Hirschsprung Disease



Hirschsprung disease is a developmental disorder of the enteric nervous system that is characterized by the absence of ganglion cells in the myenteric and submucosal plexuses of the distal intestine. This results in absent peristalsis in the affected bowel and the development of a functional intestinal obstruction. In most cases, the aganglionosis involves the rectum or rectosigmoid, but it can extend for varying lengths and in 5–10% of cases can involve the entire colon or even a significant amount of the small intestine. The incidence of Hirschsprung disease is approximately one in 5,000 live born infants.


Ganglion Cell Anorectal Manometry Rectal Biopsy Contrast Enema Intestinal Neuronal Dysplasia 
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Suggested Reading

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Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.Hospital for Sick Children, Division of Thoracic and General SurgeryUniversity of TorontoTorontoCanada

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