Hirschsprung Disease

 Hirschsprung disease is a motility disorder due to defective migration of ganglion cell resulting in aganglionic segment in the bowel wall


Clinical Features

  ● Abdominal distention

  ● Failure to pass meconium

  ● Feed intolerance and aspiration

  ● Bilious vomiting

  ● Rectum is usually empty of faeces in peranal examination, but there is rapid expulsion of faeces and gases when the finger removed.


Investigations

  ● Gold standard investigation is rectal suction biopsy - Hypertrophied nerve bundles are seen in biopsy

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