Hirschsprung disease


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  • Pearls

    • must be at least 2-3 cm above dentate line
    • must not be in transitional zone (anal transitional epithelium)
      • urothelial-like
      • squamous
      • columnar
      • has striated muscle

  • Histology

    • lack of ganglia cells in the plexuses
    • distractors: lymphocytes, stromal cells, endothelial cells

  • Normal

    • ganglion cells
    • superficial plexus
    • deep submucosal plexus
    • myenteric plexus

  • Stains

    • calretinin will be negative in Hirschprungs (except for mast cells)
    • calretinin will be positive in ganglia and nerve fibers in normal bowel
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