Hirschsprung's disease Congenital aganglionic megacolon

Hirschsprung's disease
  • Facts:
    Enlargement of the colon, caused by bowel obstruction resulting from an aganglionic section of bowel (the normal enteric nerves are absent)

    Starts at the anus and progresses upwards

    Length of bowel that is affected varies but seldom stretches for more than about 30 cm.
  • History / PE:
    Delayed passage of meconium
    Abdominal distension
  • Diagnosis:
    Baby who has not passed meconium within 48 hours of delivery
    Definitive diagnosis is made by suction biopsy of the distally narrowed segment
  • Treatment:
    Surgical removal (resection) of the abnormal section of the colon, followed by reanastomosis
  • Associated With:
    Down's syndrome

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September 9th 2010