washouts using 10ml/kg of 0.9% NaCl. 
  4. Bowel preparation may be required (instructions will be provided by the surgeon).

Post-operative care of the infant with Hirschsprung's Disease

  1. Follow standard steps for post-operative care and the necrotising enterocolitis guideline. 
  2. No rectal temperatures. 
  3. The retro-rectal drain is removed on the 2nd or 3rd post-operative day as per the surgeon's instructions. 
  4. There may be a single stage repair in which case the baby will be discharged and readmitted 2-weeks later for an examination under anaesthesia, then commence twice-daily dilatations. 
  5. If a colostomy has been fashioned, the baby will be discharged and will require weekly washouts with normal saline of the distal stoma. The baby will later be admitted for a pull-through procedure and closure of the colostomy.

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Document Control

  • Date last published: 04 January 2005
  • Document type: Clinical Guideline
  • Services responsible: Neonatology
  • Owner: Newborn Services Clinical Practice Committee
  • Editor: Sarah Bellhouse
  • Review frequency: 2 years