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Congenital aganglionosis of the distal bowel of unknown etiology. Begins with the anus, which is always involved, and continues proximally for a variable distance. Both the myenteric (Auerbach) and submucosal (Meissner) plexus are absent, resulting in reduced bowel peristalsis and function.

  • Transition zone from abnormally small distal to dilated proximal colon
  • Normal rectum should always be larger than sigmoid

Clinical Presentation

  • Patients do not pass meconium within the first 24 hours of life.
  • Abdominal distension with bilious vomiting or nasogastric aspirates.

See Also