Hirschsprungs Disease

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  • congenital abnormality of part of bowel
  • from recto-sigmoid junction proximally
  • all intramural autonomic nerves
  • 75% cases is confined to recto-sigmoid area
  • 8% affects all of large bowel
  • absent peristalsis in affected region and failure of relaxation of internal anal sphincter

Presentation

Examination

  • rectal examination
    • explosive release of air and meconium

Investigations

  • barium enema
    • non-dilated rectum
    • dilated proximal colon
  • mucosal suction biopsies

Treatment

  • 6-9 months
    • formation of a colostomy and resection of affected bowel
  • definitive operation later
    • frozen section to identify level
    • normal bowel anastomosed

Complications

  • severe enterocolitis
    • where diagnosis delayed
    • death from hypovolaemia
 

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