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Hirschsprung Disease, Pediatric

Lusine Ambartsumyan, MD Reviewed 10/2018
 


BASICS

DESCRIPTION

  • Congenital motor disorder of the gut characterized by lack of ganglion cells in the distal bowel beginning at the anal verge and extending proximally to varying lengths

  • Lack of intern...

DIAGNOSIS

HISTORY

  • 80% of patients present in the neonatal period.

  • 15% diagnosed in 1st month, 40–50% within 3 months, 60% within 1 year, and 85% within 4 years

  • Adult diagnosis of HD reported in 2% of pop...

TREATMENT

Surgical resection of the aganglionic segment and subsequent pull-through of the ganglionic segment to the anus 

GENERAL MEASURES

Stabilizing treatment if child presents with suspected enteroc...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

  • Followed on a regular basis for the 1st decade after surgery

  • Close monitoring for poor growth, fecal incontinence, obstructive symptoms (constipation, abdominal di...

ADDITIONAL READING

  • Chumpitazi B, Nurko S. Defecation disorders in children after surgery for Hirschsprung disease. J Pediatr Gastroenterol Nutr.  2011;53(1):75–79. [View Abstract on OvidInsights]

  • ...

CODES

ICD9

  • 751.3 Hirschsprung’s disease and other congenital functional disorders of colon

ICD10

  • Q43.1 Hirschsprung’s disease

SNOMED

  • 204739008 Hirschsprung’s disease (disorder)

  • 204745000 Total intestinal ag...

FAQ

  • Q: What is the likelihood that a newborn with a diagnosis of HD will have normal bowel function after surgery?

  • A: There is a good likelihood, but it may take a number of years: A review of 178 pati...

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