Hirschsprung’s Disease (congenital aganglionic megacolon)

 

congenital condition in which a section the colon is aganglionic

there is little urge to defecate so the feces accumulate.

  • genetic defect on Chr 10
  • 1/5000 live births
  • Sigmoid colon typically affected

 

  Mechanism:

  • absence of ganglia in parts of the colon
  • no peristalsis occurs in affected region
  • contents accumulate
  • colon becomes distended, in turn results in distended abdomen
  • bowel obstruction may occur

 

Treatment:

  • Surgical exision of aganglionic region

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